proptosis in children

Apr 11, 2023

PROPTOSISchildren

Dr. Yousaf JamalFCPS Resident

Ophthalmology UnitHayatabad Medical Complex

06-03-2010

Apr 11, 2023Proptosis in Children

CONTENTS

• Recap• Causes in children• Differentiating features• Common causes discussion• Summary• Take home message• MCQs


Recap

• Causes of Proptosis• Common causes in adults• Examination of proptosis


CONTENTS



Causes in children

• Orbital cellulitis• Dermoid cyst• Capillary

hemangioma• Optic nerve

glioma• Rhabdomyosarco

ma

• Retinoblastoma • Leukemia• Lymphangioma

• Metastasis – Metastatic

neuroblastoma– Ewing's sarcoma


CONTENTS



CAPILLARY HEMANGIOMA

ORBITAL LYMPHANGIOMA

Pathology Vascular hemartoma Isolated vascular hemartoma

Demographics

Infant, commonest benign orbital tumor in childhood, spontaneous involution

Early childhood

Associated Visceral, nasopharyngeal hemangioma, kasabach-merit syndrome

-

Presentation Nonaxial, +ve Valsalva, superficial/deep

Nonaxial, -ve Valsalva, acute episodes of spontaneous hemorrhage, may be superficial/deep

Radiology Intra/extraconal mass, poorly defined

Low density cyst-like mass, enlargement of bony orbit

Tx Observation, surgery Guarded prognosis, surgery, drainage


ON GLIOMA ON MENINGIOMA

Pathology Fusiform enlargement of ON, origin - glial tissues, meninges show reactive hyperplasia, dura is normal

Tubular enlargement of ON, origin-meninges, psammoma bodies

Demographics

Young girls (2-6yrs) Late middle aged woman

Associated NF-1 (20-40%) -

Presentation

Axial, VA decreases early, EOM normal, optociliary shunts - uncommon

Axial, gaze evoked amaurosis, EOM impaired, optociliary shunts

Radiology Fusiform enlargement, isodense to bone

Tubular enlargement, hyperdense, ON sheath enhancement

Tx Conservative, surgical, radiotherapy

Conservative, radiotherapy, surgery


RHABDOMYOSARCOMA

ORBITAL CELLULITIS

Pathology Undifferentiated mesenchymal cells, most common primary orbital malignancy of childhood

Infection of soft tissues behind the septum

Demographics

Boys > girls (avg 7 yrs), Commonest cause of proptosis in children, adults also affected

Associated Cervical L. nodes involved Usually after trauma, sinus related

Presentation Nonaxial, painful, unilateral, rapid onset, swollen bluish lids, nose bleed, Ptosis, chemosis

Painful, lids edema with reddish color, Ptosis, malaise, fever, life threatening, VA impaired, Ophthalmoplegia,

Radiology Moderately well-defined homogenous mass, Bony destruction

CT-(orbit, sinuses, brain): diffuse orbital infiltrate, sinus opacity, proptosisMRI for cavernous sinus thrombosis.

Tx Radiotherapy, chemotherapy, Exenteration for resistant cases

Medical, surgical


CONTENTS



ORBITAL CELLULITIS

• Bacterial orbital cellulitis – life-threatening

• Infection of soft tissues behind septum• More common in children• Organisms

– S. pneumoniae– S. aureus– S. pyogenes– H. influenzae


Etiology

• Sinus-related– Ethmoidal (90%)

• Extension of preseptal cellulitis

• Local spread– Dacryocystitis– Facial cellulitis– Dental infections

• Systemic bacteremia

• Post-trauma– Penetrating septum

(48-72 hrs)– Retained foreign body

• Post-surgical– Lacrimal– Orbital– Retinal


Presentation…

• Malaise• Fever• Pain• Visual impairment• Tender, warm, red periorbital edema• Proptosis


… Presentation

• Painful Ophthalmoplegia• Chemosis• Headache• Rhinorrhea• Elevated IOP


Courtesy: Medline


Work-up

• Hx• Examination• Vital signs record• WBC/blood culture• Lumbar puncture• CT orbit• MRI

