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UNIVERSITY OF THE PHILIPPINES – PHILIPPINE GENERAL HOSPITAL

DEPARTMENT OF OTORHINOLARYNGOLOGY

with the continuing medical education support of

GLAXO SMITHKLINE PHILIPPINES

CLINICAL PRACTICE GUIDELINES

RUZANNE MAGIBA-CARO MD EDILBERTO M. JOSE MD Co-chairs

ERASMO GONZALO D.V. LLANES MD JASON S. GUEVARA MD CHRISTINE JOY S. ARQUIZA MD VINCENT MARK M. JARDIN MD CHRISTOPHER MALORRE E. CALAQUIAN MD JERIEL JOHN C. MAJAM MD JOSE ROBERTO V. CLARIDAD MD DANILO R. LEGITA MD ERICK G. DUCUT MD

CONSENSUS PANEL

GENEROSO T. ABES MD MPH Department Chair

MARIANO B. CAPARAS MD ROMEO L. VILLARTA, JR. MD MPH JOSELITO C. JAMIR MD JOSE FLORENCIO F. LAPEÑA, JR. MA MD EUTRAPIO S. GUEVARA JR MD TERESA LUISA I. GLORIA-CRUZ MD MHPEd ALFREDO Q.Y. PONTEJOS JR MD RAMON ANTONIO B. LOPA MD JAIME F. FLOR MD ROBERTO M. PANGAN DMD MD PhD JOSEFINO G. HERNANDEZ MD MARIA RINA T. REYES-QUINTOS MD MCAud RENE S. TUAZON MD MELFRED L. HERNANDEZ MD MHA JACOB S. MATUBIS MD NATHANIEL W. YANG MD CESAR V. VILLAFUERTE JR MD MHA ARMANDO M. CHIONG, JR. MD CHARLOTTE M. CHIONG MD AGNES N. TIRONA-REMULLA MD ABNER L. CHAN MD JEANNETTE MARIE S. MATSUO MD FELIX P. NOLASCO MD

RESIDENTS

LINA ROSE A. ALCANCES MD MOH MICHAEL F. GALICIA MD ERWIN M. ESLAVA MD DESIREE B. VANGUARDIA MD HERBERT Q. GUTIERREZ MD CAMILLE SIDONIE A. ESPINA MD ERIC T. VINCULADO MD MARY APPLE PIE M. GARCIA MD MARIO ADRIAN M. ZAFRA MD FELICIDAD B. MENDOZA MD FORTUNA CORAZON A. ABERIN MD LEI-JOAN V. MOLO MD ARSENIO CLARO A. CABUNGCAL MD IVY D. PATDU MD

RYNER JOSE C. CARRILLO MD FLORENCE YUL N. SAQUIAN MD

PHILIP B. FULLANTE MD JOSEPH ROY VINCENT B. UMALI MD

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UP-PGH DEPARTMENT OF OTORHINOLARYNGOLOGY CLINICAL PRACTICE GUIDELINES

PURPOSE OF THE CLINICAL PRACTICE GUIDELINES The UP-PGH Department of Otorhinolaryngology handles about 45,000 patients a year. It must ermbrace the influx of new concepts, current techniques, diagnostic and therapeutic options. At the same time, it has to struggle to keep a balance between this and the availability of its limited resources. The development of clinical practice guidelines in selected areas is designed to help fill in the gap to maximize patient care in the department. TARGET POPULATION, SETTING AND PROVIDERS OF CARE Charity patients seen by the consultant and resident staff of the UP-PGH Department of Otorhinolaryngology are the target population. The conditions include: (1) chronic suppurative otitis media in adults; (2) nasal polyps in adults; (3) acute and chronic tonsillitis in children and adults; (4) obstructive sleep apnea in children; (5) thyroid masses in adults; (6) cleft lip and palate in children and adolescents; and (7) tracheostomy and decannulation in children and adults. METHODS OF GUIDELINE DEVELOPMENT The UP-PGH Department of Otorhinolaryngology has six subspecialty study groups to which consultants and residents are assigned. Each of the study groups chose one common clinical condition, which they felt needed a protocol for effective and efficient management. They conducted the literature search and developed evidence-based recommendations (EBR). In the case of the Cranio-Maxillofacial, Plastic and Reconstructive Surgery study group, they met with the rest of the CLAP team in the development of the EBR. The consultant and resident staff convened in Caliraya, Laguna on September 2-3, 2003, to deliberate on the EBRs, modify, arrive at a consensus and ratify the CPG. PANEL RECOMMENDATIONS AND LEVELS OF EVIDENCE All literature were classified according to levels of evidence and grades of recommendations based on guidelines from the US Agency for Health Care Policy and Research and are set out as follows:

STATEMENTS OF EVIDENCE GRADES OF RECOMMENDATION

Ia Obtained from meta-analysis of randomized controlled trials

A

Requires at least one randomized controlled trial as part of a body of literature of overall good quality and consistency addressing the specific recommendation

Ib Obtained from at least one randomized controlled trial

IIa Obtained from at least one well-designed controlled study without randomization

B

Requires the availability of well conducted clinical trials but no randomized clinical trials on the topic of recommendation IIb Obtained from at least one other type

of well-designed quasi-experimental study

III Obtained from well-designed non-experimental descriptive studies, such as comparative studies, correlation studies and case studies

IV Obtained from expert committee reports or opinions and/or clinical experience of respected authorities

C

Requires evidence obtained from expert committee reports or opinions and/or clinical experiences of respected authorities. Indicates an absence of directly applicable clinical studies of good quality

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UP-PGH DEPARTMENT OF OTORHINOLARYNGOLOGY CLINICAL PRACTICE GUIDELINES CHRONIC SUPPURATIVE OTITIS MEDIA IN ADULTS SCOPE OF THE PRACTICE GUIDELINE This clinical practice guideline is for use by the Department of Otorhinolaryngology of the College of Medicine - Philippine General Hospital, University of the Philippines Manila. It covers the diagnosis and management of chronic suppurative otitis media in adults (19 years old and above). OBJECTIVES The objectives of the guideline are (1) to emphasize the requisites of diagnosis of chronic suppurative otitis media in adults; (2) to evaluate current diagnostic techniques; and (3) to describe treatment options. LITERATURE SEARCH This guideline is based on the 1997 Clinical Practice Guidelines of the Philippine Society of Otolaryngology– Head and Neck Surgery and revised according to new evidence. The National Library of Medicine’s PubMed database and Cochrane Reviews database were searched for literature using the keyword otitis media, suppurative. The search was limited to articles involving humans and those published in English in the last fifteen years, WHO reports, and the PGH Annual Report. It yielded 549 articles. Thirty-eight (38) abstracts were chosen and results were further assessed for relevance. Full text articles were obtained when possible. The chosen articles were divided as follows:

Meta-analysis 2 Randomized controlled trial 2 Non-randomized controlled study 3 Descriptive study 1 Committee report 1

DEFINITION Chronic suppurative otitis media (CSOM) is a persistent inflammation of the middle ear or mastoid cavity. Synonyms include “chronic otitis media (without effusion)”, “chronic mastoiditis” and “chronic tympanomastoiditis”. Chronic suppurative otitis media is characterized by persistent or recurrent ear discharge (otorrhea) over 3 months through a perforation of the tympanic membrane.

9 Typical findings may include thickened granular middle ear mucosa, mucosal polyps

and cholesteatoma within the middle ear. Chronic suppurative otitis media does not include chronic perforations of the eardrum that are dry, discharge only occasionally, and have no signs of active infection.

2

PREVALENCE Worldwide prevalence of chronic suppurative otitis media is 65-330 million people. Between 39 to 200 million (60%) suffer from significant hearing impairment. Otitis media has been estimated to cost 28,000 deaths and a loss of over 2 million in Disability Adjusted Life Years (DALY) in 2000, 94% of which are in developing countries. Most of these deaths are presumably due to chronic suppurative otitis media, because acute otitis media is a self-limiting infection

2.

In the Philippines, the prevalence of CSOM is estimated at 2.5% to 29.5% based on several surveys among children in Metro Manila and Mindanao.

9 It has been reported that CSOM

patients constitute 14% of outpatient consults at the University of Santo Tomas Hospital 4, and

30% of emergency cases and 60% of operated ears at the PGH 9. The number of referrals

4

(pediatric and adult patients) with diagnosis of CSOM in the ORL-Outpatient Ear Specialty Clinic of the Philippine General Hospital numbers to 325 in 2002. RECOMMENDATIONS ON THE DIAGNOSIS OF CHRONIC SUPPURATIVE OTITIS MEDIA The assessment begins with a thorough history of the frequency, duration, and characteristics of the discharge. Physical examination of the affected ear requires cleansing of the external auditory canal before the tympanic membrane can be accurately assessed. The eardrum must be adequately visualized for accurate diagnosis and treatment.

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1. CSOM is diagnosed by the presence of a tympanic membrane perforation and a history

of persistent or recurrent ear discharge for more than 3 months. Grade C Recommendation

The presence of tympanic membrane perforation and persistent or recurrent otorrhea for more than 3 months is still considered by the panel to be diagnostic of CSOM. (Task Force of the Fourth International Symposium of Otitis Media Florida, June 1987).

4 Critical to this

definition is the history of chronic active otorrhea for more than 3 months. The histopathologic definition of CSOM was not used.

2. Pure tone audiometry and speech testing (PTA-ST) must be performed as part of the

total diagnostic assessment. Grade C Recommendation

The panel recognized the value of the PTA-ST in the initial evaluation of patients with CSOM because it provides information on the etiology of hearing loss (conductive, mixed and sensorineural) in the ipsilateral and contralateral ear. Moreover, it gives baseline data on the pre-operative hearing status of a patient, which is important in surgical planning and in evaluating the effectiveness of tympanoplasty and ossiculoplasty.

3. Radiographic imaging studies, in the form of mastoid radiograph or computed tomography scanning, are considered ancillary diagnostic tools.

Grade B Recommendation

Mastoid radiographs are used to evaluate the degree of pneumatization particularly in surgical ears. Previous studies showed high false negative rates and low sensitivity (54%)

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when mastoid radiographs were correlated with intra-operative findings of cholesteatoma. There is also a marked paucity of studies on the use of mastoid radiographs in our review of literature. High-resolution computed tomography (HRCT) of the temporal bone is not routinely requested but may have a value in (1) children, (2) medically unfit patients, (3) only or better hearing ear, (4) patients in whom the tympanic membrane cannot be adequately visualized, (5) patients who have had previous mastoid surgery, and (6) patients with intra-temporal or intracranial complications.

4, 5, 6 ,7

4. Culture and sensitivity of ear discharge is not part of the routine initial diagnostic

assessment. Grade A Recommendation

In the prospective comparative study of Khanna et al

3 of 110 patients with active CSOM, the

group with culture and sensitivity prior to antibiotic treatment (broad-spectrum antibiotic drops) attained dry ears in 100%, while the group without culture and sensitivity had a cure rate of 74%. Based on these findings, they concluded that there is no definite role of culture

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and sensitivity in the initial management of all cases of CSOM. This is further supported by local studies that show no significant change in the pathogenic organisms in patients with CSOM within the last twenty years. Therefore, patients with CSOM should initially be prescribed a broad-spectrum antibiotic. Only in cases with failure of initial therapy should culture and sensitivity be done.

RECOMMENDATIONS ON THE TREATMENT OF CHRONIC SUPPURATIVE OTITIS MEDIA

1. Aural toilet is an essential part of the treatment of CSOM in all patients. Grade A Recommendation

Ear cleansing, also known as aural toilet, consists of mechanically removing ear discharge and other debris from the ear canal and middle ear by mopping with cotton pledgets, wicking with gauze, flushing with sterile solution, or suctioning. This can be done with an oto-microscope, or under direct vision with adequate illumination of the middle ear.

2

The meta-analysis by Acuin et al

2 showed that aural toilet, especially when combined with

antibiotic treatment is more effective in drying up otorrhea and eradicating middle ear bacteria than no treatment. There was no good evidence of benefit from simple ear cleansing. Thus, the panel agreed that aural toilet should be part of the initial management of CSOM in order (1) to clean the ear canal and middle ear cavity; (2) adequately visualize and assess the middle ear; (3) to allow the topical antibiotic to reach the middle ear cavity; and (4) to provide symptomatic relief for the patient.

2. Topical quinolones, specifically ofloxacin, are recommended for the initial management of CSOM for a period of 10-14 days. For persistent otorrhea, antibiotic therapy for an additional two weeks is recommended.

Grade A Recommendation

Treatment of CSOM with aural toilet and topical antibiotics, particularly quinolones, is effective in resolving otorrhea and eradicating bacteria from the middle ear. Acuin et al

2, in a

systematic review of five trials, reported that topical quinolones are more effective than non-quinolones. Abes et al

1, concluded in their meta-analysis that 0.3% ofloxacin otic solution is

better than other antibiotic otic drops and oral antibiotics in terms of overall cure rate and resolution of secondary outcome parameters. Thus, the topical ofloxacin given for 10-14 days is highly recommended. If there is insufficient symptomatic improvement or treatment failure, topical quinolones can be applied for two more weeks, increasing clinical efficacy without causing safety problems.

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3. Systemic antibiotics may be given for CSOM with associated bacterial upper repiratory

infections and complications. Grade C Recommendation

One systematic review found that systemic antibiotics were significantly less effective than topical antibiotics in reducing otoscopic features of chronic suppurative otitis media.

2 Thus,

topical antibiotics remain to be the mainstay of treatment in CSOM. However, systemic anitibotics may still be given to CSOM patients with associated bacterial upper respiratory tract infections and complications.

4. Surgery must be performed on all cases of CSOM with suppurative complications.

Grade C Recommendation Panel members agreed that the presence of intracranial and extracranial complications in patients with CSOM is an absolute indication for mastoidectomy based on pathophysiologic

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understanding of the disease and numerous case series.2

These complications include brain abscess, meningitis, otitic hydrocephalus, lateral sinus thrombophlebitis, facial nerve paralysis, labyrinthitis, and subperiosteal abscesses.

5. Surgery may be performed for those who fail to respond to adequate medical treatment. Grade C Recommendation

There are no randomized clinical trials to date comparing medical treatment and mastoidectomy in those patients in whom either procedure is a valid alternative. However, case series describing the intra-operative findings of medically intractable cases have been published.

9 The indications for abandoning medical therapy are currently unclear; thus, the

panel saw no justification in making definite recommendations for the performance of either procedure.

REFERENCES

1. Abes G, Espallardo N, Tong M, Subramaniam KN, Hermani B, Lasiminigrum L, Anggraeni R. “A Systematic Review Of The Effectiveness of Ofloxacin Otic Solution For The Treatment Of Suppurative Otitis Media.” J ORL & Health Specialties; Mar-Apr 2003.

2. Acuin J, Smith A, Mackenzie I. “Interventions For Chronic Suppurative Otitis Media.” Cochrane Database Syst Rev. 2000; (2): CD000473. 3. Khanna, V., Chander J. Nagarkar NM, Dass A. “ Clinicomicrobiologic evaluation of active tubotympanic type of chronic suppurative otitis media.” J. Otolaryngol. 2000 June; 29(3):148-53. 4. Leighton SE, Robson AK, Anslov P., Melford CA, “The Role of CT Imaging in the Management of CSOM. Clin. Otolaryngol. 1993 Feb; 18(1):23-9. 5. O ‘Reilly BJ, et al. “The Value of CT Scanning in Chronic Suppurative Otitis Media.” J. Laryngol. Otol. 1991 Dec; 105(12):990-4. 6. Ramsey AM. “Diagnosis and Treatment of the Child with a Draining Ear” J. Pediatr. Health Care. 2002 Jul-Aug; 16(4) :161-9. (abstract) 7. Suzuki K, Nishimura T, Baba S, Yanagita N, Ishigami H. “Topical Ofloxacin For Chronic Suppurative Otitis Media And Acute Exacerbation Of Chronic Otitis Media: Optimum Duration Of Treatment.” Otol Neurotol. 2003 May; 24(3): 447-52. 8. 2002 Annual Report, Out-patient Department, Philippine General Hospital. 9. Clinical Practice Guidelines 1997, PSO-HNS.

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Y

N

Y

N

Y

N

Y

N

N

Y

Persistent / Recurrent EAR DISCHARGE > 3 mos in an adult

OTOSCOPY and other relevant ORL exams

Aural Toilet

TM Perforation? Appropriate Management

DIAGNOSIS OF CHRONIC SUPPURATIVE OTITIS MEDIA

PTA-ST

With Cholesteatoma and/or Complications?

Appropriate Management

Resolution of Discharge?

OBSERVE

GS/CS, AFB, Fungal Studies

APPROPRIATE MANAGEMENT

Resolution of Discharge?

OBSERVE

Continue TOPICAL OFLOXACIN x 2 wks

Resolution of Discharge? OBSERVE

Consider common pathogens e.g. Pseudomonas, Staphylococcus aureus, Proteus mirabilis

Consider other microbial pathogens

TOPICAL OFLOXACIN x 10-14 days

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UP-PGH DEPARTMENTOF OTORHINOLARYNGOLOGY CLINICAL PRACTICE GUIDELINES MEDICAL MANAGEMENT OF NASAL POLYPS IN ADULTS

SCOPE OF THE PRACTICE GUIDELINE This clinical practice guideline is for use by the Department of Otorhinolaryngology of the College of Medicine - Philippine General Hospital, University of the Philippines Manila. It covers the diagnosis and medical treatment of nasal polyps in adults. OBJECTIVES The objectives of the guideline are (1) to evaluate current diagnostic techniques; and (2) to present steroid treatment options. LITERATURE SEARCH This guideline is based on the 1998 Clinical Practice Guidelines Consensus Report of the Philippine Society of Otorhinolaryngology- Head and Neck Surgery and revised according to evidence. The Medline and PubMed were searched for literature using the following keywords nasal polyposis, treatment, steroids, nasal spray. The search was limited to articles involving humans and published in English. It yielded 21articles. Relevant full text articles were obtained and assessed as follows:

Meta-analysis 0 Randomized controlled trial 4 Non-randomized controlled study 2

Descriptive study 4 Committee report 4 DEFINITION Nasal polyp is a smooth, gelatinous, semi-translucent and pale white mass arising from the mucosa surrounding the ostiomeatal complex. This was adapted from the 1998 PSO-HNS consensus report on nasal polyps. PREVALENCE Nasal polyps occur in less than 5% of the general population.

