Abstract
Prune Belly Syndrome is a rare birth defect with only few cases reported in our part. A newborn baby withPrune belly syndrome is presented along with review of literature.Key Words: Prune belly, abdominal muscle, urinary tract anomaly
Highlights
Prune Belly syndrome is an uncommon birth defect seen in 1 in 50,000 live births.[1]
We report a case of Prune Belly syndrome with rare association of spina bifida occulta and anorectal agenesis from our institute which is not reported from our part
Prune Belly syndrome is an autosomal recessive disease which occurs more commonly in male with less than 3% cases in female
Summary
Prune Belly syndrome is an uncommon birth defect seen in 1 in 50,000 live births.[1]. Musculoskeletal evaluation showed bilateral talipes equinovarus with a dermal sinus over back in midline, lumbar region.Cardiovascular examinations were normal With these clinical findings ,a diagnosis of Prune Belly syndrome was made. Right kidney had multiple subcortical cyst suggestive of polycystic disease whereas left kidney was dysplastic Both pelves were dilated but ureter and bladder were normal. The increased echogenicity of parenchyma is an indicator of underlying dysplasia of renal tissue during differentiation and maturation.[5,6] Our patient had polycystic changes in right and dysplastic changes in left kidney, Antenatal diagnosis by amniocentesis and ultrasonography and vesiculo-amniotic shunt in utero is the most promising therapeutic options in such babies.[7] Other surgical intervention recommended are vesicostomy, reconstruction of urinary tract and reimplantation of ureters
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