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Case Reports: Rare disease
Challenges in the management of extensive aplasia cutis congenita
  1. Alvin Ngeow1,
  2. Cheryl Hui2,
  3. Alvin Wen Choong Chua3,
  4. Mark Koh4,
  5. Yee Siang Ong2 and
  6. Woei Bing Poon1
  1. 1Department of Neonatal and Developmental Medicine, Singapore General Hospital, Singapore
  2. 2Plastic, Reconstructive and Aesthetic Surgery, Singapore General Hospital, Singapore
  3. 3Skin Bank Unit, Singapore General Hospital, Singapore
  4. 4Department of Dermatology, KK Women’s and Children’s Hospital, Singapore
  1. Correspondence to Dr Alvin Ngeow; alvin.ngeow.j.h{at}singhealth.com.sg

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.

  • dermatology
  • neonatal and paediatric intensive care
  • plastic and reconstructive surgery

https://doi.org/10.1136/bcr-2021-246627

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    Footnotes

    • Contributors AN was the primary physician who took care of the patient throughout the 6 months of hospitalisation and wrote the manuscript. CH is the plastic surgeon who meticulously applied the artificial dermal matrix, followed by cultured epithelial autograft, and carried out the subsequent wound dressings with AWCC. AWCC of the Singapore General Hospital Skin Bank provided the cultured epithelial autograft. MK assisted with the diagnosis of Aplasia Cutis Congenita, provided advice on management and edited the manuscript. Dr PWB and OYS were involved in the management of the patient, and approved of the final manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.