27,31 It has also been estimated that

it is the diagnosis made in 1 out of 20 referrals to otolaryngologists.31

In the PGH Department of ORL-Outpatient Clinic, the prevalence of diagnosed cases with nasal polyps for 2002 is approximately 2.1% (Unpublished PGH-OPD Annual Report, 2002). One percent to 5% of asthmatics and allergic rhinitis sufferers have nasal polyps. It is more common in the older age group (12.4% in those above 40 years old and 3.15% in those below 40 years old.) Among children, it is even lower at 0.1%. There is no sex predilection. Nasal polyps are often associated with other conditions

1.

9

RECOMMENDATIONS ON THE DIAGNOSIS OF NASAL POLYPS 1. Patients with nasal polyps present with common and less common signs and

symptoms. Some patients may be asymptomatic. Grade C Recommendation The following signs and symptoms are found among patients with nasal polyps

30, 17

COMMON LESS COMMON

Nasal obstruction Epistaxis

Rhinorrhea Cough

Smell disturbances Hyponasal speech

Sneezing Mouth breathing

Headache / facial pain Halitosis

2. Anterior and posterior rhinoscopy should be done to visualize the nasal polyps. Grade C Recommendation Nasal polyps and normal nasal turbinate can be differentiated as shown below:

NASAL POLYP NASAL TURBINATE

Location Usually at the osteomeatal area

Along entire lateral nasal wall

Moves on probing Yes No

Pain on probing No Yes

Shrinks with decongestion

No Yes

Bleeds with manipulation Yes / No Yes

A nasal polyp and polypoid nasal mucosa may be difficult to differentiate clinically but a peduncle seen on rhinoscopy would indicate a polyp. Massive nasal polyps may produce nasal deformity.

3. Endoscopy can detect small polyps, which may not be seen on anterior rhinoscopy. Grade C Recommendation It can also assess the extent of the disease posterior and superiorly in Grade 1 and 2 nasal polyps.

4. The classification of nasal polyps, based on size and extent, is used as a guide in the

management. Grade C Recommendation

The endoscopic grading proposed by Mackay and Lund was adopted by the panel.

27, 28

Grade 0 – absence of polyps

Grade I (mild) – small polyps not reaching the lower edge of the middle turbinate Grade 2 (moderate) – medium-sized polyps extending between the upper and lower edges of the inferior turbinate Grade 3 (severe) – large polyps extending below the lower edge of the inferior

turbinate

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5. Plain sinus x-rays are of limited value in the determination of the actual extent of the nasal polyposis. Grade C Recommendation

Plain sinus x-rays may be helpful to detect the presence of chronic sinusitis and to demonstrate bone erosion in massive polyposis. Bone erosions may indicate malignancy or Woakes syndrome (aggressive recurrent sinonasal polyposis, erosion of ethmoid bones and other sinuses, facial malformation).

31

6. The use of computed tomography of the paranasal sinuses is highly recommended for

medical treatment failures, complications of associated sinus disease and candidates for surgery. Grade C Recommendation

The recommended cuts for the CT scan are (1) screening CT scan and (2) coronal and axial cuts for cases of recurrent nasal polyps.

RECOMMENDATIONS ON THE MEDICAL MANAGEMENT OF NASAL POLYPS

1. The management of Grade 1 and 2 nasal polyps is primarily medical. Grade A Recommendation

The goals of medical management of nasal polyps are (1) to eliminate rhinitis symptoms, (2) to restore the nasal airway, (3) to completely remove or reduce the size of nasal polyps, (4) to improve the sense of smell, and (5) to prevent recurrence in post-operative patients.

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Symptomatic relief of the patient is of utmost priority. After adequate medical treatment, a patient who is symptom-free without an increase in the size of the polyp can be observed. Concurrent disease (e.g. sinusitis, asthma, allergic rhinitis) should be addressed concomitantly. The concept of “medical polypectomy” with increased reliance on steroids (over surgery) has to be adapted to the patient population. Financial capability is a primary concern for PGH patients and a senior consultant expressed that prolonged costly topical steroid treatment requiring regular follow-up may be deemed “expensive” and difficult along with deterioration of skills in polyp and nasal surgery.

2. Grade 1 nasal polyps are managed with intranasal corticosteroid for 4 to 6 weeks. If

there is positive response to treatment, it is continued on a maintenance basis. If there is no response to treatment, CT scan is recommended and the patient is re-evaluated.

Grade A Recommendation Long-term topical steroids have shown significant results in at least 13 controlled trials. Reduction of rhinitis symptoms, nasal polyp size and recurrence rate have been demonstrated. Nasal breathing also improved. There is negligible effect on the sense of smell.

31 The following topical steroid preparations may be used:

Budesonide aqueous or powder 400-800 ug/day 26, 31

Fluticasone proprionate aqueous 400-800 ug/day

19, 27, 29

Mometasone 100 ug/day

The advantage of topical steroids is that they can be administered for long periods of time without major side effects. The disadvantages of topical steroids are unequal distribution in the nasal mucosa due to infection or large polyps, and insufficient delivery due to structural abnormalities.

2. Grade 2 nasal polyps are managed with short-term systemic steroid and intranasal

steroid. They may be given concurrently or sequentially.

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There are no studies yet showing the advantage of concurrent versus sequential steroid therapy. 3.1 Concurrent treatment with short-term systemic steroids and intranasal steroids

Grade B Recommendation Dual steroid therapy (topical and oral) based on the study by Bonfils

5 showed that a

combined protocol gives better results than intranasal steroid therapy alone. Short-term treatment courses of systemic steroid combined with long-term steroid intranasal spray led to satisfactory results in 85% of the patients, and the mean clinical severity index was reduced by 77.7%

5. A combined short-term steroid therapy is highly effective in chronic

polypoid rhinosinusitis.7

3.2 Sequential treatment – short-term systemic steroids followed by intranasal steroids Grade C Recommendation

Slavin

32 asserted that the reasonable approach to nasal polyposis should be based on

the oral prednisolone administration over a 10- to 14-day period followed by a course of intranasal spray of either beclomethasone or flunisolide.

Short-term oral steroids have an effect on all types of symptoms and pathology, including the sense of smell.

3 Oral steroids recommended are the following:

Dexamethasone 12 mg to 3 mg within 9 days 27

Methylprednisolone 64 mg to 10 mg within 10 days 31

Prednisone 1 mg/kg for 10 days 32

or 1mg/kg for 5 days 5

The advantages of oral steroids include (1) equal distribution; (2) more dramatic decrease in the size of polyps

26; and (3) significant early relief of symptoms.The disadvantage of oral

steroids is the occurrence of many major side effects. Presence of osteoporosis, gastrointestinal disorders, severe hypertension, diabetes, herpetic keratosis and tuberculosis contraindicate the use of oral steroids.

4. There is divergence in the management of Grade 3 polyps. Steroid treatment is usually

given initially. It is also where the surgical option is traditionally given the primacy. Grade C Recommendation

In grade 3 polyps, many clinicians would commence with systemic corticosteroids and continue with intranasal corticosteroids to maintain improvement.

28,30 Research will be

undertaken in the department to ascertain its cost-effectiveness for PGH patients. However, it is recommended if patients cannot be cleared for surgery.

3. Response to medical treatment is graded accordingly: 0 – no response 1 – equivocal 2 – significant improvement

Grade C Recommendation

No improvement of symptoms after 2 to 3 months of treatment and increase in the size of polyps despite adequate medical treatment are considered treatment failures.

4. Topical steroid given after polypectomy reduces the number of recurrences. Grade B Recommendation Fluticasone and budesonide used for 6 to 12 months decreased the recurrence rate

18. These

medications are especially valuable in patients who have previously been subjected to frequent polypectomies.

31

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5. Adjunctive treatment is given depending on the patient’s concomitant problems. Grade C Recommendation Adjunctive treatment include the following:

1. Nasal douche – to clean out discharge prior to application of intranasal steroids 2. Nasal decongestants – for “priming” 3. Antibiotics – to treat rhinosinusitis 4. Antihistamines – to treat allergic rhinitis 5. Proton pump inhibitors (PPI) and H2-blockers – prophylaxis for steroid-induced

gastritis 6. Office polypectomy

REFERENCES 1. Batsakis J, Sniege N. Choanal and Angiomatous Polyps of the Sinonasal Tract. Annals

of Otology Rhinology and Laryngology. 1992. 101:623-625. 2. Batsakis J. Pathology Consultation: Stromal Cells Atypia in Sinonasal Polyposis. Annals

of Otology Rhinology and Laryngology. 1986. 95:321-322. 3. Berg.O. Origin of the Choanal Polyp. Archives of Otolaryngology and Head Neck

Surgery. 1988; 114:1270-1271. 4. Biewenga J/ Albumin and immunoglobulin level in nasal secrections of patients with

nasal polyps treated with endoscopic sinus surgery and topical corticosteroids. Journal of Allergy and Clinical Immunology. 1995; 96: 334-340.

5. Bonfils P, Nores J, Halimi P, Avan P. Corticosteroid Treatment in Nasal Polyposis with a Three-Year Follow-up Period. Laryngoscope. April 2003; 113:683-687.

6. Coste A, et al. Increased epithelial cell proliferation in nasal polyps. Archives of Otolaryngology and Head Neck Surgery. 1996; 122:432-436.

7. Damm, M., Jungehulsing, M., Eckel, H., Schmidt, M., Theissen, P. Effects of systemic steroid treatment in chronic polypoid rhinosinusitis evaluated with magnetic resonance imaging. Otolaryngology-Head and Neck Surgery. April 1999.pp 517-523

8. De la Cruz-Mera A, et al. Premalignant changes in nasal and sinus polyps: a retrospective 10 year study (1976-1988). The Journal of Laryngology and Otology. 1990; 104: 210-212.

9. Dowell M, Pahor A. Nasal polypectomy: should antrral washout be a routine? The Journal of Laryngology and Otology. 1992;106:695-696.

10. Drake-Lee A, et al. The effects of different fixation on the distribution and numbers of mast cell in patients with nasal polyps. The Journal of Laryngology and Otology. 1988; 102:1099-1101.

11. Drake-Lee A, McLaughlan P. The Release of histamine from nasal polyp tissue and peripheral blood when challenged with antihuman IgE, house dust mite extract and mixed grass pollen extract and compared with positive skin tests. The Journal of Laryngology and Otology. 1988; 102:886-889.

12. Drake-Lee A. Nasal polyps in identical twins. The Journal of Laryngology and Otology. 1992; 106:1084-1085.

13. Dunnete S, et al. Microbiologic analysis of nasal polyp tissue. Journal of Allergy and Clinical Immunology. 1986: 78: 102-108.

14. El-Guindy A, Mousour MH. The role of transcanine surgery in antrochoanal polyp. The Journal of Laryngology and Otology. 108: 1055-1057.

15. Gilbert Jg. Aaantroscopy in maxillary sinus disease associated with nasal polyposis. The Journal of Laryngology and Otology 1989. 103:861-863.

16. Hasegawa M, Nasu M, Ohki M, Sugiuchi Y, Watanabe I. Malignant transformation of Nasal polyp. Arch Otolaryngol Head Neck Surg. 1988;114:336-337.

17. Holmberg K, Karlsson G. Nasal Polyps: medical or surgical management? Clinical and Experimental Allergy. 1996; 26(3):23-30.

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18. Holmberg K, Juliusson S, Balder B, Smith D, Richard D, Karlsson G. Fluticasone propionate aqueous nasal spray in the treatment of nasal Polyposis. Annals of Allergy, Asthma and Immunology. 1997; 78:270-6.

19. Jacobs R, Freda A, Culver W. Primary nasal Polyposis. Annals of Allergy. 1983;51:500-505.

20. Jamal A, Maran A. Atopy and nasal Polyposis. The Journal of Laryngology and Otology.

1987; 101:355-358. 21. Jang Y, et al. Immunohistochemical characteristics of cultured nasal polyp epithelial

cells: expression of Cytokeratin, vimentin and proliferating cell nuclear antigen. Journal of Rhinology. 1996; 3(2): 151-156.

22. Jin H. Angiomatous nasal polyp presenting recurrent epistaxis. Journal of Rhinology. 1996; 3(2) 174-176.

23. Ki Hwan Hong, Sam Hyun Kwon, Sang Sool Jung. The assessment of Nasality with nasometer and sound spectography in patients with nasal Polyposis. Otolaryngology- Head and Neck Surgery. 1997; 117:4

24. Kim Y, Denburg Ja. Stem cell factor in nasal polyposis: increased expression by structural cells is suppressed by topical corticosteroids. Journal of Rhinology. 1996; 3(2) 157-165.

25. Kingdom T. Clinical characteristics and genotype analysis of patients with cystic fibrosis and nasal polyposis requiring surgery. Archives of Otorlayngology and Head Neck surgery. 1996; 122: 1209-1213.

26. Lildholdt T, Rundcrantz H, Bende M, Larsen K. Glucocorticoid Treatment for Nasal Polyps: the use of topical budesonide powder, intramuscular betamethanosone, and surgical treatment. Arch Otolaryngol Head Neck Surg. 1997; 123: 595-600.

27. Lund V. Diagnosis and treatment of nasal polyps. British Medical Journal. 1995; 311: 1411-1414.

28. Mackay IS; Lund VJ. Imaging and Staging. In: Mygind N, Lindholdt, Y. ed. Nasal Polyposis: An Inflammatory Disease and its Treatment. Copenhage: Munksgaard, 1997: 137-144.

29. Mostafa, B. Fluticasone Proprionate is associated with severe infection after endoscopic polypectomy. Arch Otolaryngol Head Neck Surg. 1996; 122:729-731.

30.

Naclerio R, MacKay I. Guidelines for the Management of Nasal Polyps. In: Nasal Polyposis: An Inflammatory Disease and Its Treatment. (Eds. Mygind M, LildholtT). Munksgaard, Copenhagen, pp 177-180, 1997.

31. PSO-HNS consensus report on Nasal Polyps, 1998 32. Slavin, Raymond G. Medical Management of Nasal polyps and Sinusitis. Journal of

Allergy and Clinical Immunology

14

ALGORITHM FOR THE MEDICAL MANAGEMENT OF NASAL POLYPOSIS

OR N Y(2) Y(1) Y N Y

History and Physical Exam

Diagnostics (Anterior and Posterior Rhinoscopy/Endoscopy

Grading of polyps Grade 1

Intranasal Steroid Spray 4-6 weeks

Response? (Grade 1 or 2 response)

Continue use of topical intranasal steroids; Observe every 2-3 months

Management of unresponsive Grade 1 polyp

Systemic corticosteroids for 1-2 weeks + nasal steroid spray

Continue topical intranasal steroids; Follow up every month for 6 months; Follow up regularly every 2-3 months thereafter

Response?

Grade 2 & 3

Management of

Grade 3 polyps

Go to Mgt of Grade 2 polyps

CT scan highly recommended & re-evaluation & pre-op preparation

Consider Surgery (ESS/PEA)

Medical Management of Grade 2 polyps

SEQUENTIAL Systemic corticosteroids 1-2 weeks

CONCURRENT Systemic corticosteroids 1-2 weeks + Intranasal steroids x 4-6 weeks

Followed 4-6 weeks nasal steroid spray

Response? 0,1,2

CT scan highly recommended & re-evaluation & pre-op preparation

Ffup q1 mo. for 6 mos; encourage use of intranasal steroids; ffup regularly q2-3 mos thereafter

Continue intranasal steroids, may give systemic steroids in 3-4 cycles per year (at least 3 mo-interval per cycle

Response grading 0 No response 1 Equivocal 2 Significant improvement

NOTE: FOR RECURRENT NASAL POLYPS

Grade 1 – proceed to management of grade 1 polyps Grade 2-3 – proceed to management of grade 2&3 polyps (may repeat for 3-4 cycles per year)

15

UP-PGH DEPARTMENT OF OTORHINOLARYNGOLOGY CLINICAL PRACTICE GUIDELINES ACUTE AND CHRONIC TONSILLITIS IN CHILDREN SCOPE OF THE PRACTICE GUIDELINE This clinical practice guideline is for use by the Department of Otorhinolaryngology of the College of Medicine - Philippine General Hospital, University of the Philippines Manila. It covers the diagnosis and management of tonsillitis in otherwise healthy children, older than 1 year of age. OBJECTIVES The objectives of the guideline are: (1) to heighten the recognition of tonsillitis, particularly due to Group A Beta-hemolytic Streptococcus, to avoid delay and serious sequelae; (2) to evaluate current diagnostic techniques; and (3) to describe treatment options. LITERATURE SEARCH This guideline is based on the 1997 Clinical Practice Guidelines of the Philippine Society of Otorhinolaryngology – Head and Neck Surgery and revised according to new evidence. The National Library of Medicine’s PubMed database was searched for literature using the keyword tonsillitis. The search was limited to articles involving humans and those published in English in the last ten years. It yielded 919 articles, the titles of which were carefully screened for possible relevance to the guideline. Thirty-seven (37) abstracts were chosen and results were synthesized. Full text articles were obtained when possible. In addition, several current guidelines on sore throat / pharyngitis and indications for tonsillectomy were included after evaluation of the references on which they were based. The chosen articles were divided as follows:

Meta-analysis 6 Randomized controlled trial 8 Non-randomized controlled study 3 Descriptive study 15 Committee report 5 DEFINITION Acute tonsillitis is defined as the presence of erythematous and/or exudative tonsils with any one of the following symptoms: sore throat, dysphagia, odynophagia, fever and accompanying tender enlarged cervical lymph nodes.

8

Chronic tonsillitis is defined as tonsillar inflammation resulting from recurrent clinically documented acute attacks of tonsillitis occurring at least 5 times per year.

8

These definitions were adapted from the 1997 CPG of the PSO-HNS. However, the frequency was increased from 3-4 times a year to at least 5 times a year based on a study by Paradise

25which indicated that present guidelines for tonsillectomy are not sufficiently stringent.

These patients are usually candidates for surgery, but the modest benefit offered by tonsillectomy to these moderately affected patients is outweighed by the risks and cost of the operation.

25

PREVALENCE The major issue in most cases of acute tonsillitis is whether it is associated with Group A Beta-hemolytic Streptococci (GABHS) or to other self-limited etiologies of sore throat, such as a viral infection. Only about 20% of cases of pharyngitis and tonsillitis are caused by GABHS, but its associated risks of subsequent acute rheumatic fever and/or acute glomerulonephritis remain a cause for concern.

