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History: This premature male was born via C-section at 29 weeks of gestation to a 29 yo G4P2Ab1 female with a monochorionic triplet pregnancy. Triplet A was born with extinsive skin defects (Figures) involving the trunk, scalp and extremeties. Triplet B died in utero at 14 weeks of gestation. Triplet C died of hydrops fetalis at delivery.
Physical Examination: On the flanks (Fig A), abdomen (Fig B), and scalp (Fig C) showedsharply demarcated lesions covered with a glistening membrane, an absence of the epidermis and dermis very with prominent vasculature. No syndactyly, limb reduction or cleft palate was present. Corneal opacities and glaucoma were present.
Laboratory Findings: An MRI of the head revealed cerebral dysmorphism posteriorly with abscence of the corpus callosum. Colpocephaly, Schizocephaly, & Lissencephaly were also found. Abdomenal film revealed mild left hydronephrosis.
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