The management of paediatric subglottic haemangioma (SGH)is controversial. Two children of nearly the same age presentedto the John Hunter Children’s Hospital, Newcastle, New SouthWales within a month of each other and were definitivelymanaged using different treatment modalities. We present thesechildren and offer some comments on the management optionsin this life-threatening condition.

CASE 1

An 11-week-old female infant was referred by her paediatricianwith a provisional diagnosis of SGH. She had a 1-week historyof stridor at rest and a 10-week history of a slowly enlarging leftupper eyelid and supraorbital cutaneous haemangioma (Fig. 1).She had been commenced on oral prednisolone syrup,1mg/kg/day, with some beneficial effect. At elective rigid laryngo-bronchoscopy, two haemangiomata were found – a flat leftlateral pharyngeal wall haemangioma, and a SGH arising fromthe left postero-lateral cricoid. The SGH (Fig. 2) was 1.25 cmlong and arose from 25% of the inner circumference of thecricoid. It obstructed approximately 75% of the lumen. The SGHwas vaporized to a residual of 10% using the CO2 laser. Theamount of bleeding suggested a predominant vascularcomponent. The child was monitored postoperatively in Paedi-atric Intensive Care. Postoperative prednisolone was continuedat 1 mg/kg/day for 1 week, then 0.5 mg/kg/day for 1 week, andthen 0.25 mg/kg/day for a further week. Her paediatricianfollowed her condition closely. The SGH was lasered electivelyagain when she was 14 weeks old, 18 weeks old, and then22 weeks old (a total of four lasers). At each laser there had

been re-growth, but to a lesser extent each time. The post-operative tapering steroid regimen was halved in dose after thesecond laser. Check-endoscopy at 28 weeks of age when shewas asymptomatic for stridor, except during an upper respiratoryinfection or exertion, showed a residual of 15–20% lumenalobstruction. Final endoscopy at 49 weeks of age whilstcompletely asymptomatic, showed a residual obstruction ofabout 10% of the lumen with complete involution in the middlepart and no evidence of damage from the laser. Computedtomography (CT) scan, to exclude Sturge–Weber syndrome,was normal. Ophthalmic follow up of the orbit is in progress.

CASE 2

An 8-week-old female infant (twin I, 36 weeks gestation)presented with fatigue from significant airway obstruction,biphasic stridor and haemangiomata on the lower lip, lower facein a ‘beard’ distribution1 (Fig. 3), and neck over the trachea andsternal notch. Emergency rigid laryngo-bronchoscopy on theevening of admission revealed an extensive SGH obstructing80% of the lumen. It arose from the left postero-lateral one-thirdof the cricoid. There were no other airway lesions. The SGH waslasered to 10% residual over approximately one-third of theinner circumference. The lack of bleeding suggested a pre-dominant fibrous component. Postoperative recovery wasuneventful. She had check endoscopy at 11 weeks of age whichshowed no early re-growth. At 15 weeks she developed virallaryngotracheobronchitis, required admission to PaediatricIntensive Care and underwent repeat endoscopy. The SGH hadgrown further posteriorly, obstructing approximately 50% of thelumen. There was diffuse subglottic oedema superimposed, anda new airway lesion in the mid-trachea posteriorly. Laser wasdirected to the SGH posteriorly and laterally leaving a mucosalbridge, with good improvement. At 21 weeks of age she was re-admitted with significant airway obstruction despite continuingoral prednisolone. Repeat endoscopy showed that her SGH hadspread circumferentially and distally, with a subglottic lumenwhich only accepted a 2.7 mm telescope. Lasering was judged

J. Paediatr. Child Health (1999) 35, 392–395

Subglottic haemangioma: Controversiesin management

PJ WALKER,1 D COOPER1 and D MA CDONALD2

1Department of Paediatrics, John Hunter Children’s Hospital, Newcastle, and 2Department of Paediatrics, Port MacquarieBase Hospital, Port Macquarie, New South Wales, Australia

Objective: To discuss treatment modalities for subglottic haemangioma (SGH).Methodology: Case report of two children definitively managed by different modalities.Results: Management by CO2 laser vaporization in one child, and laser followed by interferon 2-alpha in the second childwere both successful in controlling the SGH without the need for tracheostomy.Conclusions: The stepped-care approach at John Hunter Children’s Hospital, Newcastle, New South Wales, is presented.Both laser surgery and interferon can help control SGH. Careful surveillance and interdisciplinary cooperation are essential toachieve a good outcome.

Key words: CO2 laser; interferon 2-alpha; paediatric; subglottic haemangioma.

