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.

SGHnatural history

Proliferation: 8-18 months

Involution : 5-8 years

SGHpathology

Predominantly capillary type

Charaterized by: - proliferation of capillary endothelial

cells – multilamination of the basement membrane – accumilation of mast cells, macrophages, plasma cells, pericytes

SGHpathophysiology

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

SGHmanagement

Tracheotomy: - disadvantages:

* obstruction * delayed

speech

SGHmanagement

Low dose external beam radiation

Intralesional radioactive gold grain implant

The possibility of 2ndary malignancy

SGHmanagement

Sclerosing agents

Cryotherapy

- limited success rate – possible hemorrhage - stenosis

SGHmanagement

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%

SGHmanagement

Interferon alpha-2a – for large refractory hemangioma – spastic

diplegia in 10%

SGHmanagement

Intralesional steroid injection

- prolonged intubation – prolonged

hospitalization

SGHtreatment with

systemic steroidsUsed successfully to treat SGH

Al-Sebeih, Manoukian:treated 9 of 10 cases of SGH Using alternate-day course

Case 1RA

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 2MD

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 3ZN

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 4WH

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

SGHsteroid

Mechanism of action is unknown – anti-inflammatory effect

– sensitivity of B.V endogenous vasoconstrictors

– postulated estradiol receptors that are occupied by steroids

SGHsteroids

Mechanism of action – increase mast cells – reduced growth

factors PDGF-A and B, IL6TGF-Beta 1 and 3

SGHsystemic steroids

Effective

Safe

Do not disturb the anatomy