Neurosurgical management of occult spinal dysraphism associated with OEIS complex.

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.

• Authors: Takato Morioka, Kimiaki Hashiguchi, Fumiaki Yoshida, Kenichi Matsumoto, Yasushi Miyagi, Shinji Nagata, Takashi Yoshiura, Kouji Masumoto, Tomoaki Taguchi, Tomio Sasaki
• Journal: Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery (Childs Nerv Syst) Vol. 24 Issue 6 Pg. 723-9 (Jun 2008) ISSN: 0256-7040 [Print] Germany
• PMID: 17962955 (Publication Type: Case Reports, Journal Article)

Topics

• Anus, Imperforate (complications, surgery)
• Child
• Hernia, Umbilical (complications, surgery)
• Humans
• Infant
• Longitudinal Studies
• Magnetic Resonance Imaging
• Meningomyelocele (complications, surgery)
• Neurosurgical Procedures (methods)
• Retrospective Studies
• Spinal Dysraphism (complications, surgery)