Surgical management of aplasia cutis congenita

• Published in: Anales de pediatría (Barcelona, Spain : 2003) Vol. 83; no. 5; pp. 341 - 345
• Main Authors: Betancourth-Alvarenga, J E, Vázquez-Rueda, F, Vargas-Cruz, V, Paredes-Esteban, R M, Ayala-Montoro, J
• Format: Journal Article
• Language: Spanish
• Published: Spain 01.11.2015
• Subjects: Child; Ectodermal Dysplasia - surgery; Humans; Retrospective Studies; Scalp - pathology; Skin Transplantation; Surgical Flaps - surgery; Adams-Oliver's syndrome; Vertex; Vértex; Aplasia cutis; Aplasia cutis congénita; Colgajos cutáneos; Skin flaps; Síndrome de Adams-Oliver; Síndrome de Bart; Skin grafts; Bart's syndrome; Injertos cutáneos
• ISSN: 1695-9531
• DOI: 10.1016/j.anpedi.2015.02.005

Aplasia cutis congenita (ACC) is a rare congenital malformation that commonly involves the scalp, but can affect pericranium, bone and dura mater. Complications are rare, but can be fatal, so early treatment must be achieved. The treatment remains controversial with no consensus between the conservative and surgical approach. The aim of this study is to describe our experience in the management of ACC. Retrospective review of the medical records of all children up to 14 years diagnosed with ACC and treated between 2000 and 2013. There were a total of 22 cases of ACC with lesions ranging from 1cm (0.79 cm(2)) to 14cm (153.94 cm(2)). ACC of the scalp was found in 18 cases, with 3 in extremities and 1 in trunk. Conservative treatment was performed on 9 patients and 13 underwent surgical treatment (8 primary closures, 2 plasties, 2 skin grafts, and 1 skin flap). Two patients died due to complications of other diseases not related with the ACC. ACC is a rare disease that can be fatal. A complete initial assessment to establish early treatment is necessary to prevent this. Surgery should be considered as an initial therapeutic option in defects >4cm (>12.6 cm(2)) as it prevents the risk of fatal complications.