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• W1925752006 abstract "A 37-weeks-gestational-age boy was born to a 33-year-old mother whose pregnancy was complicated by late prenatal care, gestational diabetes, and oligohydramnios. Fetal ultrasound was notable for intrauterine growth restriction, the presence of ventricular septal defect, and abnormal (shortened) long bones and cranium. Physical examination upon delivery revealed extensive cutis aplasia estimated to be >50% body surface area including circumferential involvement of the anterior and posterior thorax, involvement of bilateral buttocks, posterior and lateral thighs, lateral calves, and upper arm involvement (Figure 1). Placental pathology showed a firm plaque measuring 4.3 × 3.9 cm, which contained necrotic material with immature fetal tissue compatible with fetal papyraceus or “vanishing twin.” Aplasia cutis congenita, a rare but well-described condition in the literature, refers to congenital absence of all skin layers.1Tempark T. Shwayder T. Aplasia cutis congenital with fetus papyraceus: report and review of the literature.Int J Dermatol. 2012; 51: 1419-1426Crossref PubMed Scopus (30) Google Scholar It most commonly affects the scalp as a single lesion, although it can also affect other areas of the body.2Lemke R.P. Machin G. Muttitt S. Bamforth F. Rao S. Welch R. A case of aplasia cutis congenita in dizygotic twins.J Perinatol. 1993; 13: 22-27PubMed Google Scholar, 3Maccario S. Fasolato V. Brunelli A. Martinelli S. Aplasia cutis congenital: an association with vanishing twin syndrome.Eur J Dermatol. 2009; 19: 372-374PubMed Google Scholar Typically, conservative medical management is sufficient,4Bui D. Ikeda C. Reconstruction of aplasia cutis congenital (group V) of the trunk in a newborn.Plast Reconstr Surg. 2003; 111: 2119-2120Crossref PubMed Scopus (16) Google Scholar, 5Simman R. Priebe Jr., C.J. Simon M. Reconstruction of aplasia cutis congenital of the trunk in a newborn infant using acellular allogenic dermal graft and cultured epithelial autografts.Ann Plast Surg. 2000; 44: 451-454Crossref PubMed Scopus (39) Google Scholar although owing to the extensive involvement in this case, innovative surgical grafting was warranted. This patient was treated with Integra (Plainsboro, New Jersey), an acellular dermal regeneration template, to the anterior thorax lesion (13 × 5.5 cm), and EpiFix (Marietta, Georgia), a dehydrated human amnion/chorion membrane allograft, to the posterior thorax lesion (13 × 8 cm) (Figure 2 available at www.jpeds.com). Circumcision was subsequently performed to send tissue samples for Epicel cultured epithelial autograft, which were then applied at 6 weeks of age. Ultimately, the anterior thorax graft was successful with evidence of confluent and well healed skin, and the posterior thorax showed evidence of hypergranulation without intact Epicel graft. Within several months, this patient has demonstrated complete resolution of his anterior thorax lesion and diminishing size of the posterior thorax lesion (4 × 2 cm)." @default.
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• W1925752006 date "2015-09-01" @default.
• W1925752006 modified "2023-10-16" @default.
• W1925752006 title "Extensive Aplasia Cutis Congenita with Associated Vanishing Twin Syndrome" @default.
• W1925752006 cites W1523147987 @default.
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• W1925752006 doi "https://doi.org/10.1016/j.jpeds.2015.06.041" @default.
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