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Split notochord syndrome variant: prenatal findings and neonatal management
Author(s) -
Agangi A.,
Paladini D.,
Bagolan P.,
Maruotti G. M.,
Martinelli P.
Publication year - 2005
Publication title -
prenatal diagnosis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.956
H-Index - 97
eISSN - 1097-0223
pISSN - 0197-3851
DOI - 10.1002/pd.1076
Subject(s) - imperforate anus , notochord , medicine , colostomy , anatomy , anus , sagittal plane , prenatal diagnosis , surgery , pregnancy , fetus , biology , embryo , genetics , embryogenesis , microbiology and biotechnology
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.