referral criteria from primary care - infantile haemangioma (haemangiomas)

Last edited 07/2022 and last reviewed 08/2022

Immediate referral of infantile haemangiomas is indicated if (1):

• active bleeding or clinical compromise (eg, tachycardia, stridor, cardiac murmur, absent femoral pulses)
• beard distribution infantile haemangiomas and stridor: risk of obstructive airway haemangiomas (requires ear, nose, and throat assessment)
• uncontrolled pain from ulcerated lesions

There are 5 major indications for consideration of early treatment or need for further evaluation of infantile haemangiomas (IH):

• 1) life-threatening complications
  • include obstructing IHs of the airway, liver IHs associated with high-output congestive heart failure and severe hypothyroidism, and, rarely, profuse bleeding from an ulcerated IH
    • obstructing IHs of the airway
      • typically involve the subglottis, further compromising the narrowest portion of the pediatric airway
      • although the mean age at the time of diagnosis is about 4 months, symptoms usually present much earlier but are often mistaken as infectious or inflammatory croup or reactive airway disease
      • most children who are affected develop biphasic stridor and barky cough as the IH enlarges
      • segmental IH of the lower face ("beard distribution") or anterior neck and oral and/or pharyngeal mucosal IHs are the greatest risk factors for an airway IH
  • a consumptive form of hypothyroidism caused by the inactivation of thyroid hormones by type 3 iodothyronine deiodinase present in IH tissue can also be a complication of multifocal or diffuse hepatic IH
    
    
• 2) functional impairment or risk thereof
  • examples of functional impairment include visual disturbance and interference with feeding because of IH involvement of the lips or mouth (1,2)
    • periocular infantile haemangiomas; can result in astigmatism, anisometropia (where the refractive error differs between the two eyes), proptosis (bulging or protruding eyeballs), or amblyopia (inability to see clearly through one eye- also known as "lazy eye")
      • specific characteristics that place an infant at a higher risk for amblyopia include an IH size of >1 cm, upper eyelid involvement, associated ptosis, eyelid margin changes, medial location, and segmental morphology or displacement of the globe
    • lip or oral cavity haemangiomas; can result in breathing or feeding difficulties
    • more than five cutaneous haemangiomas; requires prompt further assessment for liver haemangiomas. Large liver haemangiomas can result in cardiac failure or consumptive hypothyroidism (1)
      
      
• 3) ulceration or risk thereof (1,2)
  • skin or mucosal ulceration of the IH surface occurs with an estimated incidence of 5% to 21% in referral populations
  • ulceration can lead to significant pain, bleeding, and secondary infection and virtually always results in scarring
  • ulceration occurs most frequently in infants younger than 4 months, during the period of active IH proliferation
  • IH associated with a high risk of ulceration (1)
    • segmental infantile haemangiomas >5 cm in size
    • haemangiomas of intertriginous sites or areas of frequent friction:
      • lips, columella, superior helix of ear, gluteal cleft, and/or perineum, perianal skin
        
        
• 4) evaluation to identify important associated structural anomalies
  • a small subset of children with IHs have associated congenital anomalies
  • PHACE syndrome (OMIM 606519)
    • Posterior fossa and other structural brain malformations; large haemangiomas of the face, neck, and/or scalp; anomalies of cerebral or cervical
      arteries; cardiac anomalies/coarctation of the aorta; eye abnormalities and sternal clefting or supraumbilical raphe
    • acronym "PHACES" is sometimes used instead to include potential ventral midline defects, specifically sternal cleft and/or supraumbilical raphe (2)
    • crebrovascular anomalies, present in more than 90% of patients with PHACE syndrome, are the most common extracutaneous feature of the syndrome, followed by cardiac anomalies (67%) and structural brain anomalies (52%) (2)
    • risk of PHACE syndrome in an infant presenting with a large segmental IH of the head or neck is approximately 30% (2)
  • LUMBAR syndrome
    • lower body haemangioma, urogenital abnormalities/ulceration, myelopathy, bony deformities, anorectal malformations
    • may best be viewed as the "lower half of the body" equivalent of PHACE syndrome (2)
      • IHs in LUMBAR syndrome are almost invariably segmental, involving the lumbosacral or perineal skin and often extending onto 1 leg
        
        
• 5) risk of leaving permanent scarring or distortion of anatomic landmarks
  • IHs can lead to permanent disfigurement either via scarring of the skin or distortion of anatomic landmarks
  • risk of disfigurement is much higher than the risk of functional or life-threatening consequences
  • measurements refer to infants under 6 months
    • facial infantile haemangiomas: risk of disfigurement via distortion of anatomical landmarks, scarring, or permanent skin changes
    • haemangiomas on nasal tip or lip and those >2 cm at any other facial location (or >1 cm if baby is 3 months or younger)
    • scalp infantile haemangiomas >2 cm: permanent alopecia (especially if the haemangioma becomes thick or bulky); also risk of profuse bleeding if ulcerates (typically more bleeding than at other sites)
    • neck, trunk, or extremity infantile haemangiomas >2 cm, especially if early in growth phase or if there is an abrupt transition from normal to affected skin (ledge effect): Greater risk of leaving permanent skin changes depending on anatomical location
    • breast infantile haemangiomas (female infants): problems with breast development (eg, breast asymmetry) or nipple contour

Reference:

• Surlis T, De Sa Reilly H, Sadlier M, Nelson J. Infantile haemangiomas BMJ 2022; 378 :e068734 doi:10.1136/bmj-2021-068734
• Krowchuk DP, Frieden IJ, Mancini AJ, Darrow DH, Blei F, Greene AK, Annam A, Baker CN, Frommelt PC, Hodak A, Pate BM, Pelletier JL, Sandrock D, Weinberg ST, Whelan MA; SUBCOMMITTEE ON THE MANAGEMENT OF INFANTILE HEMANGIOMAS. Clinical Practice Guideline for the Management of Infantile Hemangiomas. Pediatrics. 2019 Jan;143(1):e20183475.