Diagnosis and Management of Infantile Hemangioma

defects, urogenital anomalies, ulceration, myelopathy, bony deformities, anorectal
malformations, arterial anomalies, and renal anomalies], PELVIS [perineal hemangioma,
external genitalia malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate
anus, and skin tag] and SACRAL [spinal dysraphism, anogenital, cutaneous, renal, and urologic
anomalies, associated with an angioma of lumbosacral localization]) and may require treatment
of the IH to avoid functional or disfiguring sequelae. 76,77
CQ2. Evidence for Association of Cutaneous IH and Occult IH
Overall, limited literature addresses the association of a higher number of cutaneous IH and
extracutaneous IH. Some data from case series suggest support for a higher index of suspicion in
children with multiple lesions or with facial lesions in a beard distribution. Studies have
primarily assessed associations between cutaneous IH and hepatic IH and cutaneous facial IH
and airway IH. One study addressed associations with IH in the spinal area and reported that nine
of 48 children with cutaneous IH on the lumbosacral skin had intraspinal lesions (19%), though
the study did not report the number of cutaneous lesions. We summarize studies addressing
hepatic and airway IH below.
The basis for the association of a greater number of cutaneous IH with hepatic IH comes
primarily from case series including 453 infants with IH. 71,72,78-82 In one retrospective series,
investigators analyzed data from 26 children with hepatic IH (presentation of IH at birth or up to
4 months of age). 72 Among the 26, 18 also had multiple or diffuse cutaneous lesions (69%) and
underwent imaging, and 15 of 18 had multiple or diffuse liver IH. Investigators classified the
liver IH as focal (n=8 children), multifocal (n=12 children), or diffuse (n=6 children). Among
children with focal lesions, three had multiple cutaneous IH, two had a single cutaneous IH, and
three had no cutaneous IH. In the multifocal group, 11 of 12 children had multiple cutaneous
lesions (mean 14.25 ± 12.50 lesions) and liver IH. All but one of the 6 children with diffuse
hepatic IH had multiple cutaneous lesions. Across lesion types, cutaneous lesions generally
resolved before hepatic lesions.
Another series included 37 children, 16 percent of whom had three to five small cutaneous
lesions; 43 percent had six or more small cutaneous lesions; 16 percent had cutaneous miliary
(30-100 pinpoint lesions) lesions; 11 percent had a single large IH; and 14 percent had a
combination of a large and one or more small cutaneous IH. 78 Eight of 37 (22%) children had
concurrent hepatic IH. Children with cutaneous miliary IH had a greater number of hepatic IH
(n=7 to 35) than did infants with other cutaneous patterns. Another retrospective series reported
that 17 of 23 infants (53%) with hepatic IH had multiple (≥5) cutaneous IH. 80
In another retrospective series of children seen at referral centers, 62 children had six or more
cutaneous IH or one large (≥5 cm) cutaneous IH and seven had three to five small (