Periocular Infantile Hemangioma With Induced Astigmatism
A 5-month-old girl has a 1.5 cm deep infantile hemangioma involving the left upper eyelid, first noted at 3 weeks and growing steadily. The lesion extends onto the left cheek and glabella in a segmental distribution. The eyelid margin is pushed down and there is 2 mm of ptosis, with the eyelid edge covering the superior pupil when she fixates. Cycloplegic refraction shows 2.25 D of astigmatism in the left eye versus 0.75 D on the right. She was born at 34 weeks and now weighs 6.2 kg. Her mother is worried about "steroids" and asks whether a cream could be used instead of an oral medicine. There is no stridor and the cardiac examination is normal.
1. Choose a treatment and justify it against topical timolol. Include starting dose, escalation, and monitoring.
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Oral propranolol is indicated; topical timolol is not adequate. The decision drivers are the deep component, the eyelid-margin position, pupillary coverage on fixation, and - most importantly - a 1.5 D anisometropia with 2.25 D of astigmatism, which is amblyogenic. Topical beta-blockers penetrate poorly through eyelid skin and do not reliably shrink a deep hemangioma fast enough to relieve optical deprivation.
Regimen:
- Baseline heart rate, blood pressure, weight, and glucose; ECG is not routinely required in an otherwise healthy infant, though many centres do one.
- Start 1 mg/kg/day divided three times daily, escalate over 1–2 weeks to 2–3 mg/kg/day if tolerated and needed. At 6.2 kg that is approximately 6 mg/day initially, rising to 12–18 mg/day.
- Observe 1–2 hours after the first dose and after each escalation for bradycardia and hypotension.
- Hypoglycaemia counselling is the key safety message: feed on a regular schedule, never allow prolonged fasting, hold the drug during vomiting or acute illness, and give a glucose-containing feed if a feed is missed.
- Expect colour change and softening within 2 weeks; continue to 12–18 months corrected age, then taper over 2–3 weeks to avoid rebound.
2. Outline the ophthalmological and developmental follow-up, and explain how you would treat her amblyopia risk.
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The eyelid haemangioma is only half the problem; the refractive error is what threatens binocular vision.
- Ophthalmology review within 2–4 weeks and then every 4–8 weeks while the lesion is active. Assess fixation pattern, cover test, cycloplegic refraction, and optic disc and media.
- Glasses for the astigmatism if the anisometropia persists beyond about 1.5 D, with part-time occlusion of the better eye if amblyopia develops. The duration is titrated to age and compliance, typically 1–2 hours daily in this age group.
- Re-refract at each visit - refractive error can improve as the hemangioma shrinks, so the prescription must be rechecked rather than left unchanged for a year.
- Dermatology review monthly during the proliferative phase, with photographs for objective comparison and a low threshold for escalation (intralesional or systemic corticosteroid, or surgical debulking) if there is no response within 2–4 weeks or if vision becomes obstructed.
- Watch for ulceration at the eyelid margin, which needs wound care and analgesia.
3. The segmental distribution prompts a PHACE question. What would you screen for, and how does it alter your use of propranolol?
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Segmental facial haemangioma involving the frontonasal and periorbital region is a recognised marker for PHACE (posterior fossa anomalies, haemangioma, arterial anomalies, cardiac defects, eye anomalies).
Screening:
- MRI/MRA of the brain and neck to look for posterior fossa malformations, cerebral arterial anomalies, and coarctation-related flow changes.
- Echocardiogram for coarctation of the aorta and other cardiac defects.
- Ophthalmological examination beyond refraction - look for optic nerve hypoplasia and retinal vascular anomalies.
- Endocrine and airway assessment if there are midline or ventral lesions; therapeutic laryngoscopy if any stridor develops.
Effect on propranolol: PHACE is not an absolute contraindication and most children tolerate propranolol, but severe cerebrovascular anomalies or significant aortic coarctation warrant caution and cardiology or neurology input before starting. Practically: begin treatment because the eye is at risk now, arrange imaging urgently in parallel, and involve cardiology if the echocardiogram is abnormal. Never let the PHACE work-up delay treatment of a vision-threatening haemangioma.