– For cavernous sinus thrombosis


Complications

• Ocular– Exposure

keratopathy– Raised IOP– CRVO,CRAO– Endopthalmitis– Optic neuropathy

• Intracranial– Meningitis (2%)– Brain abscess– Cavernous sinus

thrombosis (1%)

• Orbital abscess• Subperiosteal

abscess


Treatment

MEDICAL…• Hospital admission + ENT consultation• I.V. antibiotics

– Ceftazidime, Cefotaxime, Ceftriaxone– Flucloxacillin – Vancomycin– Ampicillin-Sulbactam– Piperacillin-Tazobactam– Ciprofloxacin/Levofloxacin


• Metronidazole• Nasal decongestant

– Phenylephrine – Oxymetazoline

• Antibiotic eye ointment


SURGICAL…

• Indications– Decreasing vision– Afferent pupillary defect– No response to antibiotics in 48-72 hrs– Orbital/Subperiosteal abscess– Atypical picture…..Needs biopsy


• Canthotomy/Cantholysis in emergency• Surgical drainage of fluid • Orbital surgery + sinusotomy• For fungal

– Debridement– Exenteration


Doses

• Ceftazidime (Fortum®)– Adults=1-2g/8hr I.V.– Pediatric

• Neonates=30mg/kg IV• 1 month-12 years=30-50mg/kg IV

• Ceftriaxone (Rocephin®)– Adults=2g/12hr IV– Pediatric

• 20-50mg/kg/day IV


• Cefotaxime (Claforan®)– Adults=1-2g/10ml/4hr IV in 3-5 min

(max=12g/day)– Pediatric

• 0-1 week=50mg/kg/12hr IV• 1-4weeks=50mg/kg/8hr IV• 1month-12years=50-80mg/kg/day IV in 4-6 doses


• Vancomycin (Vancocin®)– Adults=1g/12hr IV (over 1 hour with rate <

10mg/min at concentration <5mg/ml)– Pediatric

• Infants & neonates=15mg/kg loading

• Ciprofloxacin– In > 17 years age=400mg/12hr IV

• Levofloxacin– In > 17 years age=750mg/6hr IV


• Ampicillin-Sulbactam (Unasyn®)– Adults=3g/6hr IV– Children=300mg/kg/day 4 divided doses

• Piperacillin-Tazobactam (Zosyn®)– Adults=4.5g/8hr IV– Children=240mg Piperacillin

component/kg/day 3 divided doses

• Metronidazole (Flagyl®)– 500mg/6-8hr IV


Tx combinations

• Metronidazole to all• Vancomycin + Ceftriaxone (3rd gen

ceph)– Allergy to penicillin

• Fluoroquinolone– Allergy to penicillin + cephalosporins

• Vancomycin + Ampicillin-Sulbactam or Piperacillin-Tazobactam– For MRSA


Follow-up

• Daily monitor– Symptoms– Temperature– Visual acuity– Ocular motility– Proptosis– Cornea– IOP– Retina and optic nerve


• Change to oral to complete 14 days course if– Afebrile for 48 hours

• Oral Drugs– Amoxicillin-clavulanate (Augmentin®)

• Adults=500mg TDS• Children=20-40mg/kg/day 3 divided doses

– Cefaclor (Ceclor®)• Adults=500mg TDS• Children=20-40mg/kg/day 3 divided doses

– Metronidazole


DERMOID CYST

• A choristoma, found adjacent to suture lines

• Connected to periorbita by fibrovascular tissue

• Contain…– Keratinized stratified squamous epithelium

– Blood vessels, fat, collagen, sebaceous glands, and hair follicles

– a tan oily liquid to a white or yellow substance


Presentation…

• Mostly in children• Mostly…Superotemporal aspect of orbit• A painless mass• Slowly growing• Usually < 1 cm in diameter, non-tender


