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  • Title: Neurosurgical management of occult spinal dysraphism associated with OEIS complex.
    Author: Morioka T, Hashiguchi K, Yoshida F, Matsumoto K, Miyagi Y, Nagata S, Yoshiura T, Masumoto K, Taguchi T, Sasaki T.
    Journal: Childs Nerv Syst; 2008 Jun; 24(6):723-9. PubMed ID: 17962955.
    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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