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Body wall defects in two sibs

✍ Scribed by Helderman-van den Enden, A. T. J. M.; Bartelings, M. M.; van Kamp, I. L.; Oosterwijk, J. C.

Publisher: John Wiley and Sons
Year: 1997
Tongue: English
Weight: 19 KB
Volume: 73
Category: Article
ISSN: 0148-7299
DOI: 10.1002/(sici)1096-8628(19971128)73:1<15::aid-ajmg4>3.0.co;2-o

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✦ Synopsis

We describe 2 sibs, a male fetus with an unusual lumbar hernia and spina bifida occulta, and a female fetus with a median abdominoschisis. The first fetus had some signs of lumbocostovertebral syndrome (LCVS), which consists of a congenital lumbar hernia and associated abnormalities such as absent or hypoplastic ribs, hemivertebrae, and scoliosis. Abdominoschisis has not been described in LCVS, and the given abnormalities in the 2 sibs have not been published to date. One can hypothesize that vascular disruption of a somite or a group of somites may result in the described abdominal wall defects. We conclude that these abnormalities could be coincidental in the 2 sibs or could have a related, probably multifactorial, cause. Am.

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