… Presentation

• Globe displacement• Inflammation if ruptures• May

– Compress optic nerve – Diplopia


Courtesy: Medline


Work-up

CT/MRI

– Indicated if posterior extent not palpated– Cystic appearance– Well circumscribed lesion


Courtesy: eyetext.org


Treatment

Surgical• Excision in Toto


CAPILLARY HEMANGIOMA

• Most common orbital tumor of infancy• Benign endothelial cells neoplasms• Absent usually at birth• Rapid growth in infancy• Involution later on


Pathophysiology

• Thought … of placental origin• 02 phases of growth

– Proliferative• Rapid growth…8-18 months• Increase in no. of endothelial & mast cells

(stimulus for vessel growth)

– Involutional• 30-50% involute by 5 yrs• 75% by 7 yrs

Kavanagh EC, Heran MK, Peleg A, et al. Imaging of the natural history of an orbital capillary hemangioma. Orbit. Mar 2006;25(1):69-72


Mortality/Morbidity

• Kasabach-Merritt syndrome – Coagulopathy, thrombocytopenia– large visceral / Nasopharyngeal

hemangiomas– DIC may occur, high output CCF– Mortality…30-50%

• Ophthalmic morbidity – Space occupying– Amblyopia


Presentation

• F:M…3:1• 1-2% in neonates• All develop by 6 months• Typically…red spot growing in size in

Periorbital area• Inability to open lids• Bulging of the eye


Classified as…

1. Cutaneous2. Purely preseptal3. Preseptal with extraconal element4. Combination of preseptal, extraconal and

intraconal


Signs

• Usually…superonasal, brow or lid lesion• Typically blanches with pressure• Ptosis & proptosis if posterior extension• Visual loss• Preseptal…Dark blue or purple• Enlarge & change color while crying


Work-up

CT– poorly circumscribed mass with no bony

erosion – Homogenous enhancing lesion

MRI– hypointense to fat on T1-weighted &

hyperintense on T2-weighted scans US & Doppler

– For anatomic relations & extent


Treatment

Medical • Observation• Steroids

– Clobetasol propionate cream – Injectable steroid formulations – Systemic corticosteroids are used for

amblyogenic lesions

• Interferon alfa-2a – Resistant to steroid treatment


Surgery• Laser surgery

– carbon dioxide laser – Argon laser – Nd:YAG laser

• Primary excision


OPTIC NERVE GLIOMA

• Most common primary tumor of ON– In children…mostly benign– In adults…Aggressive glioma

• WHO classify it as– Grade I astrocytomas (pilocytic

astrocytomas) bcz slow growing & tend not to metastasize

• Generalized hyperplasia of glial cells in the nerve to complete disorganization


• 10-38% of pediatric pts with ON glioma have NF-1

• Bilateral ON gliomas…almost pathognomonic for NF-1

Listernick R, Charrow J, Greenwald MJ, et al. Optic gliomas in children with neurofibromatosis type 1. J Pediatr. May 1989;114(5):788-92.


Presentation…

• ON gliomas are…*– 4% of orbital tumors– 4% of intracranial gliomas– 2% of intracranial tumors – 2/3rd of all primary optic nerve tumors

• 20% gliomas extend to the optic chiasm or beyond into the optic radiations & aggressive

* Hollander MD, FitzPatrick M, O''Connor SG, et al. Optic gliomas. Radiol Clin North Am. Jan 1999;37(1):59-71.


… Presentation…

• F>M• In pediatrics

– Median age…5 yrs – 80% pts present before age 15

• In adults– 22-79 yrs with a mean age of 52 yrs

Hollander MD, FitzPatrick M, O''Connor SG, et al. Optic gliomas. Radiol Clin North Am. Jan 1999;37(1):59-71.

Millar WS, Tartaglino LM, Sergott RC, et al. MR of malignant optic glioma of adulthood. AJNR Am J Neuroradiol. Sep 1995;16(8):1673-6.


