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

Pediatric Neurosurgery
Sachin A BorkarAshok Kumar Mahapatra

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.

References

Mar 1, 1992·Journal of Neurosurgery·H R Machado, H J Hoffman
Aug 1, 1985·American Journal of Medical Genetics·J R WatersonM Barr
Apr 1, 1982·Teratology·M M Cohen, R J Lemire
Nov 1, 1996·Surgical Neurology·J F Martínez-LageT Lluch

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