Chronic intestinal pseudo-obstruction syndrome (CIPS) is a rare clinical condition in which impaired intestinal propulsion causes recurrent symptoms of bowel obstruction in the absence of mechanical obstruction. CIPS can be present as either primary or secondary although the latter is rare in children compared with adults. The primary abnormality consists of a degeneration of either the muscularis propria (visceral myopathy), or the myenteric plexus (visceral neuropathy). A 19 year old woman was recently admitted with recurrent abdominal distension and diarrhea. An abdominal plain X-ray revealed a marked dilated stomach and duodenum with some air-fluid levels. A small bowel series showed a diffusely dilated small bowel with multifocal angulation and spiculation. Computed tomograpy also revealed a dilated small bowel and distal ileal wall thickening. The patient was treated by duodenojejunostomy and ileal resection. Histologically the intestine showed thinning of the proper muscle layer with degeneration of smooth muscle cells replaced by fibrosis. Based on the specific histopathologic finding, in addition to the clinical history, physical finding and radiological evaluation, a diagnosis of sporadic visceral myopathy was rendered. The patient was treated through total parenteral nutrition with neostigmine, prokinetics, octreotide, and erythromycin postoperatively. However, she underwent ileostomy due to poor response from these therapies. After ileostomy, she improved without a recurrence of severe symptoms.
Adult ; Child ; Diagnosis ; Diarrhea ; Duodenum ; Erythromycin ; Female ; Fibrosis ; Humans ; Ileostomy ; Intestinal Pseudo-Obstruction* ; Intestines ; Myenteric Plexus ; Myocytes, Smooth Muscle ; Neostigmine ; Octreotide ; Parenteral Nutrition, Total ; Recurrence ; Stomach ; Young Adult

Chronic intestinal pseudo-obstruction syndrome (CIPS) is a rare clinical condition in which impaired intestinal propulsion causes recurrent symptoms of bowel obstruction in the absence of mechanical obstruction. CIPS can be present as either primary or secondary although the latter is rare in children compared with adults. The primary abnormality consists of a degeneration of either the muscularis propria (visceral myopathy), or the myenteric plexus (visceral neuropathy). A 19 year old woman was recently admitted with recurrent abdominal distension and diarrhea. An abdominal plain X-ray revealed a marked dilated stomach and duodenum with some air-fluid levels. A small bowel series showed a diffusely dilated small bowel with multifocal angulation and spiculation. Computed tomograpy also revealed a dilated small bowel and distal ileal wall thickening. The patient was treated by duodenojejunostomy and ileal resection. Histologically the intestine showed thinning of the proper muscle layer with degeneration of smooth muscle cells replaced by fibrosis. Based on the specific histopathologic finding, in addition to the clinical history, physical finding and radiological evaluation, a diagnosis of sporadic visceral myopathy was rendered. The patient was treated through total parenteral nutrition with neostigmine, prokinetics, octreotide, and erythromycin postoperatively. However, she underwent ileostomy due to poor response from these therapies. After ileostomy, she improved without a recurrence of severe symptoms.
Adult ; Child ; Diagnosis ; Diarrhea ; Duodenum ; Erythromycin ; Female ; Fibrosis ; Humans ; Ileostomy ; Intestinal Pseudo-Obstruction* ; Intestines ; Myenteric Plexus ; Myocytes, Smooth Muscle ; Neostigmine ; Octreotide ; Parenteral Nutrition, Total ; Recurrence ; Stomach ; Young Adult