• Archives of Plastic Surgery
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• 제33권5호
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• pp.672-675
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• 2006

Purpose: The purpose of this study is to document the surgical methods used in infants with aplasia cutis congenita treated with allogenic dermal matrix and cultured epithelial autografts. Methods: The large defects in both lower legs were replaced with allogenic dermal matrix to avoid the postoperative hypertrophic scar contracture and a full-thickness skin biopsy was taken from right groin area simultaneously. We sent the specimen to a commercial laboratory for culture and obtained cultured epithelial autografts($Holoderm^{(R)}$) after 2 weeks, placed it over the allogenic dermal matrix. Results: The skin-defected area were nearly epithelialized after 2 weeks and there were no significant problem on during 6 months follow-up. Conclusion: The surgical method using allogenic dermal matrix and cultured epithelial autograft provided an excellent coverage of large skin defects of infant with aplasia cutis congenita.

• Neonatal Medicine
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• 제28권1호
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• pp.48-52
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• 2021

Aplasia cutis congenita (ACC) is a rare congenital disorder characterized by skin defects involving the epidermis, dermis, subcutaneous tissue, bone, and sometimes dura. It commonly affects the scalp in approximately 70% of cases, but the neck, trunk, and the extremities can also be affected. ACC can occur either as an isolated condition or associated with other anomalies and congenital syndromes, and it can be acquired either genetically or sporadically. Morbidity and mortality are associated with the defects of skull bone, dura, and other multiple anomalies. We herein report the case of a female infant, with a large scalp defect accompanied by a skull defect noted at birth, who developed mental retardation in the preschool years.

• Neonatal Medicine
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• 제18권2호
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• pp.359-364
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• 2011

목적: 선천성 피부 무형성증은 피부결손을 특징으로 드물게 발생하는 선천성 질환으로, 전신에서 나타날 수 있으며 선천성 기형을 동반하기도 한다. 국내에서의 연구는 증례보고에 국한되어 있어 출생 후 선천성 피부 무형성증으로 진단된 환아들의 임상 경과를 알아 보고자 하였다. 방법: 2004년 1월부터 2010년 12월까지 관동대학교 의과대학 제일병원에서 출생한 신생아 중 선천성 피부 무형성증이 확인된 8명을 대상으로 하여 의무 기록 정보를 후향적으로 분석하여 환아의 임상적 특징, 치료 및 예후에 관한 임상 경과를 분석하였다. 결과: 8명의 환아 중 3명이 남아였고 5명이 여아였으며, 이중 질식분만이 4명, 제왕절개분만이 4명이었다. 재태기간은 36주 3일에서 39주 4일이었으며, 이 중 37주 미만의 미숙아는 2명이었고, 출생 체중의 중간값은 2,685 g (2,180-3,625 g) 이었다. 신생아 황달을 보인 환아는 4명, 태변을 흡인한 환아는 1명이었다. 산모는 임신성 당뇨를 보인 경우가 4명이었으며, 임신성 고혈압은 2명, 양수과소증을 보인 경우는 3명이었다. Frieden의 분류에 의해 두부에 발생한 5명은 group 1에, 사지 및 체간에 발생한 3명은 group 7에 속했으며, 크기는 0.3 cm 에서 1.5 cm 로 모두 2 cm 이하였고, 표피나 진피에 국한된 결손이었다. 동반된 기형은 없었으며, 가족력은 1명에서 관찰되었다. 모두 보존적 치료만을 시행하였으며, 심각한 반흔을 남기지 않고 2개월에서 4개월 이내에 상처가 치유되었다. 결론: 선천성 피부 무형성증은 드물게 발생하는 질환으로 출생 후 세밀한 관찰을 통해 쉽게 진단할 수 있으며, 결손 부위가 크지 않을 경우 병변의 위치에 관계없이 보존적 치료로 치유될 수 있었다. 하지만, 동반 질환 및 다른 질환과의 감별을 위해서는 임상경과와 합병증 발생에 대한 장기적인 추적관찰이 필요하며, 결손 부위가 크거나 동반 질환의 경중에 따라 예후가 달라질 수 있기에 이에 대한 보다 많은 연구가 필요하겠다.

• Archives of Plastic Surgery
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• 제37권4호
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• pp.469-472
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• 2010

Purpose: Aplasia Cutis Congenita (ACC) is a rare disease characterized by the focal defect of the skin at birth, frequently involving scalp, but it may affect any region of the body. There are no etiology known but some conditions such as intrauterine vascular ischemia, amniotic adherences and viral infections are associated. The ideal treatment for the ACC is not known. Superficial and relatively small sized defects (< $3{\times}5\;cm$) may heal spontaneously and large defects related with risks of infection and bleeding may require aggressive surgical treatment. Hyalomatrix$^{(R)}$ is a bilayer of an esterified hyaluronan scaffold beneath a silicone membrane. It has been used as a temporary dermal substitute to cover deep thickness skin defect and has physiological functions derive from the structural role in extracellular matrix and interaction with cell surface receptor. This material has been used for the wound bed pre-treatment for skin graft to follow and especially in uncooperative patient, like a newborn, this could be a efficient and aseptic way of promoting granulation without daily irritative wound care. For this reason, using Hyalomatrix$^{(R)}$ for the treatment of ACC was preferred in this paper. Methods: We report a case of a newborn with ACC of the vertex scalp and non-ossified partial skull defect. The large sized skin and skull defect ($6{\times}6\;cm$) was found with intact dura mater. No other complications such as bleeding or abnormal neurologic sign were accompanied. Escharectomy was performed and Hyalomatrix$^{(R)}$ was applied for the protection and the induction of acute wound healing for 3 months before the split-thickness skin graft. During the 3 months period, the dressing was renewed in aseptic technique for every 3 weeks. The skin graft was achieved on the healthy granulation bed. Results: The operative procedure was uneventful without necessity of blood transfusion. Postoperative physical examination revealed no additional abnormalities. Regular wound management was performed in out-patient clinic and the grafted skin was taken completely. No other problems developed during follow-up. Conclusion: Hyalomatrix$^{(R)}$ provides protective and favorable environment for wound healing. The combination of the use of Hyalomatrix$^{(R)}$ and the skin graft will be a good alternative for the ACC patients with relatively large defect on vertex.