24 The incidence of rheumatic fever remains high at 100-200 cases per

100,000 children in developing countries in contrast with the 0.5 cases per 100,000 children in industrialized countries. The risk of developing rheumatic fever following untreated tonsillopharyngitis is 1% in the civilian population.

3

16

In 2002, there were 142 cases of acute and chronic tonsillitis seen at the ORL outpatient clinic of the Philippine General Hospital, with 94 tonsillectomies performed during the same time period. At the Department of Pediatrics, there were 178 patients with rheumatic fever and 608 patients with rheumatic heart disease for the year 2002.

1

RECOMMENDATIONS ON THE DIAGNOSIS OF ACUTE AND CHRONIC TONSILLITIS

1. The diagnosis of acute tonsillitis may be made clinically. Grade B Recommendation

The symptoms include sore throat (usually lasting more than three days), dysphagia, odynophagia, anorexia, lethargy and systemic illness. Abnormal physical signs are erythematous tonsils or pharynx, purulent exudates on tonsils, fever and tender cervical lymphadenopathy.

14, 15, 20, 21

History must ensure whether the above symptom complex occurs at least five times in a year to determine whether the patient has acute or chronic tonsillitis.

2. The diagnosis of GABHS tonsillitis should be suspected on clinical and

epidemiological grounds. Grade B Recommendation

The signs and symptoms of both bacterial and viral infection overlap broadly, but the diagnosis of GABHS infection should be suspected based on epidemiologic and clinical characteristics.

3, 21 Epidemiological features suggestive of GABHS are (1) age 5-15

years; (2) recent close contact with a documented case; and (3) known high prevalence of GABHS in the community. Pertinent clinical findings include sudden onset sore throat, odynophagia, dysphagia, fever, vomiting, pharyngeal exudates, scarlatiniform rash and tender anterior cervical lymphadenopathy. Watery eyes, rhinitis, cough and hoarseness are negative predictors. In the United States, where rheumatic fever and rheumatic heart disease are still reported, clinical diagnosis is still valuable. The Scottish Intercollegiate Guideline Network does not find the diagnosis of GABHS based on clinical grounds valid or useful, but this may be due to the rarity of the complications.

20

3. Laboratory testing is recommended for patients who are suspected to have

GABHS infection based on clinical and epidemiological grounds. Positive throat cultures and rapid antigen tests will confirm GABHS infection.

Grade C Recommendation

There is controversy regarding the use of throat culture and rapid antigen tests in the diagnosis of GABHS infection. Studies from the United Kingdom

20 show that these tests

are neither sensitive nor specific for serologically confirmed infection. These considerably increase costs and alter few management decisions.

20 On the other hand,

studies from the United States21

demonstrate that throat culture is still the gold standard for confirmation of the diagnosis of GABHS pharyngitis, with a sensitivity of 90% to 95%. Rapid antigen tests have more then 95% specificity, but only 80% to 90% sensitivity when compared to culture.

21 However, the value of early diagnosis in the minority of

cases when streptococcus is present should be weighed against the higher cost incurred in testing the majority of cases seen. Selective use of diagnostic studies is suggested.

17

RECOMMENDATIONS ON THE MANAGEMENT OF ACUTE AND CHRONIC TONSILLITIS

1. Antimicrobial therapy is reserved for symptomatic patients with positive throat cultures or positive rapid antigen testing. Grade B Recommendation

The benefits from antibiotic therapy include early resolution of symptoms (if due to bacterial pathogens) and prevention of cross-infection, rheumatic fever and glomerulonephritis. However, not all cases of tonsillitis will require the use of antibiotics, as in self-limiting viral infections. Antibiotics must be used rationally to preserve the normal flora in the aerodigestive tract, prevent development of resistance in pathogenic organisms and decrease treatment costs.

2, 21

Several strategies may be employed which strike a balance between overuse and unreasonable withholding of antibiotics. One may opt not to treat patients unlikely to have GABHS pharyngitis and treat only those strongly suspected of having GABHS wherein the diagnosis must be confirmed with laboratory tests. Antibiotics may be started while awaiting results, but discontinued if results are negative.

2. Penicillin is the drug of choice for compliant patients with GABHS infection.

Grade A Recommendation

Patients with acute GABHS tonsillitis should receive therapy with an antimicrobial agent in a dosage and for a duration that is likely to eradicate the organism from the pharynx. Penicillin has a narrow spectrum of activity, which includes GABHS, has infrequent adverse reactions and is not costly.

21 Its bacteriological eradication rate ranges from

84% to 92%, with a clinical cure rate of 96%, 23, 36

despite being given two to four times a day for ten days.

19 Alternative antibiotics with less side effects, infrequent dosing,

palatable, and efficacious with short-course therapy lead to better outcomes due to good compliance. Amoxicillin has been shown to be as effective as penicillin, with the advantage of better palatability.

21 Its bacteriological eradication rate is 86%, with a

clinical cure of 97%.16

Cefuroxime, azithromycin and clarithromycin has been shown to have bacterial (86%, 98%, 88%) and clinical (95%, unknown, 98%) cure rates comparable with penicillin.

16, 23, 31, 36 Erythromycin, which has been the drug of choice for

patients with penicillin allergy, has a lower bacterial eradication rate of 74% .5, 21

3. Supportive therapy is recommended for all patients with tonsillitis. Grade C Recommendation

Adequate supportive care should be part of the routine management of acute tonsillitis. For those with viral infection, this may be all that is necessary. These include sufficient fluid intake, bed rest, warm saline gargle and oral hygiene. The use of antipyretics for fever as well as analgesics, oral anesthetics and antiseptics for patients with marked throat pain may be recommended.

These are recommendations from the 1997 PSO-HNS CPG, based on strong panel consensus and expert opinion. They are further supported by similar recommendations from other guidelines

8, 20.

18

4. The absolute indications for tonsillectomy are the following: 4.1 Adenotonsillar hyperplasia with obstructive sleep apnea, failure to thrive or abnormal dentofacial growth

4.2 Suspicion of malignancy 4.3 Hemorrhagic tonsils Grade C Recommendation

5. The relative indications for tonsillectomy are the following: 5.1 Adenotonsillar hyperplasia with upper airway obstruction, dysphagia, speech impairment or halitosis 5.2 Recurrent or chronic tonsillitis 5.3 Peritonsillar abscess 5.4 Streptococcal carriage Grade C Recommendation

6. Patients with recurrent or chronic tonsillitis who meet all of the following criteria

are recommended for surgery: 6.1 Sore throats are due to tonsillitis. 6.2 Five or more episodes of tonsillitis per year 6.3 Symptoms for at least a year 6.4 Episodes of sore throat are disabling and prevent normal functioning. Grade A Recommendation The literature on surgery for tonsillitis is scanty; and results of the better-quality studies contradict each other. Most articles refer to a pediatric population and confuse the issue with the addition of adenoidectomy. Present guidelines from the American Academy of Otolaryngology require a minimum of 3 episodes of tonsillitis in a year before surgery is contemplated. However, Paradise found that this criterion is not stringent enough and that the modest benefit offered by tonsillectomy to these moderately affected patients are outweighed by the risks and cost of the operation.

25 The small amount of literature about

tonsillitis in adults suggests that tonsillectomy may be beneficial in terms of decreasing time off from work and minimizing costs from repeated consults and use of antibiotics.

19

REFERENCES 1. Annual Report for Rheumatic Fever/Rheumatic Heart Disease, Section of Pediatric Cardiology,

Department of Pediatrics, Philippine General Hospital. 2. Abdul-Baqi et al. Effectiveness of Treatment of Tonsillopharyngitis: Comparative Study.

Journal of Laryngology and Otology 2002 Nov: 116 (11): 917-9. 3. Bassili A. et al Identification of Clinical Criteria for Group A Beta- Hemolytic Streptococcal pharyngitis in children living in a Rheumatic Fever Area. Journal of Tropical Pediatics 2002 Oct; 48 (5) ; 285-93. 4. Brook I, Shah K. Bacteriology of Adenoids and Tonsils in Children with recurrent Adenotonsillitis. Ann Otol Rhinol Laryngol. 2001 Sep; 110(9); 844-8. 5. Brook I et al. Open label, Parallel-group, Multicenter, Randomized Study of Cefrozil versus erythromycin in Children with Group A Streptococcal Pharyngitis/Tonsillitis. Clin Ther 2001 Nov; 23(11); 1889-900 6. Burton MJ et al. Tonsillectomy versus Non-surgical Treatment for Chronic/Recurrent Acute Tonsillitis. Cochrane Database Syst Rev. 200;(2): CD001802 7. Cohen R et al. Comparison of Two Dosages of Azithromycin for 3 days versus Penicillin V for 10 days in Acute Group A Streptococcal Tonsillopharyngitis 8. Clinical Practice Guidelines on Acute and Chronic Tonsillitis, Philippine Society of

Otolaryngology – Head and Neck Surgery, 1997. 9. Darrow DH, Siemen’s C. Indication for Tonsillectomy and Adenoidectomy. Laryngoscope 2002 Aug; 112 (8 Pt 2) : 6-10 10. Deutsch ES. Tonsillectomy and Adenoidectomy: Changing indications. Ped Clin of North Am

1996 Dec; 43 (6) 11. Discolo CM et al Infective Indications for Tonsillectomy. Pediatr Clin North AM. 2003 Apr 50(2); 445-59 12. Donner Banzhoff et al Clinical Findings in Patients Presenting with sore throat. A Study in Inter-observer Reliability. Fam Pract. 2002 Oct; 19(5); 466-8 13. Gaffney RJ et al Bacteriology of tonsil and adenoid and sampling techniques of adenoidal bacteriology. Respir Med 1993 May; 87 (4); 303-9 14. Hossain P et al Clinical Features of District Hospital Paediatric Patients with Pharyngeal Group A Streptococcal. Scand J Infect Dis 2003;35(1); 77-9 15. Johnson BC Alvi A Cost- Effective Work-up for Tonsillitis. Testing, Treatment and Potential Complication. Post-grad Med. 2003 Mar;113 (3); 115-8 16. Kearsley NL et al Comparison of clarithromycin Suspension and Amoxycillin Syrup for the Treatment of Children with Pharyngitis and/or Tonsillitis. BR J Clin Pract 1997 Apr- May; 51 (3); 133-7 17. Kindo AJ et al Role of Surface swab, Core swab and Fine Needle Aspiration in isolating the core bacteria in inflamed tonsils. Indian J Pathol Microbiol 2001 Jul;44(3);293-5 18. Kurien M et al Throat Swab in the Chronic Tonsillitis: How reliable and valid is it?. Singapore Med J 200 Jul; 41 (7): 324-6 19. Lan AJ et al The impact of dosing frequency on the efficacy of 10-day Penicillin or Amoxycillin therapy for Streptococcal tonsillopharyngitis; A Meta-analysis Pediatrics 2000 Feb; 105 (2): E19 20. Management of Sore Throat and Indications for Tonsillectomy, Scottish Intercollegiate

Guidelines Network January 1999. 21. National Guideline Clearinghouse (NGC). Guideline Synthesis: Pharyngitis/Sore Throat. In:

National Guideline Clearinghouse [website]. Rockville (MD): 1999 Oct 6 (revised 2002 Aug 19).

22. Nerbrand C et al Are current rapid detection tests for Group A Streptococci sensitive enough? Evaluation of 2 Commercial Kits. Scand j Infect Dis 2002; 34(11):797-9 23. O Doherty B Azithromycin versus Penicillin V in the treatment of Pediatrics patients with Acute Streptococcal Pharyngitis/Tonsillitis Pediatrics Azithromycin Study Group .Eur J Clin Microbiol Infect Dis 1996 Sep; 15(9); 718-24 24. Olivier C Rheumatic Fever. Is it still a problem? J Antimicrob Chemother 2000

20

Feb; 45 Suppl: 13-21 25. Paradise JL et al Tonsillectomy and adenoidectomy for recurrent throat infection in

moderately affected children. Pediatrics 2002 July; 110 (1): 7-15. 26. Paulussen C et al Adenoid & Tonsils, Indication for surgery and immunological consequences of Surgery. Acta Otorhinolaryngol Belg 2000; 54(3) : 403-8 27. Pichichero ME Evaluating the needs, timing and best choice of antibiotics therapy of Acute Otitis Media and Tonsillopharyngitis infections in children. Pediatr Infect Dis J. 2000 Dec 19 (12 Suppl): S131-40 28. Pichichero ME et al. Recurrent Group A Streptococcal Tonsillopharyngitis Pediatr Infect Dis J 1998 Sep; 17(9): 809-15 29. Pichichero ME: Sore throat after sore throat after sore throat. Are you asking the critical question? Postgrad Med 1997 Jan; 101(1): 205-6 30. Prim et al Spontaneous Resolution of recurrent tonsillitis in Pediatric Patients on the Surgical Waiting lists. Int. J Pediatr Otorhinolaryngol 2002 Aug 1;65 (1): 35-8 31. Quinn J et al Efficacy & Tolerability of 5-day once daily Telithromycin compared with 10-day twice daily clarithromycin for the treatment of Group A Beta- hemolytic Streptococcal Tonsillitis/Pharyngitis a multicenter, randomized, double blind parallel group study. Clin Ther 2003 Feb;25 (2): 422-43 32. Raut VV. Management of peritonsillitis/peritonsillar abscess. Rev Laryngol Otol Rhinol (Bord) 2000;121(2):107-10 33. Raut VV, Yung MW Peritonsillar Abscess: the rationale for interval tonsillectomy. Ear Nose Throat J. 2000 Mar; 79(3) : 206-9 34. Robinson AC et al Throat Swab in chronic tonsillitis: a time-honoured practice best forgotten Br J Clin Pract. 1997 Apr-May; 51(3): 138-9 35. Sun J et al Evaluation of the etiologic agents for acute suppurative tonsillitis in children. Zhonghua Yi Xue Za Zhi (Taipei). 2002 May; 65(5): 212-7 36. Uysal S et al A comparison of the efficacy of cefuroxime axetil and intramuscular benzathine penicillin for treating streptococcal tonsillopharyngitis Trop Paediatr 2000 Sep;20(3): 199-202 37. Woolford TJ et al Spontaneous resolution of tonsillitis in children on the waiting list for tonsillectomy. Clin Otolaryngol. 2000 Oct; 25(5): 428-30.

21

No No

Yes Yes No Yes No No Yes Yes No Yes

Child with signs and symptoms of

tonsillitis

5 or more times a year?

Chronic Tonsillitis

Acute

Tonsillitis

Appropriate antibiotics

Supportive management

Consider tonsillectomy

Revise antibiotics

Bacterial? Supportive management

GABHS?

Appropriate antibiotics

Supportive management

Resolution? Throat Swab

Consider Tonsillectomy

Confirms GABHS?

Resolution?

Consider Tonsillectomy

Reassess

Reassess

Appropriate antibiotics

Supportive management

22

UP-PGH DEPARTMENT OF OTORHINOLARYNGOLOGY CLINICAL PRACTICE GUIDELINES OBSTRUCTIVE SLEEP APNEA SYNDROME IN CHILDREN

SCOPE OF THE PRACTICE GUIDELINE This clinical practice guideline is for use by the Department of Otorhinolaryngology of the College of Medicine - Philippine General Hospital, University of the Philippines Manila. It covers the diagnosis and management of obstructive sleep apnea syndrome in children. This guideline only applies to children more than 1 year of age with obstructive symptoms attributable to adenotonsillar hypertrophy and/or obesity. Children with neuromuscular, craniofacial, neurologic, and other metabolic diseases that may contribute to ventilation problems during sleep are specifically excluded from this guideline. OBJECTIVES The objectives of the guideline are (1) to enhance the ability to recognize OSAS; (2) to determine the diagnostic procedures that may effectively diagnose OSAS using the least financial resources possible; (3) to describe the ideal diagnostic modalities for pediatric OSAS; (4) to describe management options in pediatric OSAS; and (5) to identify high-risk patients for post-operative complications. LITERATURE SEARCH The guideline was based on data available from medical literature. A computerized search of the National Library of Medicine’s PubMed database was performed using the key words sleep apnea syndrome, sleep disordered breathing, apnea, tonsillectomy, adenoidectomy and polysomnography. The search was limited to clinical trials, meta-analysis, practice guideline and randomized control trial studies written in English on children (0-18 years). Editorials, letters and reviews as well as animal studies were excluded. The search resulted in 733 articles. Those related to the pediatric patient population with craniofacial anomalies, congenital conditions, metabolic diseases and sudden infant death syndrome were eliminated. Ninety-five articles were evaluated which yielded 28 studies. Abstracts were reviewed and full text articles obtained when possible. The Clinical Practice Guideline: Diagnosis and Management of Childhood Obstructive Sleep Apnea Syndrome (April 2002) by the American Academy of Pediatrics in particular was reviewed and its references obtained. DEFINITIONS Obstructive sleep apnea syndrome (OSAS) is characterized by partial or complete pharyngeal obstruction during sleep combined with daytime somnolence. This is better elaborated in the following definition of the American Thoracic Society:

OSAS in children is a disorder of breathing during sleep characterized by prolonged partial upper airway obstruction and/or intermittent complete obstruction (obstructive apnea) that disrupt normal ventilation during sleep and normal sleep patterns. It is associated with symptoms including habitual (nightly) snoring, sleep difficulties, and/or daytime neurobehavioral problems. Primary snoring is defined as loud upper-airway breathing sounds in sleep, without episodes of apnea or hypoventilation. Sleep disordered breathing is a broader term that encompasses all breathing disorders during sleep. These are obstructive, central and mixed sleep apnea, central hypoventilation syndrome, breathing disorders due to craniofacial, metabolic and medical conditions,etc.

PREVALENCE There are no studies to determine prevalence in the local setting. However, based on foreign literature (Britain, Iceland and the United States) similar prevalence rates were reported at 2%. It is commonly found in preschool-aged children aged 2 to 5 years, as this is the age when the tonsils and adenoids are relatively large for the airway size. There is a second peak during middle to late adolescence but is qualitatively similar to adult OSAS. It occurs equally in boys and girls. Post-pubertal distribution likewise reflects the male preponderance of adult OSAS.

