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Abstract

Our aim was to collect a large number of cases to characterize clinical presentation, outcome, and prognosis of chronic intestinal pseusoobstruction in children. We conducted a retrospective multicenter study that included children treated for chronic intestinal pseusoobstruction defined as recurrent episodes of intestinal obstruction with no mechanical obstruction, excluding Hirschsprung's disease. In all, 105 children, 57 boys and 48 girls, were studied, including five familial forms. Prenatal diagnosis was made in 18 patients. Eighty patients were less than 12 months old at onset; the disease began at birth for 37 patients. The most frequent signs were abdominal distension, vomiting, and constipation. Megacystis was noted in myopathies (7 cases), neuropathies (10 cases) and unclassified forms (13 cases). For all but three cases (two patients with CMV infection, one with Munchhausen-by-proxy syndrome), the associated diseases and disorders could not account for chronic intestinal pseusoobstruction as a secondary disorder. At least one full-thickness biopsy from the digestive tract was studied for 99 patients. The diagnosis recorded was visceral neuropathy in 58 cases, visceral myopathy in 17 cases, and uncertain or normal biopsy results in 24 cases. Seventy-eight children were fed intravenously, and only 18 were able to be fed orally throughout their illness. Seventy-one patients underwent surgery during their illness, and 217 surgical procedures, a mean of 3 per patient, were performed. Ostomy was the most performed procedure. Follow-up continued in 89 patients for 3 months to 16 years (mean 85 months). Forty-two patients were still fed by parenteral (39 patients) or enteral nutrition (3 patients) at the time of the study. Eleven patients died between the age of 1 month and 14 years 7 months.

Myotonic dystrophy as a cause of colonic pseudoobstruction: not just another constipated child

“…Bruinenberg et al., in 1996, also described a case of pseudoobstruction following appendectomy that was then determined to be to the result of DM 3 . Because the disease is relatively rare (incidence 1:8000), it is often not explained in detail in reviews of chronic intestinal pseudoobstruction syndrome 4 , 5 , or it is not mentioned at all 6 .…”

Volvulus as a complication of chronic intestinal pseudo-obstruction syndrome

“…Symptoms at first presentation in CIPS patients are abdominal distension (98%), vomiting (91%), constipation (77%), failure to thrive (62%), abdominal pain (58%), and diarrhea (31%) [7]. Radiographic evidence of dilated bowel with air–fluid levels is obligatory to diagnose CIPS [14, 15].…”

“…If enteral nutrition is not possible due to insufficient motility or enteral uptake, TPN is a necessity. Severely affected individuals may become chronically TPN dependent, but long-term TPN is related to high morbidity and mortality [7, 9]. Pharmacological therapy, such as prokinetics, may help in controlling symptoms [3, 5].…”

“…Multiple surgical interventions are strongly discouraged, though. In retrospective studies, 50–60% of CIPS patients were diagnosed via a laparotomy excluding mechanical obstruction [7, 9]. Those patients presenting with an acute onset suggestive for mechanical obstruction had a high risk to undergo laparotomy.…”

“…The decision for another, possibly unnecessary, intervention might be made on these grounds. Indeed in one of the retrospective studies, only few patients underwent single laparotomy [7]. In the other study, procedures performed during primary surgical intervention did not relieve symptoms in over 50% of patients [9].…”