Challenges in the management of extensive aplasia cutis congenita

Author:

Ngeow AlvinORCID,Hui Cheryl,Chua Alvin Wen Choong,Koh MarkORCID,Ong Yee Siang,Poon Woei Bing

Abstract

Aplasia cutis congenita (ACC) is a rare group of congenital disorders characterised by focal or widespread absence of skin, predominantly affecting the scalp. A Malay female infant was born at 37 weeks with extensive ACC, affecting 37% of total body surface area, including her scalp and trunk. There is no consensus on the management of ACC given the rarity and variable presentation. A multi-disciplinary team comprising neonatologists, paediatric dermatologists, plastic surgeons and medical laboratory scientists at the skin bank, employed a more aggressive surgical approach with the aim of avoiding potentially catastrophic morbidity, including sagittal sinus haemorrhage and brain herniation. Out of several surgical options, the team used a staged artificial dermal matrix (Integra) and cultured epithelial autograft application, followed by regular wound dressing, and eventually allowed the child to achieve complete epithelialisation of her trunk, and most of scalp before she was discharged from hospital.

Publisher

BMJ

Subject

General Medicine

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Cited by 1 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. A rare case of extensive aplasia cutis congenita: Our surgical approach;Journal of Plastic, Reconstructive & Aesthetic Surgery;2023-05

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