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A baby boy weighing 1605 g was born at 35 weeks’ gestation with an unexpected large abdominal wall skin defect. This had been an in vitro fertilisation pregnancy in which two embryos were returned, one of which divided resulting in two monochorionic fetuses and a further third fetus. Interstitial laser therapy was performed at 13 weeks to one fetus resulting in a dichorionic twin pregnancy. The other live twin was completely unaffected. Formal pathological examination of the placenta of the affected twin revealed a fetus papyraceus.
The skin defect was symmetrical extending posteriorly to the erectae spinal muscles, inferiorly to below the umbilicus and superiorly to just below the nipple. There was an island of normal skin around the umbilicus. The abdominal contents were visible through a partially translucent membrane (fig 1). The abdominal wall musculature could not be identified. There were no other abnormalities found.
This presentation would fit the diagnostic criteria for type V cutis aplasia. There are nine recognised subgroups of aplasia cutis based on inheritance, skin defect pattern, location and associated abnormalities. Type V consists of a symmetrical defect of the trunk and/or limbs in association with a fetus papyraceus,1 which is more likely to be related to variation in blood flow through the placental blood vessels, embolic phenomena in vessels or a coagulopathy related to the dead fetus in utero.2
The baby has remained well but the skin defect has been slow to epithelialise. Treatment is currently conservative with regular dressings and there remains an option to skin graft. Skin grafting using allogenic cultured keratinocytes is thought to possibly reduce scar formation.3
This article has been adapted from Wadams Stephen, Garrett-Cox Robin, Kitteringham L. Aplasia cutis in association with a triplet pregnancy and fetus papyraceus Archives of Disease in Childhood - Fetal and Neonatal Edition 2008;93:206
Competing interests: None.
PAtient consent: Parental/guardian consent was obtained for publication of figure 1.
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