23

RECOMMENDATIONS ON THE DIAGNOSIS OF OBSTRUCTIVE SLEEP APNEA SYNDROME A child is commonly referred for sleep evaluation because the parents are anxious. Common complaints are persistent snoring, labored breathing and witnessed apnea. 1. Routine sleep history and physical examination should be obtained. Grade B Recommendation

Whenever a child is referred for sleep apnea or snoring, the height and weight must be recorded. A routine sleep history and physical examination should be developed. History should include inquiries on the following: 1.1 Habitual snoring 1.2 Labored breathing during sleep 1.3 Observed apnea 1.4 Paradoxical chest / abdomen motion 1.5 Restless sleep 1.6 Diaphoresis, enuresis, retractions 1.7 Cyanosis 1.8 Excessive daytime sleepiness 1.9 Behavior and learning problems (including attention-deficit hyperactivity disorder)

Physical examination may not be productive but may include the following: 1.10 Adenotonsillar hypertrophy 1.11 Mouth breathing 1.12 Nasal obstruction 1.13 Adenoidal facies 1.14 Hyponasal speech 1.15 Pectus carinatum with flaring of lower ribs 1.16 Poor growth / failure to thrive OR obesity It should be noted that clinical evaluation is not very effective in determining the presence of OSAS. It is up to the clinician to determine if the problem should be pursued. If the history and physical examination are not consistent with OSAS and the suspicion for disease is low, the parents are reassured and the patient is followed up.

2. If the patient has signs of right-sided heart failure such as dyspnea and orthopnea, it would be best that the patient be managed by a pediatric pulmonologist or a cardiologist with a backgound in sleep medicine. Grade C Recommendation

3. Witnessed apnea as seen by a laboratory technician or clinician is considered

acceptable evidence to proceed with treatment of OSAS according to the guidelines published by the American Thoracic Society. Grade C Recommendation

However, witnessed apnea as seen by the parents may be considered a positive finding. This should manifest as cessation of breathing with continued effort (increased abdominal and chest wall motion). If the parents are unsure if the child is breathing, they may be advised to place a piece of thin tissue paper in front of the child’s nose when an apneic event is suspected. Ask them to count duration of the event. If the clinician doubts the report of the parents, he/she may request for diagnostic tests.

4. Nocturnal polysomnography is the gold standard in the diagnosis of OSAS. Grade B Recommendation

24

It can distinguish between OSAS and primary snoring provided that a criterion appropriate for pediatric patients is used. It can determine severity of OSAS, degree of respiratory compromise and sleep architecture. This is the only study where the grading of severity of OSAS may be used. There has been some concern over the first-night effect of overnight polysomnography wherein a single-night’s study may not always provide accurate information. However, it has been shown that the first-night effect is insignificant. In a study of 30 children with 2 overnight PSGs done between 7 to 27 days apart, all parameters measured had no significant difference. No child changed diagnosis from primary snoring to OSA or the other way around. Therefore, the result of a single technically correct PSG is an adequate measure of OSAS.

Unfortunately, nocturnal polysomonography is time consuming and expensive. A number of less costly tests may be used as screening tools. If positive, they are highly predictive of OSAS. But when negative, they provide no diagnostic value. Nocturnal polysomnography should, therefore be done in patients when the screening methods are equivocal or negative. It should be the first line diagnostic modality for those patients who can afford it. 4.1 Audiotaping and videotaping. Results of 3 studies showed a sensitivity of 71% to 94%

and a specificity of 29% to 80%. Positive predictive values were 50% and 75% for audiotaping and 83% for videotaping. Sounds of struggle during sleep were more predictive than respiratory pauses. Negative predictive value was 73% to 88%. The methods used for evaluating audio and video recordings were not standardized. Though deficient in supporting data, these techniques, when highly suspicious for OSAS may be used in deciding to proceed with treatment. During videotaping, if a suspected apneic event occurs, the parent places a thin piece of tissue in front of the child’s nose to determine if there is airflow.

4.2 Overnight pulse oximetry. A positive predictive value (PPV) of 97% and a negative

predictive value (NPV) of 47% was shown in 1 study. This test is considered positive when there is a cyclic desaturation pattern during sleep. Thus, results are only useful when positive and may be used in deciding to proceed with treatment.

4.3 Nap polysomnography. This is done for 1 hour during the day. Children unable to sleep

are sedated with choral hydrate without adversely affecting the results. PPV was 77% to 100% and NPV was 17% to 49%. This test is only useful when positive but it tends to underestimate severity of disease.

4.4 Unattended home polysomnography. This yielded similar results to laboratory studies but

was done using sophisticated equipment that is unavailable in the Philippines. Standard 6 to 8-channel home equipment will probably be technically difficult to maintain in children in an unattended study and is, therefore, not recommended.

RECOMMENDATIONS ON THE MANAGEMENT OF OBSTRUCTIVE SLEEP APNEA SYNDROME 1. Parental reassurance and close patient follow-up are recommended if the

polysomnography result is negative. Grade C Recommendation 2. Tonsillectomy and adenoidectomy are recommended if (1) the polysomnography result

is positive; or (2) the screening tests are positive. Grade A Recommendation The most common cause of OSAS in children is still adenotonsillar hypertrophy.

Adenotonsillectomy is usually curative and has been shown to significantly improve quality of life and learning capacity of patients. Polysomnographic resolution occurs in 75% to 100%

25

and is associated with resolution of symptoms. Results may be less satisfactory in obese children.

3. All OSAS patients for surgery should be done on an in-patient basis with post-operative

overnight monitoring by pulse oximetry. Post-operative PICU admission is prudent in cases where the risk factors are present. Grade B Recommendation

The risk factors for post-operative complications in children with OSAS undergoing T&A are the following: 1) age younger than 3 years, 2) severe OSAS on polysomnography, 3) cardiac complications of OSAS (e.g. right ventricular hypertrophy), 4) failure to thrive, 5) obesity, 6) recent respiratory infection, 7) prematurity, 8) craniofacial anomalies, and 9) neuromuscular disorders.

4. All patients should have regular follow-up after treatment. Grade C Recommendation

In other countries, frequency or follow-up is determined by OSAS severity as graded by nocturnal polysomnography. However, this does not apply to this guideline due to the acceptance of positive results from alternative screening tools. These do not quantify respiratory events during sleep and no grade can be derived. The frequency of follow-up should then be determined by the presence of symptoms. Children with continued symptoms may need laboratory polysomnography.

5. Continuous Positive Airway Pressure (CPAP) may be used for some patients with

special situations. Grade C Recommendation

CPAP may be used in patients with (1) minimal adenotonsillar tissue; (2) surgical contraindications; or (3) persistent OSAS after tonsillectomy and adenoidectomy. This will need to be used indefinitely and may result in midfacial hypoplasia. Small children and those with learning disabilities may require behavioral techniques to improve acceptance. Another option is the use of mandibular repositioning dental appliances.

6. Adjunctive treatment modalities should not delay initiation of more definitive

treatment. Grade B Recommendation The adjunctive treatments include: 1) weight loss for obese patients, 2) avoidance of

tobacco/cigarette smoke exposure, 3) avoidance of indoor pollutants, 4) environmental allergen control in the home, 5) treatment of accompanying rhinitis, 6) avoidance of sleep deprivation, and 7) avoidance of sedating medications.

REFERENCES

1. American Academy of Pediatrics, Section on Pediatric Pulmonology, Subcommittee on Obstructive Sleep Apnea Syndrome. Clinical practice guideline: diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics. 2002;109(4):704-712.

2. Schechter MS, and the American Academy of Pediatrics, Section on Pediatric Pulmonology, Subcommittee on Obstructive Sleep Apnea Syndrome. Technical Report: diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics. 2002;109(4).

3. American Thoracic Society. Standards and indications for cardiopulmonary sleep studies in children. Am J Respir Crit Care Med. 1996;153:866-878.

4. American Sleep Disorders Association. International Classification of Sleep Disorders, Revised: Diagnostic and Coding Manual. Rochester, MN: American Sleep Disorders Association; 2001.

26

5. Marcus C, State of the Art, sleep disordered breathing in children. Am J Respir Crit Care Med. 2001;164:16-30.

6. de Serres L, Derkay C, Sie K, Biavati M, Jones J, Tunkel D, Manning S, Inglis A, Haddad J, Tampakopoulou D, Weinberg A. Impact of Adenotonsillectomy on the quality of life in children with obstructive sleep disorders. Arch Otolaryngol Head Neck Surg. 2002;128:489-496.

7. Urschitz M, Wolff J, von Einem V, Urschitz-Duprat P, Schlaud M, Poets C. Reference values for nocturnal home pulse oximetry during sleep in primary school children. Chest. 2003;123:96-101.

8. von Someren V, Burmester M, Alusi G, Lane R. Are sleep studies worth doing? Arch Dis Child. 2000;83:76-81.

9. Gozal D. Sleep-disordered breathing and school performance in children. Pediatrics. 1998;102:616-620.

10. Redline S, Tishler P, Schluchter M, Aylor J, Clark K, Graham G. Risk factors for sleep-disordered breathing in children, associations with obesity, race, and respiratory problems. Am J Respir Crit Care Med. 1999;159:1527-1532.

11. Marcus C, Mc Colley S, Carroll J, Loughlin G, Smith P, Schwartz A. Upper airway collapsibility in children with obstructive sleep apnea syndrome. J Appl Physiol. 1994;77:918-924.

12. Brilouette R, Morielli A, Liemanis A, Waters K, Luciano R, Ducharme, F. Nocturnal pulse oximetry as an abbreviated testing modality for pediatric obstructive sleep apnea. Pediatrics. 2000;105:405-412.

13. Saeed M, Keens T, Stabile M, Bolokowicz J, Davidson Ward S. Should children with suspected obstructive sleep apnea syndrome and normal nap sleep studies have overnight sleep studies? Chest. 2000;118:360-365.

14. Li K, Riley R, Guilleminault C. An unreported risk in the use of home nasal continuous positive airway pressure and home nasal ventilation in children. Chest. 2000;117:916-918.

15. Bower C, Gungor A. Pediatric obstructive sleep apnea syndrome. Otolaryngol Clinics of North Am. 2000;33:49-75.

16. Wiatrak BJ, Myer CM, Andrews TM. Complications of adenotonsillectomy in children under 3 years of age. Am J Otolaryngol. 1991;12:170-172.

17. Marcus CL, Keens TG, Ward SL. Comparison of nap and overnight polysomnography in children. Pediatr Pulmonol. 1992;13:16-21.

18. Marcus CL, Greene MG, Carrol JL. Blood pressure in children with obstructive sleep apnea. Am J Respir Crit Care Med. 1998;157:1098-1103.

19. Wang RC, Elkins TP, Keech D, Wauquier A, Hubbard D. Accuracy of clinical evaluation in pediatric obstructive sleep apnea. Otolaryngol Head Neck Surg. 1998;118:69-73.

20. Sivan Y, Kornecki A, Schonfeld T. Screening obstructive sleep apnea syndrome by home video tape recording in children. Eur Respir J. 1996;9:2127-2131.

21. Carroll JL, Mc Colley SA, Marcus CL, Curtis S, Loughlin GM. Inability of clinical history to distinguish primary snoring from obstructive sleep apnea syndrome in children. Chest. 1995;108:610-618.

22. Goldstein NA, Sculerati N, Walsleben JA, Bhatia N, Friedman DM, Rapoport DM. Clinical diagnosis of pediatric obstructive sleep apnea validated by polysomnography. Otolaryngol Head Neck Surg. 1994;111:611-617.

23. Mc Colley SA, April MM, Carroll JL, Naclerio RM, Loughlin GM. Respiratory compromise after adenotonsillectomy in children with obstructive sleep apnea. Arch Otolaryngol Head Neck Surg.

1992;118:940-943. 24. Nieminen P, Tolonen U, Lopponen H, Lopponen T, Luotonen J, Jokinen K. Snoring children:

factors predicting sleep apnea. Acta Otolaryngol Suppl. 1997;529:190-194. 25. Galvis AG. Pulmonary edema complicating relief of upper airway obstruction. Am J Emerg Med.

1987;5:294-297. 26. Marcus CL, Ward SL, Mallory GB, Rosen CL, Beckerman RC, Weese-Mayer DE, Brouillette RT,

Trang HT, Brooks LJ. Use of nasal continuous positive airway pressure as treatment of childhood obstructive sleep apnea. J Pediatr. 1995;127:88-94.

27. Nieminen P, Tolonen U, Lopponen H. Snoring and obstructive sleep apnea in children: a 6-month follow-up study. Arch Otolaryngol Head Neck Surg. 2000;126:481-486.

28. Elsherif I, Kareemullah C. Tonsil and adenoid surgery for upper airway obstruction in children. Ear Nose Throat J. 1999;78:617-620.

27

ALGORITHM FOR UNCOMPLICATED OBSTRUCTIVE SLEEP APNEA SYNDROME IN CHILDREN 1 2 5 3 4 7 6 7 9 8 9 10 11 12 13 14 15

Advise parents and follow-up

History and PE suggestive of OSAS

Cardio-respiratory failure?

Refer to pediatric cardiologist or pulmonologist specialist preferably with sleep medicine background

Child presents with snoring or other symptoms suspicious for OSAS

Intermittent screening on follow-up

Screening studies: Audiovisual taping

Overnight pulse oximetry Nap study

Screening shows OSAS is highly probable

Is the patient a candidate for

T & A?

Tonsillectomy & Adenoidectomy

Was surgery successful?

Laboratory Polysomnography

Treatment other than T&A

implemented

Positive for OSAS?

PROCEED TO BOX 11; MAY ALSO CONSIDER BOX 7 IF NOT YET DONE

High-risk patients may need PICU admission post-op for monitoring

Witnessed apnea?

PROCEED TO BOX 10

PROCEED TO BOX 10 Intermittent screening on follow-up

Appropriate follow-up & monitoring

28

Evidence for Guidelines on Pediatric Obstructive Sleep Apnea

Author Year No. Age Diagnostic Technique Study Methodology and Rating

Results and Comments Algorithm Box

Caroll 1995 83 5.4m – 14.8y Questionnaire and overnight PSG

Retrospective III

Obstructive Sleep Apnea (OSA) Score misclassified 1 in 4 patients; PS cannot be distinguished from OSAS on history alone Se 76.8%, Sp 71.4%, PPV 64.7%, NPV 83.3%

2

Wang 1998 82 No mention Overnight PSG II No significant association between clinical parameters and OSA Highest PPV 46% for enuresis; highest NPV 100% for small tonsils

2

Leach 1992 93 18 m – 12 y Overnight PSG Retrospective III

OSA by PSG had no significant association with age, sex, symptoms and obesity OSA by PSG associated with cor pulmonale, tonsil hypertrophy and failure to thrive

2

Redline 1999 399 2 – 18 y Home Overnight PSG Cohort II

Moderate SDB associated with: obesity (odds ratio 4.59; 95% CI, 1.58 – 13.33) African-American race (OR 3.49; 95% CI, 1.56-8.32) Not associated with sex and age Independent predictors of SDB: obesity, proband sampling, race and familial clustering, sinus problems, persistent wheeze

2

Goldstein

1994 30 No mention Audio recording, PSG II Clinical assessment: Se 92.3%, Sp 29.4%, PPV 50.0%, NPV 83.3% 2, 7

Nieminen

1997 78 < 18 y Parent’s reports, PSG II No Se and Sp data; conclusion: OSA cannot be diagnosed without PSG 2, 7

Marcus 1998 67 OSAS 5 +/- 3 y PS 8 +/- 4 y

Overnight pulse oximetry & BP monitoring

II Childhood OSAS associated with systemic diastolic hypertension 3

Sivan 1996 58 No mention 30-min video recording, overnight PSG

II Se 94.4%, Sp 68.2%, PPV 82.9%, NPV 88.2% 5

Goldstein

1994 30 No mention Questionnaire, audio recording, overnight PSG

III Se 92.3%, Sp 29.4%, PPV 50.0%, NPV 83.3% 5

Marcus 1992 40 5.4 +/- 0.8 y Nap and overnight PSG

III Nap studies: Se 74%, Sp 100%, PPV 100%, NPV 17% 5

Saeed 2000 143 1 –18 y Questionnaire, Nap and overnight PSG

Nap parameters compared to overnight PSG: Se 68.4%, Sp 60.4%, PPV 77.4%, NPV 49.2% Normal to mildly abnormal nap studies should be confirmed by overnight PSG Naps studies underestimate severity of OSA

5

Brouilette

2000 349 6 m – 18 y Overnight pulse oximetry & PSG

Cross-sectional III

Indeterminate 46% Se 42.9%, Sp 97.8%, PPV 96.8%, NPV 53.1% Oximetry may be used in the diagnosis of > 12 m children May be a screening tool for children who will be referred for overnight PSG to determine type and severity Negative oximetry cannot rule out OSA

5

Urschitz 2003 468 3rd – 4

th grade

students Overnight oximetry II Defined SpO2 for children at sea level at >/= 98% with frequent falls >/= 4%; </=

90% rare 5

Van 2000 120 6 m – 15.5 y Questionnaire, Prospective survey PSG contributes to assessing need for operation, likelihood of post op respiratory 7

29

Someren

PSG, clinicians impression

III failure, or as a baseline or outcome measure

Marcus 1995 94 < 1 – 19 y Questionnaire Retrospective III

CPAP effective in 86% Minor complications

11

Galvis AG

1987 20 <18 y Chart review III Pulmonary edema may occur post op in children with severe obstruction; this is sometimes unrecognized

12

Mc Colley

1992 69 < 18 y Chart review, PSG III Most significant risk factors for post op respiratory compromise: age < 3 y, AHI > 10.

