Management Quandary
Extensive Perineal Infantile Hemangioma with Associated Congenital Anomalies: An Example of the PELVIS Syndrome

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Case Presentation

A 2.9-kg fullterm female was noted to have a plaque-type infantile hemangioma (IH) involving the left labial, perineal, and coccygeal skin at birth. She also had a horizontally divided right labia, anteriorly-displaced anus, and sacral dimple. Cardiac examination revealed a 2/6 systolic ejection murmur at the left upper sternal border radiating to the left axilla, consistent with peripheral pulmonary stenosis. Physical examination was otherwise normal. Abdominal, pelvic, and spinal

Case

Examination in pediatric gynecology clinic confirmed the presence of a patent vagina. Examination in pediatric dermatology clinic during the fourth week of life demonstrated progression of the hemangioma of the labia, coccyx, and sacrum since birth, without ulceration (Figure 1). She was treated with topical clobetasol cream 3 times daily. Improvement of the IH was noted at 7 weeks of age (Figure 2). Spinal magnetic resonance imaging (MRI) at 3 months of age showed a tethered cord, with filum

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