… Presentation

• Painless proptosis with inferior dystopia• Decreased vision• Nystagmus• Changes in appetite or sleep• Large lesion can obstruct 3rd ventricle &

ICP• Optic atrophy• Opticociliary collaterals• CRVO


Work-up

CT• Diffuse Fusiform enlargement of the

optic nerveMRI

• T1-weighted images– Isointense to hypointense

• T2-weighted images– Isointense to hyperintense


Treatment

• Not required if– Not growing– Good vision– No cosmetic blemish

• Surgical excision– Large & growing lesions– Confined to orbit

• Radiotherapy + chemotherapy– Intracranial extension


EMBRYONAL SARCOMA

• Most common primary orbital malignancy of childhood

• Origin…undifferentiated mesenchymal cells

• Called Rhabdomyosarcoma if differentiate into striated muscle


Presentation…

• Usually first decade (average…7yrs)• Boys > girls• Proptosis

– Rapid (days to weeks)– Unilateral– Painful– Non-axial

• Mimics orbital cellulitis


… Presentation…

• Swollen lids but bluish appearance• Ptosis • Location

– Superonasal– Retro bulbar– Superior– Inferior

• Chemosis• Sinuses involvement & nose bleeds


… Presentation

• Other findings– Disc edema– Choroidal folds– Decreased vision – Cervical lymph nodes involvement


Courtesy: medline


Work-up

USG• Relatively well-circumscribed mass• Low-medium amplitude echoes

CT & MRI• Moderately well-defined homogenous mass• Bony destruction

Biopsy Systemic investigations


Treatment

• Referred to pediatric oncologist• Radiotherapy• Chemotherapy• Surgery

– Exenteration for recurrent & resistant cases


LYMPHANGIOMA

• Introduction – Non-Neoplastic– Non-functional– Abortive– Vascular malformation

• Hemodynamically isolated• Bleeding into lumen…chocolate cysts


Presentation…

• Early childhood (average=5-6 yrs)• Multiple bluish soft masses • Upper nasal quadrant• Cystic conjunctival component• Proptosis

– Due to spontaneous hemorrhage– Painful – Axial/non-axial


… Presentation

• Worsened by RTI• Palatal involvement• Ptosis• Restricted eye movements• Optic nerve compression• Papilledema• Secondary glaucoma


Work-up

CT• Multilobulated cystic mass within the

orbit and/or intralesional calcificationsMRI

• Hemorrhagic cyst…Hyperintense• Fluid level


Treatment

• Conservative • Partial surgical

resection of the major cyst

• Needle aspiration• CO2 laser

• Cryotherapy

• Intralesional injection of sclerosing agents– sodium tetradecyl

sulfate (Sotradecol®)

– OK-432 (Picibanil®)

• local radiotherapy


• Sodium tetradecyl sulfate (Sotradecol®)• An anionic surface-active agent used for its wetting

properties in industry and used in medicine as an irritant and sclerosing agent for hemorrhoids and varicose veins

• $259.00


• OK-432 (Picibanil®)• lyophilized preparation of a low-virulence strain of S.

pyogenes (S. hemolyticus), inactivated by heating with penicillin G. It has been proposed as a noncytotoxic antineoplastic agent because of its immune system-stimulating activity.


CONTENTS



PROPTOSIS

Lid edemaSinus diseaseRestricted motilityPain/TenderLid traumaFever

Little/no inflammationOrbital lesionYoung child/adultSlow growingLocated near bone sutures

ORBITAL CELLULITIS Dermoid cyst


PROPTOSIS

Child (5 yrs)Upper nasal quadrant massPainfulFew episodes of proptosisWorsened by RTIMRI shows fluid levels

Child (7-8yrs)Hx of nose bleedsBluish color lids

UnilateralLymph node involved

Painful

ORBITAL LYMPHANGIOMA Rhabdomyosarcoma


CONTENTS



TAKE HOME MESSAGE

• Proptosis in children may be – Benign as … Dermoid– Malignant as … Rhabdomyosarcoma