Correspondence: Dr PJ Walker, Paediatric Otolaryngologist, Suite 2, TheHeights Private Medical Centre, PO Box 293, New Lambton, NSW 2305,Australia. Fax: (02) 4957–2960.

PJ Walker, FRACS, Paediatric Otolaryngologist. D Cooper, MSc, FRACP,FRCP(C), Respiratory Paediatrician. D MacDonald, FRACP, Paediatrician.

Accepted for publication 1 October 1998.

inappropriate. She was intubated initially with a 2.5 endotrachealtube and then a 3.5 endotracheal tube and returned to PaediatricIntensive Care. Her facial cutaneous haemangiomata had alsosignificantly increased in size. Interferon 2-alpha was com-menced at a dose of 1 3 106 u/m2/day and she was successfullyextubated after 1 week. Check endoscopy at 22 weeks showeddramatic reduction in the size of the SGH with only a thin residual‘rim’ evident circumferentially not significantly narrowing hersubglottic airway (Fig. 4). She continued on interferon. At checkendoscopy at 50 weeks of age, when asymptomatic on interferon0.1 mL/day, there was no identifiable SGH but some minor laserchar tattoos. Her cutaneous lesions had also significantlyreduced in size. Interferon was ceased at 56 weeks of age.

DISCUSSION

All except tiny subglottic haemangiomata require treatment topreserve a patent airway. There are two difficult issues in thetreatment of SGH: the first is the natural history of subglottic

haemangioma to spontaneously regress; the second is thepaucity of literature on which to make a level 1 or level 2evidence-based decision on which of several treatment modal-ities is best. Most published series are small. Few institutions

393Subglottic haemangioma

Fig. 1 Left upper eyelid and supraorbital haemangiomata.

Fig. 2 Subglottic haemangioma arising from the left postero-lateralsubglottis.

Fig. 3 Facial haemangiomata in ‘beard’ distribution.

Fig. 4 Thin residual ‘rim’ of subglottic haemangioma.

report a single modality – most combine treatments, makingassessment of efficacy more difficult. Few SGH have beenmanaged by observation alone. Reported mortality from obser-vation alone is as high as 45%.2 The natural history of involution,its timing, and completeness are unknown. Assessment of invo-lution itself is unclear, and continues to evolve as standards ofendoscopic–calibration improve and CT and magnetic resonanceimaging (MRI) gain greater resolution. Cellular markers inhaemangiomata have been demonstrated to clearly vary with thedifferent phases of haemangioma development (proliferatingphase, involuting phase, involuted phase)3 and should proveuseful in objectively monitoring response to treatment.

Because SGH do eventually spontaneously regress, treat-ment must be supportive of the child’s airway until involution hasoccurred – without causing side effects either locally to thelarynx or systemically. Although haemangiomata may take up to5–12 years to involute to a fibro-fatty residuum,3,4 SGH aretypically symptom-free after 12–18 months. Simple ‘bypass’ ofthe obstructing SGH while awaiting spontaneous involutionseems attractive as a sole modality.5 Aside from the potentialmorbidity of tracheostomy itself, as a sole modality this assumesthat the expanding SGH has no ill-effect on the growinglaryngeal cartilages and interconnecting membranes. Largecutaneous or orbital haemangiomata can leave residual damageto adjacent structures after involution and, although not clinicallyapparent, similar damage may occur to the larynx. Duration oftracheostomy cannulation is reported as a median of 455 daysbut can be as long as 4 years.6

Systemic corticosteroids are used widely, alone or in combin-ation with laser vaporization. The mechanism of action of corti-costeroids is likely to be via blocking oestradiol-17 betareceptors active in the proliferation of endothelial cell cytoplasmrather than simply sensitizing the haemangioma to circulatingvasoconstrictors. Reported efficacy of corticosteroids alone inhaemangiomata from different sites is between 30 and 60%.4,7,8

Our preference is for a pulse of oral prednisolone 1–2 mg/kg/dayfor 1 week, reducing to 0.5–1 mg/kg/day for 1 week, and then to0.25–0.5 mg/kg/day for 1 week. Corticosteroids also may beinjected into the lesion but this requires postoperative intubation.A 100% success rate has been reported in six small haeman-giomata9 but intubation for up to 2–3 weeks may be required, asmay multiple injections.

