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A Rare Combination of VACTERL Association with Prune Belly Syndrome - Case Report and Review of Literature

Journal: International Journal of Forensic Science & Pathology (IJFP) (Vol.02, No. 06)

Publication Date:

Authors : ; ; ; ; ; ;

Page : 43-46

Keywords : Autopsy; Prune Belly Syndrome; Single Umbilical Artery; VACTERL;

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Abstract

VACTERL association (any three of vertebral, anal, cardiac, tracheo-esophageal, renal/radial and limb defects) and Prune belly syndrome (complete or partial triad of abdominal muscle deficiency, bilateral cryptorchidism and dilated urinary tract) are cngenital malformations affecting many body systems. These are derivatives of causally nonspecific disruptive events of the developmental field occuring during early blastogenesis. Many of these cases are sporadic in families without much recurrence. These lesions may be associated with other malformations but concurrence of VACTERL with Prune belly syndrome is extremely rare. Routine antenatal check up and ultrasonography can help in diagnosis of most of them. But, many times a detail autopsy examination is needed to detect the variety of malformations and categorise them into specific syndromes. We describe a rare combination of VCTERL with Prune belly syndrome and many associated anomalies of a 28 weeks neonate delivered vaginally by a 37 years second gravida.

Last modified: 2015-10-21 16:21:02