12

Gozal 1998 297 1st grade

students Questionnaire, overnight oximetry, transcutaneous partial pressure of CO2

Prospective cohort II

High prevalence of snoring and nocturnal gas exchange abnormalities in academically poor achievers with significant in improvement of grades after intervention (usually tonsillectomy)

13

Nieminen

2000 58 3 – 10 y Overnight PSG RCT I

46.7% of subjects with OSA; 53.3 % with PS 78% of OSA patients cured after T&A

13

De Serres

2002 101 6.2 +/- 2.5 y Questionnaire III 90% of children had improvement after adenotonsillectomy large improvement 74.5% moderate 6.1% small 7.1%

13

30

UP-PGH DEPARTMENT OF OTORHINOLARYNGOLOGY CLINICAL PRACTICE GUIDELINES THYROID MASSES SCOPE OF THE PRACTICE GUIDELINE This clinical practice guideline is for use by the Department of Otorhinolaryngology of the College of Medicine - Philippine General Hospital, University of the Philippines Manila. It covers the diagnosis and management of non-toxic thyroid masses. It excludes the management of extremely rare malignancies of the thyroid gland such as lymphomas. OBJECTIVES The objectives of the guideline are: (1) to Identify points in the history and physical examination that will help in the classification of thyroid neoplasms; (2) to recommend relevant diagnostic examinations; (3) to highlight areas of controversy in the management; and (4) to suggest treatment options. LITERATURE SEARCH This guideline is based on the Management Protocol for Head and Neck Malignancies developed by the department’s Head and Neck Division in 2002. A journal search using the Medline of PubMed (full texts and abstracts were obtained) for more recent publications was done. Keywords used were “thyroid nodule, thyroid neoplasm and thyroid cancer.” Very limited literature containing prospective randomized control trials were obtained probably due to the need for a very long follow-up. Current guidelines on the diagnosis and management of thyroid nodules were also reviewed. The chosen articles were divided as follows: Meta-Analysis of Randomized Controlled Trials: 1 Randomized Controlled Trials: 2 Well-Designed Clinical Studies without Randomization: 1 Well-Designed Quasi-Experimental Studies: 2 Well-Designed Non-Experimental Study: 4 Expert Committee Reports: 4 DEFINITION Nodular goiter is the presence of a discrete mass within the substance of the thyroid gland. Diffuse goiter is the enlargement of the entire thyroid gland. Papillary carcinoma is a well-differentiated type of thyroid carcinoma derived from the thyroid follicular cells. It is the most common malignant neoplasm of the thyroid gland. Generally, it is not an aggressive tumor and it may be compatible with life.

15

Follicular tumor is of thyroid follicular cell origin. It is encapsulated, composed of follicles of various histologic appearances. A follicular adenoma can only be differentiated from a follicular carcinoma microscopically. Capsular and/or vascular invasion are found in follicular carcinoma.

1

Hurthle cell carcinoma or oncocytic neoplasms are composed of cells with abundant pink (oxyphilic) cytoplasm containing ample abnormal mitochondria on ultrastructural examination. Majority of these lesions are benign. As with follicular carcinoma, presence of capsular or vascular invasion is a prerequisite for the diagnosis of malignancy. They are more aggressive than the conventional papillary or follicular carcinoma, suggesting an intermediate-grade malignant behavior.

13

31

Medullary carcinoma is a malignant tumor derived from the calcitonin-secreting parafollicular C-cells of the thyroid. Occurring in less than 10% of cases, it may arise spontaneously, or as part of a familial pattern of inheritance (MEN Syndrome). It is associated with mutations in the RET proto-oncogene in chromosome 10.

13

Anaplastic carcinoma is an undifferentiated carcinoma of the thyroid gland that is clinically very aggressive---characterized by rapid growth, infiltration of the surrounding tissues and easy spread. It may also arise as a de-differentiation from an existing well-differentiated papillary or follicular carcinoma. All patients die, mostly due to respiratory compromise caused by the tumor.

13

Risk stratification is the classification of well-differentiated CA into low, intermediate and high risk based on given parameters. The panel adopted the Memorial Sloan-Kettering Cancer Center scheme.

14

Table 1: Risk Stratification for Thyroid Carcinoma

PREVALENCE The incidence of clinically apparent thyroid nodule in the general population is approximately 4% to 5%. However, in autopsy studies, thyroid nodularity is approximately 37%. The incidence of malignancy in a solitary thyroid nodule is 10 – 15%

1.

In the Philippines, thyroid mass occurs in 6.6 / 100,000 of the general population. 3.1 / 100,000 males and 9.8 / 100,000 females are affected (Philippine Cancer Registry). In the PGH Department of ORL outpatient clinic, 28% of the new patients consulted for thyroid mass in the year 2002 (Annual Report 2002).

LOW INTERMEDIATE INTERMEDIATE HIGH

Age <45 <45 >45 >45

Distant Metastasis

M0 M+ M0 M+

Tumor size T1, T2 (<4cm)

T3, T4 (>4cm)

T1, T2 (<4cm)

T3, T4 (>4 cm)

Histology and grade

Papillary Follicular &/or High-grade

Papillary Follicular &/or High-grade

32

RECOMMENDATIONS ON THE DIAGNOSIS OF THYROID NODULES 1. Clinical evaluation of patients with thyroid enlargement begins with a

comprehensive history and physical examination. Grade C Recommendation 1.1 The following elements of history and physical exam favor benign disease but do

not exclude the presence of thyroid cancer:16,17

1.1.1 Soft, smooth, mobile, doughy solitary nodule 1.1.2 Multinodular goiter without a dominant nodule 1.1.3 Symptoms of hypo- or hyperthyroidism 1.1.4 Pain or tenderness associated with the nodule 1.1.5 Family history of Hashimoto’s thyroiditis or autoimmune disease 1.1.6 Family history of benign thyroid nodule or goiter

1.2 The following are points of history and physical examination increasing the

suspicion of malignant thyroid disease: 1.2.1 Solitary (versus multinodular), hard (versus doughy), fixed (versus

mobile) nodule 1.2.2 Rapid growth (doubling of size in 6 months) 1.2.3 Vocal cord paralysis 1.2.4 History of irradiation to the neck during childhood or adolescence 1.2.5 Age (very young at <20, or older at >60) 1.2.6 Gender (male)

The proportion of nodules that are malignant in males is double compared to females.

15

1.2.7 Recurrent cystic nodule 1.2.8 Presence of lateral neck nodes 1.2.9 Presence of distant (pulmonary) metastasis

Complete examination of the head and neck area includes not only inspection and palpation of the thyroid gland but also the lateral aspects of the neck for cervical lymphadenopathy. Laryngoscopy to document the mobility of vocal cords is a must.

2. TSH and T4 assays should be done for all patients with thyroid enlargement, with

or without functional disturbance. Grade B Recommendation

It is important to establish the status of the thyroid function, as this would dictate the initial medical management. TSH and T4 assays are requested for screening purposes only, that is, to determine the presence of hyperthyroidism or hypothyroidism. Special cases will be co-managed with the endocrinologists. The cost-effectiveness of requesting free T4 as a screening diagnostic tool is being questioned especially for indigent patients. Only rarely do patients with malignant solitary nodules have hyperthyroidism or hypothyroidism. An abnormal TSH level decreases the suspicion but does not eliminate the possibility of malignancy.

17

3. FNAB should be done for all cases of thyroid nodules and enlargement of the

thyroid suspicious for malignancy (lymphoma, anaplasic carcinoma, medullary carcinoma, metastatic tumor).

Grade B Recommendation

FNAB is the cornerstone in the evaluation of solitary thyroid nodules and dominant nodules within multinodular goiters. If done properly, sensitivity is 83.9%.

6 The results

can generally be divided into malignant, benign, suspicious or insufficient/indeterminate.

33

4. Thyroid ultrasound is not routinely recommended in the initial evaluation of a thyroid nodule. Grade B Recommendation The ultrasound is sensitive in determining the size and number of nodules. This may provide a sensitive and objective indicator of whether a nodule is increasing or decreasing in size over time. It differentiates between a cystic and a solid nodule but not between a benign and malignant nodule. Its ability to identify good surgical candidates without the assistance of other diagnostic modalities is very limited.

6

The indications for requesting a thyroid ultrasound are:

4.1 Differentiate between a cystic and solid nodule 4.2 Differentiate thyroid from extra-thyroid masses in the neck 4.3 Determine the location of a thyroid nodule of interest 4.4 Provide baseline measurement to monitor clinical response of obscure nodules

to medical treatment 4.5 Provide patient and/or physician reassurance of a doubtful thyroid enlargement

5. Thyroid scan is not routinely recommended in the initial evaluation of a thyroid

nodule. Grade B Recommendation

The thyroid scan may be helpful in the evaluation of thyroid nodules, specifically its

functional nature (hot or cold). The incidence of malignancy in cold thyroid nodules is approximately 20% and that in hot thyroid nodules is less than 5%. (sensitivity 91%, specificity 19%). Only 10% of nodules are delineated as benign; hence results are uncertain in the remaining 90% (sensitivity 91%, specificity 19%).

16 Thyroid scan is

recommended for nodular toxic goiters but will not differentiate a benign from a malignant mass.

6

6. Chest X-ray, MRI or CT scan are not routinely done for patients with thyroid

neoplasm. Grade C Recommendation

The imaging tests are reserved for clinical cases where the true extend of tumor

involvement cannot be clinically established. They are used to evaluate for possible metastatic disease and when there is a suspicion of tumor extension to the larynx, trachea or mediastinum.

RECOMMENDATIONS ON THE MANAGEMENT OF THYROID NODULES BENIGN NODULE 1. Levothyroxine suppression may be used as management for benign thyroid

nodules. Grade B Recommendation

Several studies have shown levothyroxine suppression to be effective in shrinking

nodules, particularly those less than 4 cm.10,19

There are also randomized controlled trials that have failed to document its efficacy.

18 Most clinicians use it for a limited time (6-12

months) with the serum TSH maintained in the range of 0.1 to 0.3 mU/mL in an attempt to decrease the risks of long-term suppressive therapy like osteoporosis, cardiac hypertrophy and atrial fibrillation. Although there is an enduring controversy regarding the role of levothyroxine suppression treatment for patients with benign thyroid nodules, some patients may benefit from it. Individualized clinical judgment is recommended.

34

The indications for medical treatment are: (1) nodule less than 4 cm with a stable

pattern of growth; (2) nodule bigger than 4 cm but with a clinically slim chance of malignancy; and (3) patient opts for a trial of medical treatment before surgery

Patients with thyroid nodules in whom malignancy has been excluded require

periodic clinical observation with judicious use of laboratory tests, imaging procedures, needle biopsy and levothyroxine sodium suppression therapy. The goals of follow-up are (1) to recognize progressive enlargement that could result in local compressive and invasive complications (e.g. dysphagia, cough, pain, dyspnea, hoarseness) or cosmetic concerns or signs of malignancy; (2) to diagnose associated clinical or subclinical thyroid dysfunction; and (3) to identify patients in whom there may be an undiagnosed or subsequent thyroid malignancy.

During the course of follow-up, repeated FNAB may be required in the following situations: 1.1 increase in size regardless of medical treatment 1.2 development of new clinical features suggestive of malignancy 1.3 indeterminate FNAB done previously

FNAB may be done twice if the first reveals indeterminate results. If the 2nd

FNAB has still inconclusive results, a 3

rd FNAB may be done by the Pathologist. If the 3

rd FNAB

is still indeterminate, then surgery may be done with frozen section.

2. Surgery is another option in the management of benign thyroid nodules

Grade C Recommendation

The absolute indications for surgery are obstructive symptoms and hemorrhage (iatrogenic or spontaneous). Non-response to medical treatment and cosmesis are relative indications for surgery. Some clinicians would resort to surgery when there is an increase in size during suppressive therapy without repeating the FNAB.

THYROID CANCER 1. Surgery is the primary mode of management for thyroid cancer.

Grade B Recommendation

Basic considerations in the management of thyroid cancer are the following: 1.1 Outcome is generally excellent. 1.2 Prognostic and risk factors are well-defined. 1.3 Generally, a low-risk patient has excellent outcome. 1.4 Risk group stratification is the main factor in treatment decisions. 1.5 High-risk groups are well-defined and need aggressive treatment with total

thyroidectomy and radioactive iodine (RAI) therapy. 1.6 The role of RAI in low-risk groups is currently not well-defined. 1.7 The presence of nodal metastasis has few prognostic implications. 1.8 Age is the most important prognostic factor.

35

2. Well-differentiated thyroid cancers are classified into low, intermediate and high-risk. Grade B Recommendation

LOW INTERMEDIATE INTERMEDIATE HIGH

Age <45 <45 >45 >45

Distant Metastasis

M0 M+ M0 M+

Tumor size T1, T2 (<4cm)

T3, T4 (>4cm)

T1, T2 (<4cm)

T3, T4 (>4 cm)

Histology and grade

Papillary Follicular &/or high grade

Papillary Follicular &/or high grade

5-year survival (%)

100 96 96 72

20-yr survival (%)

99 85 85 57

3. Papillary thyroid cancer (PTC)

Grade B Recommendation

A unilateral lobectomy and isthmusectomy is recommended for minimal PTC (less than 1 cm.

16,17 There is a continuing debate between doing a total thyroidectomy or just a

lobectomy for low-risk patients1, 15

. Total or near-total thyroidectomy is recommended in the following situations: 3.1 Intermediate and high-risk patients 3.2 Presence of bilateral nodules 3.3 Extension of the primary tumor beyond the thyroid capsule 3.4 Presence of local or distant metastases Central and lateral neck dissection is recommended for patients with clinically palpable nodes.

4. Follicular or Hurthle cell cancer Grade B Recommendation

Follicular adenomas and carcinomas are difficult to distinguish at the time of surgical intervention. Frozen section is necessary but has a limited value if the cytologic diagnosis is that of a follicular tumor.

A unilateral lobectomy and isthmusectomy is recommended. If the mass is benign on final histopathology, no further therapy is needed. However, If it is malignant, completion (total) thyroidectomy is indicated.

A histologic diagnosis of a follicular thyroid carcinoma immediately places the patient in the intermediate-risk category, at the very least. Thus, a total or near-total thyroidectomy is warranted.

Central and lateral neck dissection is recommended for patients with clinically palpable nodes.

36

5. Medullary thyroid cancer Grade B Recommendation

Management precludes a total thyroidectomy with central neck dissection and ipsilateral modified radical neck dissection. Furthermore, the patient and his family should undergo screening and evaluation by an endocrinologist.

6. Anaplastic thyroid cancer

Grade B Recommendation

Surgical treatment is of limited use and is indicated primarily for relief of airway obstruction.

External beam radiotherapy can aid in local disease control, although anaplastic thyroid cancer is generally considered a radioresistant tumor compared with other solid neoplasms. There is reported improvement in overall survival and respectability with the use of external beam radiotherapy preoperatively and in combination with chemotherapy.

15

7. Adjuvant treatment

Grade B Recommendation

Postoperative adjuvant treatment consists of: 7.1 Radioactive iodine scan – In well-differentiated thyroid cancers, it is

recommended when there is: 7.1.1 Distant metastasis 7.1.2 Gross residual tumor following surgery 7.1.3 High risk of local recurrence following total thyroidectomy for a large

tumor 7.1.4 Resection of multiple lymph node metastasis in the lateral compartment

of the neck or superior mediastinum 7.2 Levothyroxine suppression – It is recommended to keep TSH level as low as

possible.

7.3 External beam radiation therapy – It is used for cancers with a poorly- differentiated histology. It is helpful for patients with residual tumor in the central compartment of the neck, particularly if the tumor uptake of radioactive iodine is poor.

37

Risk

Stratification

Low Risk? Lobectomy w/

Isthmusectomy; or

Near total/Total

Thyroidectomy; No adjuvant

treatment

Total Thyroidectomy Post-op RAI/

LT4 Suppression

Anterior Neck Mass

History Physical Examination

TSH, T4

Hyperthyroid?

Refer to Endocrinology

for Medical Management

Diffuse?

Fine Needle

Aspiration Biopsy

Definitely Malignant?

Well –

Differentiate

d? (Yes, if

Papillary or

Follicular)

Medullary

Carcinoma?

Anaplastic?

Secure Airway, Radiotherapy

Total Thyroidectomy,

Neck Dissection; Post –

operative Radiotherapy

Total Thyroidectomy; Radiotherapy

Definitely Benign?

3

Repeat FNAB

Definite Diagnosis?

4

38

Lobectomy w/

Isthmusectomy, OR

Near total/Total Thyroidectomy;

No adjuvant treatment

4

Thyroidectomy w/

Frozen Section

Frozen

Section: Benign?

Observe Follow-Up

Risk Stratification

Low

Risk?

Total

Thyroidectomy,

LT4 Suppression,

RAI

Final Histopath Benign?

Completion Thyroidectomy,

RAI, LT4 Suppression

Low Risk?

Risk Stratification

Thyroidectomy

Decrease

in Size?

LT4 Suppression x 3 mos

>4

cm

?

3

Thyroidectomy

Continue LT4 Suppression x 3

mos

End of

treatment

Observe

LT4 Suppression

x 6 months

Decrease in size?

Continue LT4

x 6 months

Decrease

in size?

Continue LT4 x 6 months

(monitor TSH

levels, should not

be less than 0.1

mU/mL)

Poor surgical risk

or no consent?

RAI

Subtotal Thyroidectomy

39

REFERENCES

1. Cummings C, et al. Otolaryngology, Head and Neck Surgery 3rd

ed. Vol 1. Mosby-Year Book Inc., St. Louis, Missouri, 1998.

2. Huysmans DA, Hermus RMM, Corstens FHM, Barentsz J and Kloppenborg KWC, Large, Compressive Goiters Treated with Radioiodine. Annals of Internal Medicine. 121:757-62. Nov 1994.

3. Massaterri E & Lkoos R. Current Approaches to primary therapy for papillary and follicular thyroid cancer. Journal of Clinical Endocrinology and Metabolism, 86(4): 1447-63.

4. Hegedus L, Nygaard B & Hansen JM. Is routine thyroxine treatment to hinder postoperative recurrence of nontoxic goiter justifies? Journal of Clinical Endocrinology and Metabolism, 84(2): 756-760, 1999.

5. Huysmans DA, Nieuwlaat W, Erdtsieck RJ, Schellekens AP, Bus JW, Bravenboer B, and Hermus AR. Administration of a Single Low Dose of Recombinant Human Thyrotropin Significantly Enhances Thyroid Radioiodide Uptake in Nontoxic Nodular Goiter. Journal of Clinical Endocrinology and Metabolism, 85(10): 3592-3596. 2000

6. Kountakis MD et al. The Radiologic work-up in thyroid surgery: fine needle biopsy versus scintigraphy and ultrasound. Ear, Nose Throat Journal, 81(3): 151-4. Mar 2002.

7. Medical Oncology Section, Department of Medicine, Philippine General Hospital, College of Medicine, University of the Philippines. Handbook on Basic Medical Oncology, 2

nd Ed.

