First Case Report of an Infant with Aplasia Cutis Congenita of Scalp and Myelomeningocele

Abstract Aplasia cutis congenita of scalp (ACCS) is a rare developmental anomaly. It has presented in children who have many concomitant anomalies. Large, deep defects can complicate by repeat local and systemic sepsis and life-threatening hemorrhage. In this paper, we describe, to the best of our k...

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Bibliographic Details
Main Authors: Angelo Silva Neto, Glena Marcelino, Cesar Garcia, Isadora Dantas, Isafran Silva, Emerson Oliveira
Format: Article
Language:English
Published: Thieme Revinter Publicações Ltda. 2016-05-01
Series:Brazilian Neurosurgery
Subjects:
Online Access:http://www.thieme-connect.de/DOI/DOI?10.1055/s-0036-1583934
Description
Summary:Abstract Aplasia cutis congenita of scalp (ACCS) is a rare developmental anomaly. It has presented in children who have many concomitant anomalies. Large, deep defects can complicate by repeat local and systemic sepsis and life-threatening hemorrhage. In this paper, we describe, to the best of our knowledge, the first case of a newborn boy with ACCS and myelomeningocele whose evolution with hydrocephalus has brought us a serious paradigm of using a shunt in the presence of tissue expanders. The treatment of hydrocephalus with third ventriculostomy associated with good aesthetic final result show an alternative to the use of shunt in this scenario, even in infants of young age. We review here therapeutic strategies and challenges with this disease.
ISSN:0103-5355
2359-5922