Aplasia Cutis Congenita: Management of a Large Skull Defect With Acrania

Aplasia Cutis Congenita: Management of a Large Skull Defect With Acrania

Author Dutra, Leandro Brum Google Scholar
Pereira, Max Domingues Autor UNIFESP Google Scholar
Kreniski, Tessie Maria Google Scholar
Zanon, Nelci Autor UNIFESP Google Scholar
Cavalheiro, Sergio Autor UNIFESP Google Scholar
Ferreira, Lydia Masako Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Abstract Aplasia cutis congenita is a rare disorder characterized by absence of skin. Lesions typically occur on the vertex and are sometimes small, but they can affect deep tissues such as the skull bone and dura. Mortality is related to the depth and size of the lesion and can amount to a rate of more than 50% when full thickness is involved. the treatment remains controversial-both surgical and conservative managements are described. Minor lesions can be controlled with nonsurgical treatment, but large defects require early surgery. We report the case of a female newborn with acrania and scalp aplasia cutis congenita, which was treated with a bipedicle scalp flap based on the temporal vessels. Full- and partial-thickness skin grafts were used to cover the donor site on the temporo-occipital region. Postoperatively, the patient developed a liquorice cyst, which was treated with a shunt, and she has been followed up for evaluation of the bony defect closure and skull morphology. Her neuropsychomotor development is normal.
Keywords Aplasia cutis congenita
acrania
large skull and scalp defect
congenital deformity
Language English
Date 2009-07-01
Published in Journal of Craniofacial Surgery. Philadelphia: Lippincott Williams & Wilkins, v. 20, n. 4, p. 1288-1292, 2009.
ISSN 1049-2275 (Sherpa/Romeo, impact factor)
Publisher Lippincott Williams & Wilkins
Extent 1288-1292
Origin http://dx.doi.org/10.1097/SCS.0b013e3181ae2108
Access rights Closed access
Type Article
Web of Science ID WOS:000268400400078
URI http://repositorio.unifesp.br/handle/11600/31661

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