We describe six patients with an uncommon variant of infantile
hemangioma that we have termed reticular, occurring in the extremity, which were associated with intractable ulceration, anogenito-urinary-sacral anomalies, and sometimes cardiac overload. The extreme end of the spectrum is exemplified by a male neonate who presented with a stained, enlarged, pulsatile lower extremity, and
cardiac failure. He also had hepatic
hemangiomas and
ambiguous genitalia. Progressive soft tissue
necrosis and bony destruction necessitated
amputation. The histopathologic features differed from those of typical infantile
hemangioma: infiltrative (not lobular) and involving fascia, muscle, and bone. The mid-spectrum is illustrated by five females with reticular infantile
hemangioma of the lower limb, buttock, and perineum. Four of these infants had a ventral-caudal anomaly, including
omphalocele, recto-
vaginal fistula, solitary/duplex kidney,
imperforate anus, and tethered cord; one infant also had hepatic
hemangiomas. Deep ulcerations healed following
corticosteroid therapy; one patient required skin graft for closure of a thigh
wound. The minor end of the spectrum is exemplified a patchy lesion in the distal limb. The reticular variant of infantile
hemangioma can be confused with other vascular anomalies in the limb, such as capillary malformation,
cutis marmorata telangiectasia congenita, diffuse
arteriovenous malformation (
Parkes Weber syndrome) and capillary-lymphatico-venous malformation (
Klippel-Trenaunay syndrome). The macular network-like appearance of the
tumor and coexisting ventral-caudal structural anomalies is analogous to the association of posterior fossa brain malformations,
hemangiomas, arterial anomalies,
coarctation of the aorta and cardiac defects, and
eye abnormalities association in the craniofacial region.