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McKusick Kaufman syndrome is an Autosomal recessive syndrome characterized by post axial  polydactyly , hydrometrocolpos and congenital heart defects. We report a case of a five months old girl child second born of non               consanguineous marriage who presented with complaints  of abdominal distension and decreased urine output. She had syndactyly and polydactyly of all four limbs, USG and CT abdomen showed large midline cystic lesion measuring 1086 cm, ECHO findings of common AV valve with moderate    pulmonary hypertension, Laparotomy was done. Per  operative findings were pyometrocolpos, vaginal atresia,        persistent urogenital sinus and normal ovaries.


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