Systemic steroid for subglottic hemangioma By Ahmed Al-Ammar, MD, FKSU

SGH Most common tumor in infancy Up to 12% of white 1.5% of congenital anomalies of the larynx Female : male 2 : 1 80-89% - in the 1st 6 mon.

SGH natural history Proliferation: 8-18 months Involution : 5-8 years

SGH pathology Predominantly capillary type Charaterized by: - proliferation of capillary endothelial cells – multilamination of the basement membrane – accumilation of mast cells, macrophages, plasma cells, pericytes

SGH pathophysiology Poorly understood Growth factors; regulators of angiogenesis – VEGF - b FGF – TGF-Beta - IL6 Some may be involved in hemangioma proliferation or involution

SGH TRATMENT Ideal treatment: - normal anatomy of the larynx – success rate…. high – complication …. few

SGH management Tracheotomy: - disadvantages: * obstruction * delayed speech

SGH management Low dose external beam radiation Intralesional radioactive gold grain implant The possibility of 2ndary malignancy

SGH management Sclerosing agents Cryotherapy - limited success rate – possible hemorrhage - stenosis

SGH management Surgical excision - for large SGH – not responding to – steroids – CO2 laser

SGH management CO2 laser: Healy et al 1980 for unilateral isolated SGH less bleeding – subglottic stenosis in 20-40%

SGH management Interferon alpha-2a – for large refractory hemangioma – spastic diplegia in 10%

SGH management Intralesional steroid injection - prolonged intubation – prolonged hospitalization

SGH treatment with systemic steroids Used successfully to treat SGH Al-Sebeih, Manoukian: treated 9 of 10 cases of SGH Using alternate-day course

Case 1 RA 3 mon male Present: - increasing difficulty of breathing – stridor - feeding difficulties – scrotal hemangioma Bronchoscopy revealed SGH ----- 90%

Case 1 cont. Treatment: prednison - high dose X 10 days – alternate-day X 6 mon. - SX improved after the 1st 48 h. – off treatment for 3 mon. breath comfortably Under FU of ped. endocrinologist Growth: HT 80-90 Percentile WT 50-90 percentile

Case 2 MD 1 mon. Female Known - holopronsencephaly – diabetes insipidus – RT pelvic kidney Present: - coughing - SOB - hemangioma on RT aurical, lips, RT parotid region Started on O2, Ventolin; deteriorated; stridor, cyanosis Bronchoscopy: SGH 80%

Case 2 cont. Treatment: prednison – high dose X10 days – alternate day X 9 mon. Sx improved gradually Off treatment for 14 mon – breath comfortably FU with ped. Endocrinologist Growth: - HT 25% - WT; low

Case 3 ZN 2 mon female Present: - SOB - difficulties of feeding – voice change – insp. Stridor – hemangioma of lower lip, oral mucosa, RT parotid region Bronchoscopy: SGH 70%

Case 3 cont. Prednison: alternate-day started 1 mon. later ( for 11 mon). SX improved gradually Off treatment for 10 mon. – breathing comfortably FU with ped. Endocrinologist Growth: - HT 25% - WT 50%

Case 4 WH 7 mon. Female Present: - SOB - difficulty in breathing (for 6 mon) – sleep disturbance - stridor - cutaneous hemangioma of neck Bronchoscopy: SGH circumferential 80%

Case 4 cont. Steroid: - started 1 WK - high dose X 10 days – alternate-day X 8 mon SX improved gradually Off treatment for 3 mon. – breathing comfortably Growth: at 10th percentile for HT & WT at the end of treatment FU with ped. Endocrinologist Complication: - moon face

SGH steroid Mechanism of action is unknown – anti-inflammatory effect – sensitivity of B.V endogenous vasoconstrictors – postulated estradiol receptors that are occupied by steroids

SGH steroids Mechanism of action – increase mast cells – reduced growth factors PDGF-A and B, IL6 TGF-Beta 1 and 3

SGH systemic steroids Effective Safe Do not disturb the anatomy