1. Systemic steroid for subglottic hemangiomaBy
Ahmed Al-Ammar, MD, FKSU

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

3. SGH natural history
Proliferation: months
Involution : years

4. 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

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

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

7. SGH management
Tracheotomy: disadvantages: * obstruction * delayed speech

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

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

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

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

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

13. SGH management
Intralesional steroid injection prolonged intubation – prolonged hospitalization

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

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

19. 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 Percentile WT percentile

20. 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 %

24. 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

25. 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 %

30. 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%

31. 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%

34. 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

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

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

37. SGH systemic steroids
Effective
Safe
Do not disturb the anatomy