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Childs Nerv Syst. 2008 Jun;24(6):723-9. Epub 2007 Oct 26.

Neurosurgical management of occult spinal dysraphism associated with OEIS complex.

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  • 1Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan. takato@ns.med.kyushu-u.ac.jp

Abstract

INTRODUCTION:

OEIS complex has been described as a combination of defects consisting of omphalocele (O), exstrophy of the cloaca (E), imperforate anus (I), and spinal defects (S). As the first three defects are life-threatening and treated on a priority basis, neurosurgical intervention for spinal defects is deferred until recuperation from abdominogenital repair. However, the best timing for neurosurgical operation has not been precisely described.

MATERIALS AND METHODS:

We reviewed our neurosurgical management of three cases (case 1: myelomeningocele; cases 2 and 3: terminal myelocystocele). At 2-6 (3.6 on average) months after birth, neurosurgical procedures, including reduction of the size of the lumbosacral cystic lesion and untethering of the spinal cord, were performed.

RESULTS:

During this period, the patients' weights increased from 1,911 to 3,368 g on average, and the lumbosacral cystic lesion was markedly enlarged. In all cases, no neurological deterioration was seen, and ventriculoperitoneal shunt was not indicated.

CONCLUSION:

Thus, neurosurgical procedures can be performed in patients weighing 3-4 kg and/or at an age of 3 months, after confirming recuperated conditions from abdominogenital repair. Careful observation should be made of the size of the lumbosacral cystic lesion and neurological deterioration.

PMID:
17962955
[PubMed - indexed for MEDLINE]
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