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

• Published in: Arquivos brasileiros de neurocirurgia Vol. 35; no. 2; pp. 152 - 156
• Main Authors: Silva Neto, Angelo, Marcelino, Glena, Garcia, Cesar, Dantas, Isadora, Silva, Isafran, Oliveira, Emerson
• Format: Journal Article
• Language: English
• Published: Rio de Janeiro, Brazil Thieme Publicações Ltda 01-06-2016; Thieme Revinter Publicações Ltda
• Subjects: Case Report | Relato de Caso; ectodermal dysplasia; hydrocephalus; myelomeningocele; neuroendoscopy; ectodermal dysplasia; neuroendoscopy; hydrocephalus; myelomeningocele; hidrocefalia; espinha bífida; aplasia cútis congênita
• ISSN: 0103-5355; 2359-5922
• DOI: 10.1055/s-0036-1583934

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.