Split notochord syndrome variant: prenatal findings and neonatal management |
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Authors: | A Agangi D Paladini P Bagolan G M Maruotti P Martinelli |
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Institution: | 1. Prenatal Diagnosis Unit, Department of Gynecology and Obstetrics, University Federico II of Naples, Naples, Italy;2. Pediatric Surgery Unit, Bambino Gesù Hospital, Rome |
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Abstract: | Spilt notochord syndrome is an extremely rare form of spinal dysraphism characterized by a complete cleft of the spine and a persistent communication between endoderm and ectoderm. A variant of split notochord syndrome was diagnosed in a 25-week-old fetus showing a prolapsed congenital colostomy and a spinal cystic lesion. The final diagnosis included protruding colon segment, imperforate anus with a rectourethral fistula and lipomyelomeningocele. The ultrasound features of the condition and the post-natal management are discussed. The neonate was successfully treated with a posterior sagittal anorectoplasty, while the lipomyelomeningocele was resected at a later stage. Copyright © 2005 John Wiley & Sons, Ltd. |
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Keywords: | split notochord syndrome prenatal diagnosis congenital colostomy dorsal enteric fistula lipomyelomeningocele |
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