IH2 Flashcards
What is the typical clinical course of an infantile hemangioma (IH)?
IHs proliferate rapidly in the first 5–6 months, plateau by 9–12 months, and then involute slowly over years.
What are the risk factors for developing IHs?
- Female sex
- Premature birth
- Low birth weight
- Advanced maternal age
- Placental abnormalities (e.g., placenta previa, pre-eclampsia)
What are the types of IHs based on morphology and distribution?
- Superficial IH – Bright red, raised lesion (strawberry-like)
- Deep IH – Bluish, subcutaneous swelling
- Mixed IH – Combination of superficial and deep features
- Segmental IH – Large, patterned growth along developmental units (higher risk of complications)
How do you differentiate IHs from congenital hemangiomas?
- IHs appear after birth, grow rapidly, and then involute.
- Congenital hemangiomas are fully formed at birth and follow either:
- RICH (Rapidly Involuting Congenital Hemangioma) – Shrinks quickly
- NICH (Non-Involuting Congenital Hemangioma) – Persists without regression
What features classify an IH as high-risk?
- Beard area IHs → Airway involvement (risk of subglottic hemangioma)
- Periocular IHs → Risk of amblyopia & visual impairment
- Lumbosacral IHs → Spinal dysraphism (LUMBAR syndrome)
- Large segmental hemangiomas → Risk of PHACE syndrome
- Ulcerated lesions → Pain, bleeding, secondary infection
What is PHACE syndrome, and when should you suspect it?
- PHACE stands for:
- Posterior fossa anomalies
- Hemangiomas (large segmental)
- Arterial anomalies
- Cardiac anomalies (coarctation of aorta)
- Eye abnormalities
- Suspect PHACE in large segmental hemangiomas (>5 cm) on the face or scalp.
What is LUMBAR syndrome?
- Lower body IH
- Urogenital anomalies
- Myelopathy
- Bony deformities
- Anorectal malformations
- Renal anomalies
What are the potential complications of untreated IHs?
- Ulceration (most common complication, occurs in ~16%)
- Airway obstruction (if in the beard area)
- Visual impairment (if periocular)
- Hearing loss (if ear involvement)
- Scarring & disfigurement (cosmetic concerns)
- Functional impairment (e.g., feeding difficulties if perioral)
What are the indications for treating IHs?
- Life-threatening IHs → Airway, liver, cardiovascular involvement
- Functional impairment → Visual, feeding, respiratory obstruction
- Ulceration → Pain, bleeding, infection, scarring
- Risk of disfigurement → Large facial lesions
- Syndromic associations → PHACE, LUMBAR
What is the first-line treatment for problematic IHs?
Oral propranolol (non-selective β-blocker).
What pre-treatment assessments are needed before starting propranolol?
- Cardiovascular & respiratory exam (auscultation, pulses, liver size)
- ECG (if arrhythmia or segmental hemangioma)
- ECHO (if PHACE syndrome or cardiac history)
- Baseline glucose (if hypoglycemia risk)
- Paediatric cardiology review (if significant comorbidities)
How is propranolol dosed for IH treatment?
- Start: 1 mg/kg/day in 2–3 divided doses
- Target dose: 2–3 mg/kg/day
- Duration: 6–12 months, adjust for weight gain
- Tapering: Over 2–4+ weeks to prevent rebound growth
What are the contraindications for propranolol in IH treatment?
- Absolute:
- Heart block (2nd/3rd degree)
- Symptomatic bradycardia
- Persistent hypoglycemia
- Hypersensitivity to propranolol
- Relative (use caution or monitor closely):
- Frequent wheezing/asthma
- Significant hypotension or bradycardia
- Extensive hepatic hemangiomas (risk of heart failure)
What are the monitoring requirements for propranolol therapy?
- First dose: Monitor HR/BP for 2 hours post-dose
- Stable treatment phase: Monitor HR/BP every 4–8 weeks
- Discontinue temporarily if: Poor feeding, intercurrent illness, wheezing
What are the alternatives to propranolol for IH treatment?
- Atenolol (selective β1-blocker) – Fewer CNS & respiratory side effects
- Topical timolol – For small, superficial IHs
- Oral corticosteroids – If β-blockers are contraindicated
- Laser therapy (Pulsed Dye Laser – PDL) – For ulcerated or residual lesions
- Surgery – Rare, used for functionally impairing or refractory IHs
What is the role of topical timolol in IH management?
Used for superficial, small hemangiomas (<2 cm).
What should be done if an IH shows rebound growth after stopping propranolol?
- Mild regrowth: Monitor and consider restarting propranolol for a few months
- Significant regrowth: Resume full-dose propranolol and reassess duration
What is involved in the management of segmental head/neck hemangiomas?
- MDT (Multidisciplinary Team)
- Screen for associated PHACE syndrome (30% risk):
- Pediatric neurologist + brain MRI with contrast before 12 weeks of age (no need for GA)
- Pediatric cardiologist, Echocardiogram
- Ophthalmologist – consider TSH for hypo/hyperthyroidism
What screening is needed for large IHs in the lumbosacral area?
- MDT (Multidisciplinary Team)
- Involve pediatrician
- US of abdomen and pelvis (urogenital defects)
- US of spine – < 4 months prior to ossification
- If abnormal, involve pediatric neurologist and spine MRI