2001 8. Nuria A, Lucas A, Salinas I, Castella E, Sanmarti A. Frozen Section in a cytological

diagnosis of thyroid follicular neoplasm. The Laryngoscope, 113: 563-6, Mar 2003. 9. Nygaard B, Hegedus L, Gervil M, Hjalgrim H, Soe-Jensen P, and Hansen JM.

Radioiodine treatment of multinodular non-toxic goitre. British Medical Journal, 307(6908): 828-832, Oct 1993.

10. Papini E, Petrucci L, Guglielmi R, Panunzi C, Rinaldi R, Bacci V, Crescenzi A, Nardi F, Fabbrinni R and Pacella CM. Long-term changes in nodular goiter: a 5-yr prospective randomized trial of Levothyroxine suppressive therapy for benign cold thyroid nodules. Journal of Clinical Endocrinology and Metabolism, 83(3): 780-3, 1998.

11. Petrone, Louis R. A Primary Care Approach to the Adult Patient with Nodular Thyroid Disease. Archives of Family Medicine 5(2): 92-100, Feb 1996.

12. Samuels MH. Evaluation & Treatment of Sporadic Nontoxic Goiter-Some Answers & More Questions (editorial). Journal of Clinical Endocrinology and Metabolism, 86(3): 994-7

13. Shah, S. Atlas of Clinical Oncology Center of the head and Neck. BC Decker Inc. Hamilton London 2001.

14. Shaha, A. Controversies in the Management of Thyroid Nodule. The Laryngoscope, 110: 183-93, Feb 2000.

15. Thawley, et al. Comprehensive Management of Head and Neck Tumors. Vol 2. W.B. Saunders Company, 1999.

16. Thyroid Carcinoma Task Force. AACE/AAES Clinical Practice guidelines for the diagnosis and management of thyroid nodules. Endocrinology Practice 2(1): 80-4, Jan/Feb 1996.

17. Thyroid Carcinoma Task Force. AACE/AAES Medical/surgical guidelines for clinical practice: management of thyroid carcinoma. Endocrinology Practice, 7(3): 203-20, May-June 2001.

18. Wesche, MFT, Tiel-v Buul MMC, Lips P, Smits NJ and Wiersinga WM. A Randomized Trial Comparing Levothyroxine with Radioactive Iodine in the Treatment of Sporadic Nontoxic Goiter. Journal of Clinical Endocrinology and Metabolism, 86(1): 998-1005. Oct 2000.

19. Zelmanovitz F, Genro S and Gross JL. Suppressive therapy with levothyroxine for solitary thyroid nodules: A double-blind controlled clinical study and cumulative meta-analyses. Journal of Clinical Endocrinology and Metabolism, 83(11): 3881-5, 1998.

40

UP-PGH DEPARTMENT OF OTORHINOLARYNGOLOGY CLINICAL PRACTICE GUIDELINES CLEFT LIP, ALVEOLUS AND PALATE (CLAP) IN CHILDREN AND ADOLESCENTS

SCOPE OF THE PRACTICE GUIDELINE This clinical practice guideline is for use by the Department of Otorhinolaryngology of the College of Medicine - Philippine General Hospital, University of the Philippines Manila in cooperation with the other members of the multi-disciplinary CLAP team. It provides a systematic approach to the diagnosis and management of cleft lip and palate deformities in newborns up to adolescents. OBJECTIVES The objective of the guideline is to provide a medically-inclined and family-centered care for children and adolescents with cleft lip and palate that employs a multi-disciplinary team, working together at all times throughout the growth of the child. LITERATURE SEARCH The National Library of Medicine’s Pubmed database was searched for literature using Boolean method on medical subject headings: cleft lip MeSH and cleft palate MeSH; middle ear ventilation MeSH; palatal prosthesis MeSH randomized clinical trial MeSH, controlled clinical trials MeSH, Meta-Analysis MeSH; Epidemiologic Studies MeSH. Specific clinical questions were answered using the medical subject headings. There were 22 researches used in the guideline development. Ten different protocols from international institutions were used as patterns for the formulation of the clinical practice guideline. The chosen articles were divided as follows:

Meta-analysis 0 Randomized controlled trial 5 Non-randomized controlled study 0 Descriptive study 21 Committee report 10

DEFINITION

Cleft lip and palate is a congenital anomaly presenting in wide varieties of forms and combinations. It is failure of fusion of embryonal facial clefts. Cleft lip ranges from notching of the lip to a complete cleft, involving the floor of the nose. It may be associated with a cleft of the primary palate (alveolus/pre-maxilla) and with clefts of the secondary palate (hard and soft palate). Cleft lip can further be described as unilateral or bilateral, complete or incomplete.

10,33

Cleft palate may occur in isolation ranging from a bifid uvula to a complete cleft of hard and soft palate. It may also present in a submucous form. It can also be classified as unilateral or bilateral. Clefts may be part of several syndromes affecting the first and second branchial arches, including the Pierre Robin sequence.

10,33

CLAP team is made up of the following:

OTORHINOLARYNGOLOGIST (Cleft Surgeon)

Performs plastic and reconstructive surgery on the cleft lip and the palate including scar revision and rhinoplasty

Prevents and manages otologic infections PEDIATRICIAN

Oversees the well-being of the child including the normal growth and development

Ensures that the child is physically fit for the surgical procedures PEDIATRIC DENTIST / PEDODONTIST

Takes care of the child’s dentition ORTHODONTIST

Helps to establish proper shape to the dental arches

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Works to achieve a normal dental arch prior to bone graft surgery and maintain its integrity

PROSTHODONTIST

Provides the prosthetic devices (e.g. obturator, bridge, retainer, implant) AUDIOLOGIST

Checks the child's hearing regularly and makes the necessary recommendations SPEECH AND LANGUAGE PATHOLOGIST

Assists the child in producing intelligible language

Provides therapy in areas of articulation and language development depending upon the child's unique needs

GENETICIST

Helps the family gain an understanding of the predisposing factors and determine risk of recurrence

MEDICAL SOCIAL WORKER

Assists the family in dealing with non-medical issues that will affect the over-all treatment of the child and the family

Provides access to appropriate resources and support PSYCHOLOGIST

Works with the child, parents and the family to ensure normal functioning

Provides intervention on issues such as parental adjustment and cleft child self-esteem

PARENTS’ SUPPORT GROUP

Establishes the link between families and the CLAP team for a better understanding of the care pathways

PREVALENCE Cleft lip and palate represents the second most frequently occurring congenital deformity. The incidence of cleft lip and palate varies considerably according to race. The incidence among Caucasians is 1:1000 live births, while American Indians is 3.6:1000 live births. The incidence for Asians is slightly higher, Japanese 2.1:1000 live births and Chinese, 1.7: 1000 liver births.

4

According to a census by the Philippine Birth Defects Registry Project from 1999-2001, cleft lip and palate is the third most common birth defect in the Philippines (first is multiple congenital anomalies, second is ankyloglossia). A total of 110 cases of cleft lip and palate were tallied, 5.6:10,000 live births. In a census done in PGH from 1996-2000, there were 378 cases of bilateral cleft lip (associated cleft palate not specified), 208 cases of cleft lip with palate and 188 cases of cleft lip alone. In 2002, an average of 21 CLAP patients per month was seen at the ORL outpatient clinic of the Philippine General Hospital. Four to eight cleft operations per month were performed.

CLAP OPD CENSUS FOR 2002

CLAP, type not indicated 5

CLAP, bilateral, complete 11

CLAP, unilateral, complete 16

CLAP, incomplete, bilateral 2

CLAP, incomplete, unilateral 1

Cleft lip, unilateral complete 2

Cleft lip, unilateral, incomplete 2

Cleft lip, bilateral, complete 1

Cleft lip, bilateral, incomplete 1

Cleft palate, unilateral incomplete 13

Cleft palate bilateral, complete 1

Cleft palate, bilateral, incomplete 2

42

Complete cleft lip and alveolar cleft 3

Incomplete cleft lip and alveolar cleft 1

CLAP complete in 1 side and incomplete palate on the other side

4

Incomplete cleft lip and alveolar cleft on one side and incomplete cleft lip On the other side

1

Total OPD Census: 66

CLAP OR CENSUS FOR 2002

Cleft lip, incomplete; cleft palate, complete 1

Cleft lip, incomplete; cleft palate, incomplete

4

Cleft lip, complete; cleft palate, complete 19

Cleft lip, complete; cleft palate, incomplete 1

Cleft palate, incomplete 14

Cleft palate, complete 2

Cleft lip, incomplete 2

Cleft lip, Complete 7

Total OR Census: 50

RECOMMENDATIONS ON THE DIAGNOSIS OF CLEFT LIP AND PALATE Initial Head and Neck Examination The head is inspected for symmetry. The auricle and the external ear canal are checked for development and location. A facial analysis is helpful to identify abnormalities of facial symmetry and harmony. Otologic examination includes pneumatic otoscopy and tuning fork tests. Cleft palate is commonly associated with Eustachian tube dysfunction due to an abnormal insertion of the levator and tensor veli palatini muscles in the posterior margin of the hard palate. Anterior and posterior rhinoscopy will identify clefting, septal abnormalities, intranasal masses and choanal atresia. Oral cavity examination will reveal any cleft, dental arch abnormalities and tongue anomalies such as bifid tongue, macroglossia, glossoptosis, or lingual thyroid. In addition, malocclusion, hemifacial hypertrophy or atrophy, and facial clefting are documented. The upper airway tract is evaluated by assessing the adequacy of phonation, cough, and deglutition, and by auscultating and palpating the neck.

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Nomenclature Cleft lip and palate patients will be classified according to the Thallwitz nomenclature and ICD-10 system. The Thallwitz nomenclature

34 (also known as the LAHSHAL) is a descriptive classification since

site, size, extent and type of cleft are considered. Severity of the deformity is objectively documented and the recorded findings can easily be stored into a computer for data analysis. Each area is divided into thirds, and cleft defects are graded as to extent of affected areas. Grading is done for both sides as shown:

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(right side) (midline) (left side)

L-lip A-alveolus H-hard palate S-soft palate H-hard palate A-alveolus L-lip

L = Lip - 1/3 or 2/3 or 3/3 A = Alveolar cleft - 1/3 or 2/3 or 3/3 H = Hard palate cleft - 1/3 or 2/3 or 3/3 S = Soft palate cleft - 1/3 or 2/3 or 3/3

The ICD-10 system is an international standard of coding.

Cleft hard and soft palate with cleft lip, bilateral Q374 Cleft hard and soft palate with cleft lip, unilateral Q375 Cleft hard palate with cleft lip, bilateral Q370 Cleft hard palate with cleft lip, unilateral Q371 Cleft hard palate with cleft soft palate, bilateral Q354 Cleft hard palate with cleft soft palate, unilateral Q355 Cleft hard palate, bilateral Q350 Cleft hard palate, unilateral Q351 Cleft lip Q36 Cleft lip, bilateral Q360 Cleft lip, medial Q361 Cleft lip, unilateral Q369 Cleft palate Q35 Cleft palate with cleft lip Q37 Cleft palate, medial Q356 Cleft palate, unspecified, bilateral Q358 Cleft palate, unspecified, unilateral Q359 Cleft soft palate with cleft lip, bilateral Q372 Cleft soft palate with cleft lip, unilateral Q373 Cleft soft palate, bilateral Q352 Cleft soft palate, unilateral Q353 Cleft uvula Q357

RECOMMENDATIONS ON THE MANAGEMENT OF CLEFT LIP AND PALATE BIRTH – 6 MONTHS 1. Registry in the Congenital Registry of the National Institutes of Health Grade C Recommendation

Cleft lip and palate ranks third among the top ten birth defects for 1999-2001 as reported by the Philippine Birth Defects Registry Project. Data gathered is used for researches. Moreover, these can be used in policy making and in recommending services needed by patients with congenital anomalies.

2. Speech development Grade C Recommendation

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Parents will be educated on normal speech and language development and they will be taught vocal play.

3. Evaluation of feeding and growth parameters Grade C Recommendation

This is a concerted effort from rehabilitation medicine, pediatrics, and prosthesis to improve patient’s feeding. Turner et al

32 noted that the combined use of palatal obturator and lactation

education reduced feeding time, increased volume intake and was associated with good growth. Mothers who had desired to breast-feed elected to use the obturator to support high volume intake, and decrease infant fatigue. Feeding instructions, molding appliance fitting and feeding plate modification are done. A study by Konst

21 showed that children treated with intra-oral prosthesis during their first year

of life followed a more normal path of phonological development between 2 and 3 years of age

32. Infant orthopedics or intraoral prosthesis has a temporary effect on maxillary arch

dimensions, which does not last beyond surgical soft palate closure.28

4. Parental education and support Grade C Recommendation Psychiatric consultation and support is started. 5. Otologic evaluation Grade A Recommendation

Evaluation of hearing status including newborn hearing screening is important. Aggressive otologic management has been recommended for children with cleft palate because of the almost universal occurrence of otitis media with effusion (OME) and the association of OME with hearing loss and possible language, cognitive, and academic delays.

5 An otoacoustic

emission test (OAE) or an auditory brainstem response (ABR) test is used as hearing screening in newborn with cleft lip and palate

31.

A study by Ceponiene

7 showed that reduced mismatch negativity (MMN) amplitudes in cleft

children imply deficiency in auditory STM trace maintenance. This dysfunction is likely to contribute to their language and learning disabilities.

6. Genetic evaluation Grade C Recommendation

Other congenital defects and syndromes are identified and treat accordingly (e.g. airway obstruction in Pierre Robins sequence).

7. General pediatric consultation for routine immunization and well baby check-ups.

8. Monthly follow-up with the CLAP team at the Department of ORL.

6 MONTHS – 1 YEAR 1. One-stage operation for unilateral cleft lip and cleft palate

Single stage modified Millard with alar plasty and two-flap palatoplasty Optional procedures: Alveolar bone grafting Ventilation tube insertion Grade C Recommendation

A one-stage operation for lip and palatal defect from 6 to 9 months of age is recommended since indigent patients have difficulty in following up regularly for multi-staged procedures (often due to financial and geographic constraints). Isaac Kaplan

18 performed the earliest

record of a simultaneous surgery for cleft lip and cleft palate in 1972. His study includes 13 infants who underwent one stage surgery for cleft lip and cleft palate at the 3

rd to 4

th month of

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age. The one staged procedure shortcuts the classical recommendation of repairing the cleft lip at 3

rd months of age followed by closure of the palate by 6

th months of age.

Controversies abound the timing of surgery for patients with cleft lip and cleft palate. In terms of speech development, palatal defects closed before the “babbling” of words would reasonably prevent hypernasal speech caused by the escape of air through the naso-oral fistula. Kirschner

20 compared 40 children with surgery done at 3 to 7 months of age with 50

children whose surgery was done after 7 months of age. The study revealed no significant difference in speech outcome between the 2 groups. Infants subjected to mid-facial surgery have a greater risk of impaired maxillary growth. However, Choudhary

8 demonstrated satisfactory outcomes on long-term midfacial growth

using Veau-Wardill-Killner technique for cleft palate done at 9 to 12 months of age. Regarding the choice of surgical technique, Holtmann and Wray

15, in a prospective

randomized study, compared triangular cleft lip repair with the Millard rotation-advancement procedure. The most significant difference between the two approaches was a greater frequency for hypertrophic scars formation following the rotation-advancement repair. However, the hypertrophied scar resolved 18 months after surgery in majority of patients. They found no major differences in the appearance of the lip and nose. Both surgeons felt the rotation-advancement repair allowed better “fine tuning” during surgery as compared to the triangular flap repair. Zheng et al

36 reported that tympanotomy and pressure equilibrium tube insertion during

palatoplasty resolved 48.7% of the ears with otitis media with effusion (OME). Furthermore, the hearing level improved by about 17decibels six months after the operation. These results were supported by the study of He et al

14 wherein they compared the influence of

palatoplasty and tympanotomy on middle ear function (hearing condition) in cleft palate patients. They recorded the pre- and post-operative hearing levels and middle ear function of two groups: (1) 22 ears with OME and cleft palate with VT tube insertion and (2) 38 patients with cleft palate who underwent palatoplasty. They reported that VT tube insertion and tympanotomy changed the pressure conditions of the middle ear cavity, raising the hearing level to about 17decibels in the middle-ear-diseased cleft palate patients. The patients who had palatoplasty alone did not show obvious changes in middle ear function. Although arm restraints have been traditionally used after cleft surgery, several authors have debunked this practice. O’Riain

25 did not find any case where straying fingers caused

disruption of the suture line in his study of 24 cleft lip repairs. Similarly, in a prospective, randomized trial of 46 children having primary cleft palate repair, Jigjinni et al

16 showed that

arm splints did not decrease the incidence of oro-nasal fistulas. Breast-feeding has been almost universally discouraged in infants undergoing cleft lip repair on the basis that muscular activity will jeopardize the surgical repair. Thus, the practice of cup/spoon/dropper feeding has been unchallenged by succeeding generations of plastic surgeons. Darzi, Chowdri and Bhat

11 performed a prospective randomized study to

determine whether breast-feeding in the early post- operative period would in any way be harmful for the child with cleft lip repair. 40 mothers were randomized into two groups, 20 for breast-feeding and 20 for spoon-feeding. Cleft lips were repaired by either a triangular flap technique or by a rotation-advancement technique. They had only one partial wound dehiscence in a spoon-fed baby who fell from his bed on the third post-op day.

2. Initiation of intensive speech therapy after single stage CLAP repair

3. Continuation of general pediatric follow-up and nutrition evaluation 4. Dental evaluation and care during primary tooth eruption

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5. Follow-up every 3 months with the CLAP team at the Department of ORL

1 YEAR – 5 YEARS 1. Psychosocial assessment Grade C Recommendation

This is the stage when body image and self-esteem are developing. Intervention of a child psychiatrist may be necessary. Erik Eriksson calls this the “autonomy versus shame” stage. Shame occurs when the child is overtly self-conscious because of negative exposure. Cleft lip and palate children are often teased for their deformity. Psychological help is needed to lessen insecurity and to promote a healthy body image.

2. Routine pediatric care for children entering school

3. Articulatory and phonetics rehabilitation before school at 1-4 yrs old Grade C Recommendation

A child usually utters his or her first word at 12 months of age. Logically, it would be beneficial to rehabilitate the patient after surgery, at the time when the child is starting to develop language skills.

