Reticular Infantile Hemangioma of the Limb Can Be Associated with Ventral‐Caudal Anomalies, Refractory Ulceration, and Cardiac Overload

Abstract

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.

Infantile Hemangiomas Involving the Neuraxis: Clinical and Imaging Findings

“…26 The reticular variant of hemangioma has been associated with underlying ventral-caudal structural anomalies. 11 As noted, reticular hemangioma was present in 2 of our patients with intraspinal extension. Thus, the presence of reticular spinal hemangioma also warrants imaging follow-up with contrast-enhanced spinal MR imaging.…”

“…There are only scattered reports of true infantile hemangiomas involving the central nervous system (CNS). [3][4][5][6][7][8][9][10][11][12] Approximately 60% of infantile hemangiomas occur in the craniofacial region. 13 The lesions are described as superficial, deep, or of combined depth.…”

Hämangiome. Neue Aspekte zu Pathogenese, Differenzialdiagnosen und Therapie

“…Im Unter schied zu diesem und zum RICH/NICH ist das retikuläre Hämangiom GLUT1po sitiv. Eine Assoziation mit urogenitalen, neurologischen und kardialen Fehlbil dungen wurde beschrieben [19], scheint jedoch sehr selten zu sein [22]. …”

Hämangiome

“…Im Unterschied zu diesem und zum RICH/NICH ist das retikuläre Hämangiom GLUT1-positiv. Eine Assoziation mit urogenitalen, neurologischen und kardialen Fehlbildungen wurde beschrieben [19], scheint jedoch sehr selten zu sein [22]. …”