Neurosurgical management of occult spinal dysraphism associated with OEIS complex

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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.

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