• Thorough Hx & clinical approach• Pediatric ophthalmologist & oncologist


MCQs

1. A 1-month old infant presents with a purplish, spongy eyelid mass. Most likely diagnosis:

a. Rhabdomyosarcomab. Neurofibromac. Dermoid cystd. Capillary hemangiomae. Metastatic Ewing sarcoma

Ans. d


2. In the evaluation of child with unilateral Proptosis, which assumption is true?

a. Cavernous hemangioma is the most common cause of unilateral Proptosis in children

b. Thyroid ophthalmopathy is the most common cause of unilateral Proptosis in children

c. Neurofibroma is the malignant tumor that most commonly produce Proptosis in children

d. ON meningioma are more common than gliomas in childrene. None of the above

Ans: e


3. The management of Rhabdomyosarcoma of the orbit usually involves

a. Lumbar puncture to rule out CNS Metsb. Exenterationc. Enucleation & orbital radiationd. Systemic chemotherapy & orbital radiatione. Radical neck dissection if cervical L. nodes are

involved

Ans: d


4. All of the following are true regarding optic nerve tumors except:

a. ON gliomas in children are associated with NF-1b. ON meningioma in children are associated with NF-2c. Radiation therapy is an accepted therapy for ON

sheath meningiomad. ON gliomas of childhood can be malignant

Ans: d


FOR MCQs 5-8

5. An 11-year old girl presents with acute, unilateral, left-sided periocular pain, Proptosis and double vision. Which condition would NOT be included in D. Dx?

a. Cavernous hemangiomab. Sinusitis with orbital abscessc. Traumatic retrobulbar hemorrhaged. Orbital Lymphangioma

Ans: a


…Continued…6. 24 hrs later & without any treatment, the pain

has resolved. Periocular ecchymosis develops, & the double vision stabilized. MRI shows cystic mass & layered hemorrhage. Most likely Dx based on clinical Hx & MRI findings is:

a. Rhabdomyosarcomab. Capillary hemangiomac. Orbital abscessd. Lymphangioma

Ans: d


…Continued…

7. If the patient was losing vision bcz of this process, you would consider:

a. Intravenous antibioticsb. Open surgery to excise the lesion in its entiretyc. CT-guided drainage of encysted bloodd. Biopsy of the lesion to establish Dx

Ans: c


…Continued

8. The disease process is an example of :a. The most common cause of proptosis in childrenb. The most common primary orbital malignancy in

childrenc. A tumor that may enlarge with upper RTId. An orbital vascular lesion that will involute after

intralesional corticosteroids

Ans: c


9. Which one of the following is the most common primary malignancy of the orbit in children?

a. Neuroblastomab. Rhabdomyosarcomac. Ewing’s sarcomad. None of the above

Ans: b


10. what is the most common cause of unilateral childhood proptosis?

A. capillary hemangiomaB. Thyroid related orbitopathyC. Orbital hemorrhageD. Orbital cellulitis

Ans: D


11. In orbital cellulitis, which of the following is false:

a. Common cause of proptosis in childrenb. Seen due to infection spread from surrounding sinusitisc. Commonly bilaterald. Can cause cavernous sinus thrombosis

Ans: c


12. Most common organism causing orbital cellulitis in children < 5 yrs:

a. Pneumococcib. Staph aureusc. Strep pyogenesd. H. influenzae

Ans: d


13. ON glioma includes following:a. Usually seen in 1st decade of lifeb. Can be seen either in the intraorbital or intracranial

part of optic nervec. Generally causes painless, bilateral proptosisd. Proptosis is axial, non-Pulsatile & irreducible

1. a+b+c 2. a+b+c+d 3. a+b+d 4. a+c+d

Ans: 3


14. A young child presents with sudden loss of vision & proptosis of right eye. On examination, the direct pupillary reflex is absent but consensual pupillary reflex is present. He is having:

a. ON gliomab. ON meningiomac. Retinoblastomad. ON angiomae. Capillary hemangioma

Ans: a

Apr 11, 2023

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