4. Assessment for 2

nd stage procedures at 3-4 yrs of age

Grade C Recommendation Velopharyngeal competency is assessed and pharyngoplasty is done if incompetent. Collumellar lengthening may be done for bilateral cleft The importance of normal speech for successful socialization cannot be overestimated. Surgery that simply restores normal palatal anatomy cannot be considered a success if the result is crippled speech. The rightful goal of cleft palate repair is normal velopharyngeal function. Persistent velopharyngeal defect (VPD) after cleft palate repair is characeterized by hypernasal resonance and nasal air escape. Significant VPD may be associated with the development of compensatory errors in articulation that may further impair speech intelligibility.

5. Dental and orthodontic follow-up. 6. Follow-up every 6 months with the CLAP team at the Department of ORL.

8 – 10 YEARS 1. Alveolar bone grafting Grade C Recommendation

The alveolar bone graft in an alveolar cleft has many advantages, namely: (1) assists in the closure of buccoalveolar oronasal fistula; (2) provides bony support for unerupted teeth and teeth adjacent to the cleft; (3) forms a continuous alveolar ridge to facilitate orthodontic correction of malocclusion; (4) supports the nasal floor and the base of the alae to improve nasal aesthetics. According to Bergland

3, the best time to do secondary bone grafting is from

9-11 years of age when the canine tooth has not erupted. Deeb12

noted a most favorable prognosis for patients with alveolar graft when there is a ¼ - ½ canine root formation (9-12 years of age).

2. Orthodontic re-evaluation and treatment

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3. Yearly follow-up

4. Higher level language and speech class 5. Continued psychiatric support

16 – 19 YEARS 1. Orthognathic surgery for malocclusion and prognathism Rhinoplasty for aesthetic purposes 1 year after orthognathic surgery Aesthetic surgery Grade C Recommendation

Primary and secondary management of cleft lip and palate in the adolescent patients vary from one center to another. Treatment is often individualized. Patient, parental and physician expectations should be discussed. Concerns regarding secondary surgeries – conspicuous lip scars, unnaturally wide central lip, flaring alae with wide nostril floors, short columella with flat nasal tip – are addressed. The deformity may present with varying degrees of midface retrusion, malocclusion, nasal deformity and lip deformity

17. Secondary and residual

dentofacial deformities should be managed by surgical and orthodontic therapy.27

Cumulative operative procedures can be done in adolescents (at least 14 years old). Surgeries performed include pharyngoplasties (38%), alveolar grafting (79%), Abbe flaps (10%) and orthognathic surgery (13.8%). Patients also undergo lip revisions and secondary nasal operations.

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Long-term results of different protocols vary from center to center. For purposes of documentation and outcome analysis, a standardized video recording to assess cleft surgery outcome has been suggested.

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2. Development of interpersonal relationships including the opposite sex Career planning and goals for higher education Grade C Recommendation

Emotional effects and psychological aspects of cleft lip and palate deformity and surgery must be considered. Overgeneralization should be avoided and treatment must be individualized.

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REFERENCES 1. Anteunis, LJ., Brienesse, P., Schrander, JJ., Otoacoustic emissions in screening cleft lip and palate

children for hearing loss- a feasibility study. International Journal of Pediatric Otorhinolaryngology.

1996, Aug, 44(3): 259-66. 2. BAOMS Cleft lip and palate. http://www.baoms.org.uk/ce/ce_cleft.html 3. Bergland O., Semb G. Abyholm F.,Elimination of the Residual Alveolar Cleft by Secondary Bone

Grafting and Subsequent Orthodontic Treatment. Cleft Palate Journal. July 1986. 23:3 pp 175-204.

4. BRISTOL Cleft Lip and Palate Treatment Schedule. http://www.bristol.org.uk/_cleftlip.html 5. Broen, PA, Moller, KT, Caristrom, J, Doyle, SS., Devers, M. Keenan, KM, Comparison of hearing

histories of children with and without cleft palate. Cleft Palate-Craniofacial Journal 1996, March. 33(2): 127-33.

6. Canady JW.. Emotional effects of plastic surgery on the adolescent with a cleft. Cleft Palate Craniofacial Journal. 1995 Mar, 32(2): 120-4.

7. Ceponiene, R., Hukki, J. Choir, M. Haapanen, ML., Ranta, R. Naatanem, R., Cortical Auditory dysfunction in children with oral clefts: relation with cleft type. Clinical Neurophysiology, 1999 Nov,

110(11): 1921-6.

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8. Choudhary, S., Cadier, M., Shinn, D., Shekhar, K., McDowall, R. Effect of Veau-Wardill-Killner Type of Cleft Palate Repair on Long-Term Midfacial Growth. Plastic and Reconstructive Surgery. February 2003; 576-585.

9. Cohen, et. al.. Cumulative operative procedures in patients aged 14 and older with unilateral and bilateral cleft lip and palate. Plastic Reconsructivet Surgery, 1995 Aug 96(2):267-71.

10. Cummings, C., Fredrickson, J., Harker, L. Pediatric Otolaryngology Head and Neck Surgery. 3rd

Ed. Mosby-Yearbook. 1998; 139.

11. Darzi MA, Chowdri A, Bhat AN. Breast-feeding or spoon-feeding after cleft lip repair: a prospective randomized study. British Journal of Plastic Surgery, 1996; 49: 24-26.

12. Deeb, Messer, Lehnert, Hebda, Waite. Canine eruption into grafted bone in Maxillary alveolar cleft deffects. Cleft Palate Journal, Jan 1982, Vol 19. No.1 pp112-115.

13. Del Mundo, Estrada, Santos Ocampo, Navarro, Textbook ofPediatric and Health Care, 3rd

Edition, 1990.

14. He Y, Xu H, Zhen Q, Liao X, Zhen Y. [The influence of palatoplasty and tympanotomy on middle ear function in cleft palate patients]. Hua Xi Kou Qiang Yi Xue Za Zhi, Aug 2001; 19(4):243-5.

15. Holtmann B, Wray R. A randomized comparison of triangular and rotation-advancement unilateral cleft lip repairs. Plastic and Reconstructive Surgery; Feb 1983: 172-1983.

16. Jigjinni V, Kagesu T, Sommerlad BC. Do babies require arm splints after cleft palate repair? British Journal of Plastic Surgery, 1993; 46: 681-5.

17. Kai S, Chishi M, Secondary correction of the cleft lip and nose deformity: a new technique for revision of whistling deformity. Cleft Palate Journal. 1985 Oct 22(4): 290-5.

18. Kaplan, I., Dresner, J., Chava,G. Radin,L. The Simultaneous Repair of Cleft Lip and Palate in the Early Infancy. British Journal of Plastic Surgery. 1974; 134-138.

19. Kaplan, Saddok, Synopsis of Psychiatry, 7th

Edition, 1994 20. Kirschner, R., et. Al. Cleft Palate Repair at 3 to 7 Months of Age. Plastic and Reconstructive Surgery.

May 2000; 2127-213. 21. Konst EM, Rietveld T, Peters HF, Prahl-Andersen B. Phonological development of toddlers with

unilateral cleft lip and palate who were treated with and without infant orthopedics: A randomized clinical Trial. Cleft Palate Craniofacial Journal, Jan 2003 40(1); 32-9.

22. 18.Leslie,J, Penko, M., Rode, H., Cognition, communication, and Hearing in young children with cleft lip and palate and in control children: A longitudinal study . American Academy of Pediatrics, 1996, April 97(4):529-534.

23. Maryland Society for Cleft Lip and Palate Children. http://www.msclpc.org/interdisciplinary_care.htm 24. Morrant and Show. Use of standardized video recordings to assess cleft surgery outcome. Cleft Palate

Craniofacial Journal. 1996 Mar 33(2); 134-42. 25. O’Riain S., Cleft lip surgery without post-operative restraints. British Journal of Plastic Surgery. 1977;

30: 140-1. 26. Olav Bergland, Gunvor Semb, Frank Abyholm. Elimination of the Residual Alveolar Cleft by Secondary

Bone Grafting and Subsequent Orthodontic Treatment. Cleft Palate Journal. July 1986. 23; 3. 175-204.

27. Posnick JC. Clin Plast Surg 1997; 24(3): 583-97. 28. Prahi, Kuijpers-Jagtman,AM, Van’t Hof, MA., Prahi-Andersen B., A randomized prospective clinical trial

into the effect of infant orthopedics on maxillary arch dimensions in unilateral cleft lip and palate. European Journal of Oral Sciences 2001, Oct, 109(5):297-305.

29. RUSH Department of Plastic and Reconstructive Surgery. Treatment for Cleft Lip and Palate. http://www.rush.edu/patients/plastissurgery/craniofacial/cleftlip.html

30. SMILES Overview of Cleft Lip and Palate Management, http://www.cleft.org/progression/newborn.htm 31. Tropper, G., Moran, L., Odell, P., Durieux-Smith, A., The contribution of brainstem electric response

audiometry (BERA) to the evaluation and management of infants with cleft palate. Journal of Otolaryngology, 1988 April, 17(2): 103-10.

32. Tumer, L., Jacobsen, C., Humenkzuk, M. Singhal, VK., Moore, D., Bell, H. The effects of lactation education and a prosthetic obturator appliance on feeding efficiency in patients with cleft lip and palate. Cletf Palate-Craniofacial Journal. 2001, Sept. 38(5): 519-24.

33. UCLA Treatment Protocol: Cleft Lip and Palate. http://www.uclaplasticsurgery.com/r_cf.html 34. Wandner, H. Dissertation: “Computergestützte Dokumentation von Patienten mit

Lippen-Kiefer-Gaumensplaten”; Aus der Klinikru Mund-, Kiefer- und Gesichtschirirgie/Plastische, Operationen des Universitatsklinkums Charite; May 1997

35. Yi Xue Za Zhi. Alveolar Bone Grafting in unilateral complete cleft lip and palate patients. BNrJ Plast Surg. 1996 Jan; (49); 24-6.

36. Zheing Q, Xu H, He Y. [Effects of tympanotomy and pressure equilibrium tube insertion during palatoplasty on prognoses of Otitis media with effusion]. Hua Xi Kou Qiang Yi Xue Za Zhi, Feb 2003;

21 (1):28-30.

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CLEFT LIP AND PALATE

0-6 months

Initial evaluation and documentation

Fabrication of intra-oral appliance with monthly modification and feeding instruction

Otologic evaluation and examination

Vocal play and initial parent counseling on speech

General pediatric evaluation and immunizations

6-12 months

CLAP surgery

Single stage modified Millard with alarplasty

Two flap palatoplasty with optional alveolar bone grafting

Possible VT tube insertion

Intensive speech therapy after single stage CLAP repair

General pediatric follow-up and nutrition evaluation

Dental evaluation and care during primary tooth eruption

1-5 years old

Secondary problem assessment and correction o Correction of velopharyngeal insufficiency (pharyngoplasty) o Minor lip revision o Closure of fistula

Psychiatric evaluation regarding body image issues

General pediatric follow-up

Speech therapy

Articulation and phonetic classes- consonant pronunciation

8-10 years old

Alveolar bone grafting

Orthodontic follow-up

Speech therapy – higher level language and speech classes

16-18 years old

Orthognathic Surgery

19 years old

Aesthetic Surgery

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UP-PGH DEPARTMENT OF OTORHINOLARYNGOLOGY CLINICAL PRACTICE GUIDELINES TRACHEOSTOMY AND DECANNULATION

SCOPE OF THE PRACTICE GUIDELINE This clinical practice guideline is meant for use by the Department of Otorhinolaryngology of the College of Medicine - Philippine General Hospital, University of the Philippines Manila. It covers tracheostomy and decannulation in children and adults. OBJECTIVES The objectives of the guideline are: (1) to discuss the indications and contraindications of tracheostomy; (2) to identify signs and symptoms of decannulation failure; and (3) to set criteria for decannulation.

LITERATURE SEARCH

Textbooks in Otorhinolaryngology were reviewed to contribute to the baseline knowledge on tracheostomy and decannulation, which was later expanded to include journals and publications. A MEDLINE database search with a Boolean combination tracheostomy or tracheotomy and decannulation was done. The search was limited to articles involving humans and those published in English in the last thirty years. Full text articles were obtained when possible. The search yielded 38 articles divided as follows:

Meta-analysis - 0 Randomized controlled trials - 0 Non-randomized controlled study – 1 Well-designed controlled study without randomization - 2 Well-designed descriptive study - 23 Committee report - 12

TRACHEOSTOMY DEFINITION Tracheostomy or tracheotomy is an opening surgically created through the neck into the trachea. A tube is usually placed through this opening (tracheostomy tube) to provide airway, and to allow removal of secretions from the lungs

3.

Tracheotomy, strictly defined, refers to the incision made in the trachea. Tracheostomy,is the creation of a stoma through which air may pass to the lungs, by-passing through the upper airway. Oftentimes both words are used interchangeably to mean a temporary opening in the anterior neck into the trachea

4,5,6.

PREVALENCE OF TRACHEOSTOMY In the Department of Otorhinolaryngology of the Philippine General Hospital, there were 107 referrals for tracheostomy in 2002. Ninety-one were adults and more than half (47/91) were over 60 years old. Fifty percent of the 16 pediatric referrals were less than one year old. There were 59 males and 48 females. The most common indications were prolonged intubation secondary to pneumonia and cerebrovascular disease. A total of 65 tracheostomies were done on patients admitted at Ward 10, 60 of the patients were adults. They were commonly due to laryngeal masses followed by oral cavity tumors and anterior neck masses. In the years prior to 1960, the incidence of pediatric tracheotomy in the United States was 1 per 1000. It has risen to 2.7 per 1000 pediatric admissions

7.

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RECOMMENDATIONS ON TRACHEOSTOMY 1. Tracheostomy is recommended in disease conditions with upper and lower airway

patency impairment. Grade B Recommendation

Current indications for tracheotomy include the group of diseases connected with upper airways patency impairment, general diseases affecting the patency of lower airways and in patients requiring prolonged intubation and mechanical ventilation

1, 8, 10, 19, 20, 21, 29, 31, 32, 33, 38, 39

1.1 Upper airways patency impairment 1.1.1 Congenital malformations of the larynx and trachea (congenital infraglottic

laryngeal constriction, laryngomalacia, laryngeal fins, laryngeal cyst, congenital vocal cord paralysis)

1.1.2 Congenital facial skeleton anomalies including micrognathia and microglossia 1.1.3 Mechanical, thermal, chemical, and iatrogenic trauma of larynx and trachea

(laryngeal tracheal fracture, foreign body wedging or obturating laryngeal lumen, thermal or corrosive burns, prolonged or traumatic intubation)

1.1.4 Maxillofacial trauma with severe edema of soft tissues and falling back of the tone

1.1.5 Acute laryngeal edema precluding intubation (inflammatory or allergic) 1.1.6 Tumors of the larynx or trachea (hemangioma, neoplastic) 1.1.7 Bilateral vocal cord paralysis 1.1.8 Laryngeal and tracheal compression by adjacent structures (vascular

anomalies, cervical tumors) 1.2 Respiratory tract patency impairment due to general disease

1.2.1 Retention of secretion in lower airway (balance impairment between secretion and elimination

1.2.2 Lack of cough reflex 1.2.3 Impaired function of esophageal constrictors and/or vocal folds (saliva or

gastric acid and esophageal contents penetrating the bronchial tree) 1.3 Patients requiring chronic mechanical ventilation 1.3.1 CNS diseases --- long term unconsciousness or coma

1.3.2 Metabolic disorders 1.3.3 Systemic diseases 1.3.4 Chronic diseases of pulmonary tissue 2. Tracheostomy should be performed in both adult and pediatric patients requiring

prolonged intubation. In adults, tracheostomy should be considered after an intubation period of 7 days. In children, the decision for tracheostomy should be individualized as they are able to tolerate longer periods of intubation.

Grade B Recommendation

Prolonged ventilation still remains as the most common indication 11,37

. There is some agreement but no consensus that a period of approximately 7 days is a reasonable time in adults for a decision to continue intubation or change to a tracheostomy. Clinical observations have shown that intubation as long as 7 days is followed by complications in 37% of cases. Several studies have shown that a 7-10 day period for adults is acceptable, after which prolongation of intubation is accompanied by an increase in incidence of laryngotracheal complications

11, 37, 39. Seventeen recent reports suggest that adults can tolerate endotracheal

intubation up to two weeks without developing permanent laryngotracheal complications 39

.

Newborns can go for a longer period of time (more than 50 days) without suffering adverse effects because the risk for subglottic stenosis is low. Other factors such as underlying systemic disease, low birth weight and endotracheal tube size are also influential factors

15, 16.

In pediatric patients (more than 1 year old), safe prolonged intubation ranges from 30-60 days

15. Their tolerance for prolonged intubation is more like that of neonates than adults

15.

52

In the last decade, the number of cases of tracheostomy has increased due to the development of new intensive care units with the use of mechanical ventilation and increasing number of patients needing prolonged ventilation support

12. There is a noted

increase in frequency of tracheostomies performed for subglottic and tracheal stenosis, respiratory papillomatosis, caustic alkali ingestion and craniofacial syndromes. Less common indications are subglottic hemangioma and laryngeal clefts

11. Carron et al

4 concluded that

neurological impairment and prolonged ventilation formed the major proportion of the pediatric tracheostomies, 28% and 26% respectively.

3. Tracheostomy should be performed with caution in patients with severe bleeding

tendency and unstable cervical spine. Grade B Recommendation

There are no absolute contraindications to tracheostomy. Severe bleeding tendency and unstable cervical spine are relative contraindications

29,39.

4. An evaluation for signs and symptoms of both early and late complications of

tracheostomy, which may occur from the intra-operative period up to decannulation should be undertaken.

Grade B Recommendation

Complications in the tracheostomy can occur anytime from the intra-operative period up to decannulation.

Tracheostomy may result in early and late complications

5, 6, 8, 13, 14, 16, 17, 19, 21, 23, 26, 29, 30, 32,33, 34,

35, 38, 39

4.1 Early complications of tracheostomy (less than 7 days) 4.1.1. Subcutaneous, mediastinal, or pleural edema 4.1.2. Subcutaneous emphysema 4.1.3. Aspiration 4.1.4. Cardiac arrest 4.1.5. Recurrent laryngeal nerve paralysis 4.1.6. Acute hemorrhage or bleeding 4.1.7. Sudden saturation decrease and breathing impairment 4.1.8. Infections 4.1.9. Dysphagia 4.1.10 Intraoperative tracheoesophageal fistula 4.1.11 Pneumothorax or pneumomediastinum 4.1.12 Tube obstruction or displaced tracheostomy tube.

