Date post: | 21-Dec-2015 |
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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