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Clinical Presentation
- Not visible at birth, only becomes apparent after days to weeks
- Growth during the first 6-9 months of life, followed by slow regression over several years (completed in 80-90% after 4 years, depending on size)
- Superficial lesions are bright red (strawberry naevus), deeper ones are bluish/violet
- Can be pressed away with a glass spatula (in contrast to port-wine stain)
- In cases of eyelid/orbital involvement: risk of amblyopia due to ptosis or pressure on the eyeball (anisometropia/astigmatism)
- Exophthalmos possible, increased when crying
- Associations
- PHACES Syndrome: Posterior fossa malformations, haemangioma over 5 cm on the head and neck, arterial anomalies, cardiac anomalies (e.g. coarctation of the aorta), eye anomalies, sternal or abdominal clefting or ectopia cordis
- Kasabach–Merritt Syndrome: multiple haemangiomas with visceral involvement, can lead to thrombocytopenia, high mortality
Work-up
- Refraction: Anisometropia/Astigmatism?
- Pediatric examination: systemic/extracutaneous involvement?
- Photodocumentation
- Ultrasound: documentation of size, depth
- MRI/CT: extent?
- Not necessarily required for small findings with classic history and clinic
Differential Diagnoses
- Vascular malformation: possible from birth, slow growth over decades, no spontaneous regression
- Port-wine stain / Naevus flammeus
- (Orbital) tumors
Treatment
- Oral propranolol (CH: Hemangiol solution 3.75 mg/ml) for at least 6 months (usually prescribed by a pediatrician)
- Caution: EKG before starting therapy
- Topical therapy with Timolol gel 0.5% (magistral medicine)
- Others: Steroid therapy (oral/topical), laser, excision, interferon therapy
- in case of risk of amblyopia: close orthoptic monitoring
Sources
- AWMF Leitlinie Infantile Hämangiome im Säuglings- und Kleinkindesalter
- EyeWiki Capillary Hemangioma
- Kanski’s Clinical Ophthalmology: A Systematic Approach; John E Salmon MD; Elsevier; 9th Edition (2019)
- Review of Ophthalmology: Neil J. Friedman; Peter K. Kaiser; William B. Trattler; Elsevier, 3rd Edition (2018)
- The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)