Laser vaporization is probably the mainstay of treatment forSGH worldwide and is our recommended first step in treatment.Lasering is performed slowly and carefully without endotrachealintubation, and is limited to no more than one-third to one-half ofthe circumference of the cricoid at a single session, avoidingcharring. Sie et al. reported that approximately two-thirds of 28children required more than one treatment and 10% requiredoperative repair of acquired subglottic stenosis complicatinglaser vaporization.10 This is more likely in prolonged or multiplelaser treatments. In his series, 80% had concurrent cortico-steroids with a reported complication rate of 20%. Seventy-fiveper cent of his series were managed without tracheostomy.

Circumferential or bilateral SGH are not usually suitable forlaser. In these lesions a ‘covering’ tracheostomy may be appro-priate to protect the airway while multiple laser sessionscontinue – with a greater risk of acquired subglottic stenosis.Open excision is recommended by some for these largehaemangioma assessed as too big for safe lasering.8,11,12

Lumenal augmentation may be performed at the same time toprevent acquired subglottic stenosis. One of the argumentsproposed is that interferon ‘is not utilized because a rapid effect

is not expected on the respiratory distress’.8 We were able tosuccessfully extubate our case after 1 week of interferon, butpresumably when complete excision is successful, cure isimmediate.

Interferon alpha inhibits angiogenesis and in vitro endothelialcell migration and proliferation. Recent reported successesinclude haemangiomata of the liver [reducing mortality from 30% in the corticosteroid group of 10, to 15% in the interferongroup of 13],13 lung,14 and in cavernous haemangioma withKasabach–Merritt syndrome.15 Other heterogeneous groups oflarge or complex haemangiomata with life-threatening airwayobstruction, platelet-trapping, and/or high-output heart failure,which were unresponsive to traditional therapy have beenreported. MacArthur et al. reported a response in all of his seriesof five patients.16 In another series of four infants and one child,after an average duration of treatment of 7 months, complete ornear-complete regression was identified in three, plus 50% re-gression in one and no progression in the fifth.17 Ezekowitz et al.reported 20 haemangiomata in different sites commenced on interferon alpha from age 3 weeks to 2 years – 18 hadregression of 50% or more after an average of 7.8 monthstreatment.4 In a further series of 10 patients with progressiveinvasive angiomatous disease, six had a marked response, twohad a moderate response, and two had no or minimalresponse.18 No response was shown in four life-threateningangiomas by Teillac-Hamel et al.19 In 15 head and neck haeman-gioma (14 infants and one adult, five with airway involvement)interferon alpha gave beneficial response in 12, with no relapsein the 10 who had been off treatment 6–53 months.20 A series of15 children with SGH who failed combined laser and cortico-steroid treatment and subsequently underwent interferon alphatreatment was reported by Ohlms et al.21 In the series by Ohlmset al. 11 of 15 were asymptomatic by 24.1 months, but 47%required tracheostomy and of these decannulation was onlypossible in six of seven by 26 months.21 Although some of theseSGH were associated with haemangiomata elsewhere in theupper airway, Froelich et al. suggests these results are notstatistically different to results for spontaneous involution.8

Reported side effects of interferon in children include fever,neutropaenia, elevated hepatic transaminases, anorexia, skinnecrosis, and haemodynamic instability. Of concern is thedevelopment of spastic diplegia22,23 and anecdotal reports of impaired neurological development. Reported frequency ofcomplications is low (20–40%) and Kemp24 suggests that theyare dose-related and transient – usually resolving whentreatment finishes. Reported side effects in adults, however, haveincluded liver failure, seizures, encephalopathy and auto-immunedisease.

A stepped-care approach is our recommendation. Diagnosticrigid laryngo-bronchoscopy is performed to assess the site, sizeand extent of the SGH and to exclude other lesions within theairway. We recommend that the first step in suitable SGH bejudicious laser vaporization with a pulse of systemic cortico-steroids. We prefer laser vaporization as the initial treatmentbecause it avoids the need for daily subcutaneous injections andweekly or fortnightly venepunctures over many months, and alsoavoids the risk of interferon-related complications. Imaging withCT or MRI is useful but great caution needs to be exercised ifsedation or intubation is required. If the SGH progresses aroundthe subglottis beyond what can safely be controlled by the laser,or is too large for laser at initial presentation, our next step is tocommence interferon alpha, with ‘prophylactic’ intubation forairway support if required, for one week. We are more likely to

394 PJ Walker et al.

395Subglottic haemangioma

commence interferon when extensive cutaneous or multiplehaemangiomata are associated with the SGH. For children withlarge SGH who have not responded to the laser or to interferonwe would consider open excision; however, tracheostomy wouldbe indicated if the SGH approached circumferential or hadspread extra-laryngeal. Inter-disciplinary cooperation is essentialfor a good outcome.

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