Huma Arshad Cheema, Arit Parkash, Hassan Suleman Malik, Zafar Fayyaz.
Congenital Short Bowel Syndrome: First Case Report from Lahore, Pakistan.
Pak Paed J Jan ;41(4):260-3.

Congenital short bowel syndrome (CSBS) is a very rare entity that is associated with malrotation and malabsorption. We are reporting a 20-day-old male with CSBS who was born to consanguineous parents and had history of death of three male siblings under 3 months of age with similar complaints and normal five female siblings. He presented with diarrhea since birth, failure to thrive and developed bilious vomiting during stay at hospital. An upper gastrointestinal endoscopy showed that gastric outlet was tilted abnormally and pouring of large amount of bile from duodenum with reverse peristalsis. Small bowel biopsies revealed focal blunting of villi, there was no crypt hyperplasia and intraepithelial lymphocytes were not increased. The lamina propria was edematous and was infiltrated by inflammatory cells composed of lymphocytes and plasma cells. An upper gastrointestinal series showed malrotation and slow passage of contrast from small bowel. A laparotomy showed the small bowel was only about 40 cm in length, confirming the diagnosis. Ladd's bands were also found and Ladd procedure was performed. Postoperatively parenteral nutrition was started and plan was made to switch gradually on an elemental (amino acid) formula through enteral route.

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