Introduction
Infantile hemangiomas (IH) are benign vascular neoplasms with a course marked by early proliferation and spontaneous involution
- IH are the most common tumors of infancy
- IH can be cutaneous or extracutaneous. Common extracutaneous sites include: Liver, Gastrointestinal tract, Larynx, CNS
Epidemiology
- Common in Caucasian infants, females more than males, premature, multiple gestation1
- 30% present at birth, 70% appear within first several weeks of life
Differential Diagnosis
Infantile Hemangioma2
A small hemangioma of infancy
https://en.wikipedia.org/wiki/Infantile_hemangioma
Clinical Course:
- Earliest sign of development: blanching of involved skin -> telangiectasias -> lesion
- Hallmark: rapid growth during the neonatal period
- Normal size 0.5-5cm, most are well circumscribed, focal crimson papules or plaques, color varies with depth from skin surface
- Common in the head and neck region
- Involution by age 9
Complications
- When they compress other structures (i.e. larynx, esophagus), occasionally they can ulcerate and bleed
- When they are large! Especially in the liver – they can grow and cause high output cardiac failure
- When associated with other congenital anomalies
PHACE Association1,2
Infantile hemangioma. Well-circumscribed red, violet, exophytic vascular tumor on the nose of a one-year-old child. Michael Sand, Daniel Sand, Christina Thrandorf, Volker Paech, Peter Altmeyer, Falk G Bechara - https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2903548/ https://en.wikipedia.org/wiki/Infantile_hemangioma
Posterior fossa brain abnormalities
Hemangiomas
Arterial malformations (MC CNS vascular abnormalities)
Coarctation of the aorta and Cardiac defects
Eye abnormalities
- Diagnosis requires hemangioma + 2 others
- Greatest source of morbidity: neurologic and cognitive impairment
- Suspect this in a patient with a large (> 5cm diameter) facial infantile hemangioma!
Work up:
- Brain MRI and MRA
- Cardiovascular imaging
- Ophthalmologic exam
SACRAL/PELVIS/LUMBAR Association1,3
- Large, segmental IH in perineal or sarcral areas that cross the midline are worrisome
- Include anogenital, spinal cord, renal abnormalities
- These warrant an ultrasound or MRI to look for associated findings
Kassabach-Merritt Syndrome
- Thrombocytopenia and/or coagulopathy resulting from platelet trapping within a vascular tumor
- Infantile hemangiomas are rarely responsible for this syndrome! More commonly tufted angioma or kaposiformhemangioendothelioma
Treatment
- First line: Propranolol – 3 mg/kg/day for 6 months4
- Timolol (topical) can be used for superficial lesions
- Corticosteroids (oral) – to slow growth and decrease size
- Surgery
- Pulsed dye laser can be an adjunct therapy
References
- Chen TS, Eichenfield LF, Friedlander SF. Infantile hemangiomas: an update on pathogenesis and therapy. Pediatrics 2013;131(1):99–108.
- Haggstrom AN, Garzon MC, Baselga E, et al. Risk for PHACE syndrome in infants with large facial hemangiomas. Pediatrics 2010;126(2):e418–26.
- Lowe LH, Marchant TC, Rivard DC, Scherbel AJ. Vascular malformations: classification and terminology the radiologist needs to know. Semin Roentgenol 2012;47(2):106–17.
- Léauté-Labrèze C, Hoeger P, Mazereeuw-Hautier J, et al. A Randomized, Controlled Trial of Oral Propranolol in Infantile Hemangioma. N Engl J Med 2015;372(8):735–46.