Aleya Khan, Abid Qazi, M.Naeem Khan.
Pseudo Prune Belly Syndrome, A Case Report.
J Coll Physicians Surg Pak Jan ;10(1):38-9.

A report is presented of a very rare occurrence of pseudo prune belly syndrome in a female child. There were multiple intestinal atresias with total length of a small intestine less than 10 cms which was thought to be incompatible with life. Details of this abnormality are discussed with a brief review of literature.

Case Report: A two days old female baby weighing 3.4 kg with bilious vomiting and marked abdominal distension was admitted. Mother had history of two previous abortions with malformed fetuses. On examination, patient was active with loose, wrinkled abdominal wall. Urinary bladder and kidneys were easily palpable. She had deformed small fingers in the left hand . Cardiovascular and respiratory status was normal. A plain abdominal X-ray in erect posture showed gas only in the stomach and first part of the duodenum. In the same X-ray, there were three shadows giving mass effects, two in the flanks and one in the lower abdomen representing hydronephrotic kidneys and distended bladder. An ultrasound confirmed grossly distended bladder with hydronephrotic kidneys. Her urea was 46mg% with 2.1mg% creatinine. An indwelling catheter per urethra allowed the urinary tract to be deflated considerably. She was stabilized with parenteralfluid infusions and nasogastric suction. With a provisional diagnosis of duodenal atresia, an exploratory laparotomy was performed that revealed multiple small bowel atresia with a microcolon. Kidneys were grossly hydronephrotic with dilated and tortuous ureters. Uterus, tubes and ovaries appeared normal. Multiple bowel atresias were such that normal bowel length was less than 10 cms. It was not considered feasible for surgical correction.Without any further procedures, her abdomen was closed. Parents having been explained decided to take the child home.

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