Infantile Hemangiomas

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

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Infantile Hemangioma2

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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

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A child with PHACE Association1,2

 

Posterior fossa brain abnormalities
Hemangiomas
Arterial malformations (MC CNS vascular abnormalities)
C
oarctation 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

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A Child with a sacral hematoma & MRI showing a spinal dyraphism1,3

  • 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

  1. Chen TS, Eichenfield LF, Friedlander SF. Infantile hemangiomas: an update on pathogenesis and therapy. Pediatrics 2013;131(1):99–108.
  2. Haggstrom AN, Garzon MC, Baselga E, et al. Risk for PHACE syndrome in infants with large facial hemangiomas. Pediatrics 2010;126(2):e418–26.
  3. 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.
  4. 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.