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Published on: March 5, 2018
A case of chronic intestinal pseudo-obstruction secondary to primary visceral myopathy
Lisa S E Shim1, Guy D Eslick, Alex E Kan
1Department of Gastroenterology, Nepean Hospital, Penrith, NSW, Australia.
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 B(12), 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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