4.2 Late complications of tracheostomy. These appear some time after the procedure is

done. These may be life-threatening or may impair decannulation. 4.2.1 Sudden breathing impairment due to lack of tracheostomy tube patency (mucus

plug 4.2.2 Tracheostomy infections 4.2.3 Recurrent lower airway infections 4.2.4 Bleeding, hemorrhage or rupture of innominate artery 4.2.5 Infraglottic stenosis 4.2.6 Tracheal cartilage intussusception above the stoma 4.2.7 Tracheo-esophageal or tracheo-cutaneous fistula after decannulation 4.2.8 Growth of granulation tissue around the stoma and in the trachea

The most frequent early complications are pneumomediastinum, pneumothorax, wound complications like infections and bleeding

8, 14, 20, 35. The most frequent causes of

tracheostomy-related death are cannula obstruction and accidental decannulation 14

. Early or

53

perioperative complication rates range from 0.1 to 22.9%. Late complications range from 0 to 37%

32. Overall, 77% of patients have one or more tracheostomy-related complications

3.

5 Suprastomal granulation tissue and tracheal stenosis are common late complications

of tracheostomy. When contemplating decannulation, endoscopic evaluation should be done in patients with prolonged periods of tracheostomy.

Grade B Recommendation

Granulation-formation and tracheal stenosis are common late complications8, 14, 20, 35

. Their occurrence necessitates a bronchoscopic examination to ensure that an adequate airway exists

2, 8, 24. In one center, a 20-year review of pediatric tracheostomy (1979-99) showed a

complication rate of as high as 46%, most commonly granulation tissue formation19

. In tracheostomies lasting more than 4 weeks, suprastomal granulation tissue occurs in 50% of pediatric patients

26.

DECANNULATION DEFINITION Decannulation refers to the removal of a cannula or the removal of the tracheostomy tube

36.

Decannulation marks the end of the tracheostomy management 25

. It is not uncommon for the practitioner to encounter problems associated with the decannulation of patients with tracheostomy. PREVALENCE OF DECANNULATION FAILURE The incidence of decannulation problems and failure are often isolated in most tracheostomies done among adults and their causes vary in each patient. This is not the case in pediatric patients. In a case series done by Midwinter, et al

38 of 89 tracheostomized children in their

center, they encountered decannulation problems in 17% (15/89) of the patients. An earlier study by Sasaki, et al

28 reported decannulation problems in 91% for children aged 6 months and below

and 23% for those 6 months to 12 years of age. This problem is noted to be significantly higher in the younger age group

9, 28, 38.

Failure of decannulation usually occurs within 12 to 36 hours after tube removal. Often, the underlying reason in such problems is failure to resolve the initial indication for tracheostomy prior to decannulation

2, 9, 25. Other reasons include the presence of associated problems in the

respiratory tract like an unresolved subglottic stenosis or the inability to clear secretions. Decannulation problems may also be related to the tracheostomy procedure itself or its possible sequelae such as tracheal stenosis, granulomas or functional disorders like tracheomalacia

7, 28.

Further evaluation by a specialist is necessary when there is failure of decannulation (Please refer to the algorithm). Endoscopic evaluation is preferred to assess the cause of decannulation failure

2,6,24. Several tests can be used to identify causes for decannulation problems like tracheal

stenosis, these include plain tracheal x-ray, tracheal tomogram, MRI, and high-resolution CT scan

23. For some cases, lateral x-ray including careful laryngoscopic examinations are not only

helpful but also essential to successful decannulation in these instances28

. Once the tracheostomy tube is removed, close observation is essential in a hospital setting for 24 hours as failure of decannulation may still occur

25. Failure of decannulation may occur as early as

12 hours and as late as 36 hours after tube removal 2, 25, 36

. Observation hospital setting is a must for at least 24-48 hours for pediatric patients (preferably ICU setting for less than two years of age)

24. Some even extend up to 7 days especially those whose residences are far from hospital,

or centers that can manage decannulation failure and complications24

.

54

RECOMMENDATIONS FOR DECANNULATION 1. Observe for respiratory distress once tracheostomy tube has been capped, since its

occurrence might indicate decannulation failure. Grade B Recommendation

When an adult patient cannot maintain adequate ventilation where a fenestrated tracheostomy tube has been capped over a 12 to 24 hour observation period

18, or when a pediatric patient

suffers respiratory distress when the tube has been blocked for 24 hours24

, then decannulation failure has occurred. The importance of knowing the indications for decannulation prior to undergoing the procedure is highlighted in the event that a patient cannot tolerate it, and thus, there is failure of decannulation. The signs and symptoms to watch out for include inadequate ventilation and respiratory distress. In adult tracheostomized patients, capping should be toleratedfor at least 24 hours before the doctors decides for decannulation

25. Patients are also taught to remove cap themselves if they

experience any breathing difficulty. For pediatric patients with non-fenetrated tubes, downsizing is initiated. Downsizing means changing to a smaller size, cuffless tube. A fenestrated tube can be inserted as an alternative if okay with primary doctor

25. The first tube change I always carried out by the doctor

26. Second

downsizing or more may be necessary25

. In some centers, one may replace the tracheostomy tube with a tube one size smaller each day until the smallest size usually 3.0 mm. (inside diameter) is used prior to decannulation

24. Some centers find it helpful to evaluate the subglittic

airway by bronchoscopy prior to decannulation. If the subglottic airway admits a bronchoscope that is no more than one size smaller than normal for the patient’s age, decannulation can be proceeded

24. After downsizing to the smallest tracheostomy tube and tolerated for 24 hours,

capping can be initiated. Once capped patient should be observed for 24 hours. Plugging for at least 24 hours is a must prior to decannulation

36. A speaker valve may also be used as a

transitioning tool between an open tracheostomy tube and plugging for decannulation. This allows the child to transition to using the upper airway for exhalation, reintroducing airflow and sensation, and easing the anxiety associated with plugging

36.

2. Decannulation should be carried out only in a stable patient where the initial indication

for has already been resolved. Grade B Recommendation 3. There should be no significant compromise of the airway either anatomically or

functionally prior to decannulation. Grade B Recommendation 4. Endoscopic findings should guide the decision to undergo decannulation. The entire airway, from the nose to the tracheo-bronchial tree should be evaluated for patency. Grade B Recommendation Successful decannulation is critical in tracheostomy management. The following criteria are

recommended to guide the practitioner in this procedure. These indications would be applicable to both adult and pediatric patients

2, 24, 25, 26, 36, .

4.1. Resolution of condition that required tracheostomy

27

The indication for tracheostomy should have been addressed prior to decannulation. The condition or particular diagnosis that required tracheostomy in the patient should be adequately controlled or effectively treated.

4.2 Patency of the Respiratory Tract The entire airway from the nose to the tracheobronchial tree should be evaluated for patency. Each part should be functioning well enough to support the respiratory needs of

55

the patient. A patent respiratory tract will allow respiration to continue adequately through the glottis while temporary occlusion of the fenestrated tube is done with the finger.

4.3 Stable Patient The condition of the patient as a whole should be stable. Problems in the other systems like cardiac, pulmonary, and central nervous system should be improving or unimpaired.

4.4 Protected Airway The patient must be able to protect the airway. There should be no significant compromise of the airway either anatomically or functionally. The patient must have the ability to clear secretions. In children, there should be no signs of aspiration during eating or drinking.

4.5 Endoscopic Findings Exhibiting Patent Airway Endoscopic findings should be near normal or at least the suprastomal airway is patent. Infrastomal airway evaluation should also be undertaken. This is most helpful for patients who are two years old or younger

2. Decannulation is recommended once the subglottic

airway admits a bronchoscope that is no more than one size smaller than normal for the patient’s age

24.

REFERENCES

. 1. Bach JR. Indications for tracheostomy and decannulation of tracheostomized

ventilator users. Monaldi Archives Chest Disease. 1995 May; 50 (3):223-7 2. Benjamin B., Curley WA. Infant Tracheostomy-endoscopy and decannulation.

International Journal of Pediatric Otorhinolaryngology 1990; 20:113-121. 3. Carr MM, Poje CP, Kingston L, Kielma D, Heard C, Complications of Pediatric

Tracheostomies, Laryngoscope 111: 1925-1928, Nov. 2001. 4. Carron JD, Derkay CS, Strope GL, Nosonchuk JE, Darrow DH. Pediatric

Tracheostomies: Changing Indications and Outcomes. Laryngoscope 2000; 110:1099-1104.

5. Chew JY, Cantrell RW, Tracheostomy, Complications and their Management. Archives of Otolaryngology-Vol 96;538-545, Dec. 1972.

6. Crysdale WS, Feldman R, Naito K, Tracheostomies: a 10 year Experience in 319 Children. Annals of Otology, Rhinology and Laryngology 97:1988.

7. Cummings CW et. al. Otolaryngology, Head and Neck Surgery. 3rd ed. 1998. Mosby-Year Book, Inc.

8. Freezer NJ, Beasley SW, Robertson CF, Tracheostomy. Archives of Otolaryngology 1972; 96: 538-545.

9. Gerson CR, Tucker CF, Infant Tracheotomy. Annals of Otology, Rhinology, Laryngology 91;413-416, 1982.

10. Guilleminault C, Simmons B, Motta J, Cummiskey J, Rosenkind M, Schroeder JS, Dement WC, Obstructive Sleep Apnea Syndrome and Tracheostomy. Archives of Internal Medicine-Vol 141, July 1981.

11. Hadfield PJ, Lloyd-Faulconbridge RV, Almeyda J, Albert DM, Bailey CM. The changing indications for pediatric tracheostomy. International Journal Pediatric Otorhinolaryngology. 2003 Jan; 67(1):7-10.

12. Ilce Z, Celayir S, tekand GT, Murat NS, Erdogan E., Yeker D. Tracheostomy in childhood: 20 years experience from pediatric surgery. Pediatric International. 2002 Jun;44(3):306-9.

13. Kearney PA, Griffen MM, Ochoa JB, Boulanger BR, Tseui BJ, Mentzer RM, A Single-Center 8-Year Experience With Percutaneous Dilational Tracheostomy. Annals of Surgery-Vol.231, No. 5, 701-709.

14. Kremer B, Botos-Kremer AI, Eckel HE, Schlondorff G. Indications, Complications, and surgical techniques for pediatric tracheostomies-an update. Journal of Pediatric Surgery. 2002 Nov; 37(11):1556-62.

56

15. Lee W, Koltai P, Harrison AM, Appachi E, Bourdakos MD, Davis S, Weise K, McHugh M, Connor J. Indications for tracheotomy in the Pediatric Intensive Care Unit Population: A pilot study. 2002 AAO-HNSF Annual Meeting Mini-Symposium, September 2002.

16. Line WS, Hawkins DB, MacLaughlin EF, Kahlstrom EJ, Ensley RN, Tracheostomy in Infants and Young Chidren: The Changing Perspective 1970-1985. The Laryngoscope 96: May 1986.

17. Maisel RH. Tracheostomy. Chap 25: 490-502. Boies Fundamentals of Otolaryngology, 6th edition, 1989

18. Meriitt RM, Bent JP, Smith RJ. Suprastomal Granulation Tissue an Pediatric Tracheostomy Decannulation. Laryngoscope Jul. 1997; 107(7): 868-71.

19. Midwinter KI, Carrie S, Bull PD. Pediatric tracheostomy: Sheffield experience 1978-1999. Journal of Laryngology and Otology. 2002 Jul; 116(7):532-35.

20. Miller FR, Eliachar I, Tucker HM, Technique, Management, and Complications of the Long-term Flap Tracheostomy, Laryngoscope 105;543-547: May 1995.

21. Myers EN. Operative Otolaryngology - Head & Neck Surgery. WB Saunders Company 1997. 575-585.

22. Nasonova NP, Egorov VM, Tracheostomy in Emergency Conditions in Children. Vestin Khir Im I I Grek.2002;161(4):52-5.

23. Norwood S, Valina VL, Short K, Saguisa M, Fernandez L, Incidence of Tracheal Stenosis and Other Late Complications After Percutaneous Tracheostomy. Annals of Surgery-Vol.232, No.2, 233-241.

24. Ochi JW, Bailey CM, Evans JNG. Pediatric Airway Reconstruction at Great Ormond Street: A Ten-Year Review. III. Decannulation and Suprastonal Collapse. Annals of Otology, Rhinology & Laryngology 1992; 101: 656-8.

25. Reibel, JF. Decannulation: How and Where. Respiratory Care 1999; 44(7): 856-59. 26. Reilly JS, Excision of Suprastomal Granulation Tissue. Laryngoscope 95;1545-1546,

Dec.1985. 27. Royal Alexandra Hospital, Head and Neck Directorate Ward 20.

Decannulation/Removal of a Tracheostomy Tube. 28. Sasaki CT, Gaudet PT, Peerless A. Tracheostomy Decannulation. American Jornal

of Diseases in Children 1978; 132: 266-9. 29. Sharpe MD, Parnes LS, Drover JW, Harris C, Translaryngeal Tracheostomy:

Experience of 340 Cases. The Laryngoscope, 1995;105:543-47. 30. Stauffer JL, Olson DE, Petty TL, Complications and Consequences of Endotracheal

Intubation and Tracheostomy. The American Journal of Medicine-Vol. 76;65-76, Jan.1981.

31. Thatcher GW, Maisel RH, The Long-Term Evaluation of Tracheostomy in the Management of Severe Obstructive Sleep Apnea. Laryngoscope 113:201-204 Feb.2003.

32. Tracheostomy, Mosby CV Co., Mosby’s Manual of Clinical Nursing, 2nd Edition, 1989.

33. Tracheostomy,http://www.1uphealth.com/health/tracheostomy_indications.html. 34. Tracheostomy Care Working Group, Tracheostomy Care Guidelines, St. James

Hospital/Royal Victoria Eye and Ear Hospital, October 2000. 35. Tucker JA, Silberman HD, Tracheotomy in Pediatrics, Annals of Otology, 1972. 36. Welcome to Aaron’s Tracheostomy Page. WWW.tracheostomy. Com 37. Wetmore RF, Thompson ME, Marsh RR, Tom LW, Pediatric Tracheostomy: a

Changing Procedure? Annals of Otology, Rhinology, Laryngology, 1999, 108: 635-699.

38. Yaremchuk K. Regular tracheostomy tube changes to prevent formation of granulation tissue. Laryngoscope 2003 Jan;113(1):1-10.

39. Zawadska-Glos L, Chmielik M. Tracheotomy in children-indications and complications. Department of Pediatric Otorhinolaryngology, The Medical University of Warsaw, Poland

57

N Y

N N

N

Y Y

N

N

Y Y N Y

Pediatric?

DOWNSIZE

Capping for

24 hours

Tolerated?

REMOVE TUBE;

Observe for 24

hours in hospital

setting

Capping for 24

hours

Tolerated?

REMOVE TUBE

Observe x 24 hrs. in

hospital setting

Tolerated

?

Ff-up after 7-

10 days

Tolerated?

Ff-up

after 7-10

days

REINSERT

TUBE

Further

evaluation by

specialist

(preferably

with

endoscopic

evaluation)

Further

evaluation by

specialist

(preferably with

endoscopic

evaluation)

MAINTAIN/ REINSERT

TUBE1

Trial of Decannulation

TRACHEOSTOMY

Criteria for

Decannulation Fulfilled

Maintain Tracheostomy

Tube

STUDY GROUPS

EAR STUDY GROUP PLASTIC STUDY GROUP GENEROSO T. ABES MD MPH (CHAIR) EUTRAPIO S. GUEVARA JR MD CHARLOTTE M. CHIONG MD CESAR V. VILLAFUERTE JR MD MHA ABNER L. CHAN MD FELIX P. NOLASCO MD TERESA LUISA I. GLORIA-CRUZ MD MHPEd JOSE FLORENCIO F. LAPEÑA JR MA MD MARIA RINA T. REYES-QUINTOS MD MCAud ROBERTO M. PANGAN DMD MD PhD NATHANIEL W. YANG MD ARMANDO M. CHIONG JR MD ERASMO GONZALO DV. LLANES MD (CHIEF RESIDENT) CHRISTOPHER MALORRE E. CALAQUIAN MD JASON S. GUEVARA MD HERBERT Q. GUTIERREZ MD MARIO ADRIAN M. ZAFRA MD DESIREE B. VANGUARDIA MD MARY APPLE PIE M. GARCIA MD FLORENCE YUL N. SAQUIAN MD JOSEPH ROY VINCENT B. UMALI MD

ORAL CAVITY STUDY GROUP LBEN STUDY GROUP EDILBERTO M. JOSE MD JOSELITO C. JAMIR MD JAIME F. FLOR MD EUTRAPIO S. GUEVARA JR MD JACOB S. MATUBIS MD RENE S. TUAZON MD ROMEO L. VILLARTA JR MD MPH JOSE FLORENCIO F. LAPEÑA JR MA MD JOSE FLORENCIO F. LAPEÑA JR MA MD MELFRED L. HERNANDEZ MD MHA AGNES N. TIRONA-REMULLA MD CHRISTINE JOY S. ARQUIZA MD VINCENT MARK M. JARDIN MD ERIC T. VINCULADO MD ERWIN M. ESLAVA MD MICHAEL F. GALICIA MD FORTUNA CORAZON A. ABERIN MD FELICIDAD B. MENDOZA MD IVY D. PATDU MD

RHINOLOGY STUDY GROUP TUMOR STUDY GROUP JOSEFINO G. HERNANDEZ MD MARIANO B. CAPARAS MD RUZANNE MAGIBA-CARO MD ALFREDO Q.Y. PONTEJOS JR MD RAMON ANTONIO B. LOPA MD JEANNETTE MARIE S. MATSUO MD

JOSE ROBERTO V. CLARIDAD MD (ORL ONCOLOGY FELLOW)

JERIEL JOHN C. MAJAM MD ERICK G. DUCUT MD DANILO R. LEGITA MD LINA ROSE A. ALCANCES MD MOH PHILIP B. FULLANTE MD ARSENIO CLARO A. CABUNGCAL MD CAMILLE SIDONIE A. ESPINA MD LEI-JOAN V. MOLO MD