Abstract
Background A 13-year-old girl presented to the emergency room at her local hospital with an acute onset of vomiting, severe abdominal pain and distension. There was evidence of small-bowel obstruction on plain abdominal x-ray. Throughout the girl's adolescent years she was admitted to hospital numerous times for recurrent abdominal symptoms and underwent multiple sequential laparotomies. She had marked weight loss and a poor quality of life. The patient's symptoms were initially managed with intravenous fluids, bowel rest, and nasogastric decompression of the upper gut.
Investigations Peripheral blood tests with biochemistry and measurement of serum folate, vitamin B12, albumin, 25-hydroxyvitamin D, inflammatory markers, autoantibodies and thyroid function; gastrointestinal imaging (plain abdominal x-ray, small-bowel series, colonic transit study, and abdominal CT with oral contrast); MRI of the brain and lumbar puncture; upper endoscopy; and laparotomy with sero-muscular biopsy of the small bowel.
Diagnosis Chronic intestinal pseudo-obstruction secondary to primary visceral myopathy.
Management Prokinetic agents including oral cisapride and tegaserod, a venting gastrostomy, and total parenteral feeding.
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Shim, L., Eslick, G., Kan, A. et al. A case of chronic intestinal pseudo-obstruction secondary to primary visceral myopathy. Nat Rev Gastroenterol Hepatol 5, 584–588 (2008). https://doi.org/10.1038/ncpgasthep1254
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DOI: https://doi.org/10.1038/ncpgasthep1254