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Pediatr Neurosurg. 2011;47(5):337-41. doi: 10.1159/000336879. Epub 2012 May 9.

Craniosynostosis associated with neural tube defects: is there a causal association?

Author information

1
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.

Abstract

The majority of primary craniosynostosis cases are sporadic. Very few articles in the literature have described cases of primary craniosynostosis associated with neural tube defects (NTDs). This co-occurrence has been seen by most authors as just a coincidence. The authors report a clinical series of 4 patients of primary craniosynostosis associated with NTDs treated at their center. Among these 4 cases, 2 had lumbosacral myelomeningocele, 1 frontoethmoidal encephalocele and 1 had occipital encephalocele. Although the co-occurrence of craniosynostosis and NTD is said to be rare, there seems to exist a justified underlying explanation for the same. The NTD causes a decrease in intracranial pressure due to egress of cerebrospinal fluid in the malformed sac that results in a deficient cerebral impulse for cranial growth that might stimulate premature sutural fusion. Thus, all patients with NTDs should be thoroughly evaluated for this association and for possible surgical management.

PMID:
22572596
DOI:
10.1159/000336879
[Indexed for MEDLINE]

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