A case of chronic intestinal pseudo-obstruction secondary to primary visceral myopathy

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.