Literature DB >> 9375916

Body wall defects in two sibs.

A T Helderman-van den Enden1, M M Bartelings, I L van Kamp, J C Oosterwijk.   

Abstract

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.

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Year:  1997        PMID: 9375916     DOI: 10.1002/(sici)1096-8628(19971128)73:1<15::aid-ajmg4>3.0.co;2-o

Source DB:  PubMed          Journal:  Am J Med Genet        ISSN: 0148-7299


  1 in total

1.  An incomplete form of lumbocostovertebral syndrome in association with atrial septal defect, arthrogryposis and clubfeet.

Authors:  Sriram Krishnamurthy; Seema Kapoor
Journal:  Indian J Pediatr       Date:  2009-02-10       Impact factor: 1.967

  1 in total

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