Cranial Neural Tube Defects

Česká verzia

Authors: F. Horn ¹; M. Smrek ¹; J. Babala ¹; M. Fuňáková ¹; D. Poruban ²
Authors place of work: Klinika detskej chirurgie LF UK a DFNsP, Bratislava ¹; Klinika stomatológie a maxilofaciálnej chirurgie LF UK a OÚSA ²
Published in the journal: Cesk Slov Neurol N 2010; 73/106(6): 706-710
Category: Short Communication

Summary

Introduction:
Neural tube defects (NTD) are congenital malformations of the central nervous system caused by failure of fusion in the course of embryological development. They occur in both the caudal and the cranial region. The authors present a group of patients with cranial NTD treated at the Department of Paediatric Surgery in Bratislava. The aim is to point out the importance of timing for surgery, especially in the management of hydrocephalus. Materials and methods: Ten patients with cranial NTD were treated at our surgery unit during the period 2000–2008. Seven patients presented with the occipital type of NTD, two patients with the parietal type, and one patient with the fronto-orbital type. Seven patients were suffering from current ventriculomegaly, and arachnoid cyst was also diagnosed in three patients. Six patients were operated on immediately after their birth, the following two, with non-severe meningocele, were operated upon at the age of three months, while two patients were referred to the institution at more advanced ages. Two patients with large bone defect were managed by two-stage repair – closure of the cephalocele and later, at the ages of four and six years respectively, reconstruction of the bone defect. Six patients with hydrocephalus had a ventriculoperitoneal (VP) shunt. Results: A total of eight children are developing normally, one patient has a slight impairment of muscle tone, and one patient is lagging significantly in her development. She was admitted too late, with ulcerations as a complication of hydrocephalus. Three patients with hydrocephalus have pharmacologically managed epilepsy. Conclusion: The group of patients is too small for proof, but it appears that patients with cranial NTD have a good chance of normal development, including those with hydrocephalus. Impairment of development arises largely out of maladministration of hydrocephalus. The authors suggest early surgical treatment for all cranial NTD, with ventriculoperitoneal shunt in concomitant hydrocephalus. Correction of bone defect can be performed as secondary treatment for patients in which it persists into later age.

Key words:
cranial neural tube defects – encephalocele – hydrocephalus – ventriculomegally

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