Chronic Intestinal Pseudo-Obstruction Syndrome in Pediatric Patients

Abstract

The aim of this study was to report the presentation and outcome of 22 consecutive children (13 female) who presented with a syndrome of chronic intestinal pseudo-obstruction with or without urinary tract involvement. We analyse the main clinical and histopathological features and discuss therapeutic management. Ten patients had signs of intestinal obstruction at birth, in which 6 presented antenatally with megacystis on ultrasound. Six children presented with constipation and/or obstruction between 1 and 6 months of age and in 6 other patients diagnosis was made between the ages of 1 and 12 years. There was a family history in 4 patients. Investigations showed diffusely dilated gut on x-ray with slow transit on small bowel follow through. Absent or abnormal motor migrating complex with low amplitude contractions were demonstrated on duodeno-jejunal manometry in 12/13. Megacystis occurred in 15/21 and megaureter in 2/21. Full thickness biopsies (n = 22) revealed involvement of muscle layers in 8, and abnormal myenteric plexus on histochemistry in 13. In 1, the biopsies were inconclusive. Recurrent urinary tract infections occurred in all with structural urinary tract abnormality and most had bacterial overgrowth. Severe recurrent episodes of obstruction which required parenteral nutrition (PN) occurred in all patients. Drugs were unhelpful and decompression ileostomies or colostomies were performed in 20/22. Five children died from sepsis (n = 3) or sudden death. Eleven patients remain partially or totally dependent on PN despite decompression ileostomy in 10/11. Six patients underwent colectomy and ileorectal pull-through, 2 of which remain on long-term PN, while the others are totally orally fed. Despite careful histological study pointing to 2 main forms, myopathy and neuropathy, the etiology of primary intestinal pseudoobstruction syndromes remains unknown. It may present antenatally while most of the time the gut and the urinary tract are diffusely involved. The condition has a high morbidity with a percentage requiring long-term PN. Although the mortality rate is high (23%), careful treatment of urinary tract infections and bacterial overgrowth, decompression surgery and judicious use of PN allows survival to adult life.

Precipitants of Constipation During Early Childhood

“…A variety of factors have been proposed as precipitants of constipation during early childhood, in- cluding a genetic predisposition, 12 beginning toilet training too early or having difficulty with toilet training, 13 dietary transitions from breast milk to formula 14 or from liquid to solid foods, 15 having painful or difficult bowel movements, 16 beginning school, 17 or a traumatic bathroom experience. 18 Until now, there has been no research examining the frequency and importance of such events.…”

Stomal prolapse in children with chronic intestinal pseudoobstruction: a frequent complication?

“…In enterostomy studies, stomal prolapse rates were 2% to 18.8% [10][11][12][13]. In the largest CIPO population studies, stomal prolapse rates varied from 5.4% to 25% [14][15][16]. However, global stomal complication rates for CIPO children are about 86% [16] and between 24% to 80.5% for other pathologies [5][6][7][8][9]12].…”

Lactulose Hydrogen Breath Test and Functional Symptoms in Pediatric Patients

“…It has also been described as a relevant condition in short bowel syndrome [5][6][7], cystic fibrosis [8], and chronic intestinal pseudo-obstruction [9]. In early studies, it is mentioned as a cause of chronic diarrhea, abdominal pain, or both [10].…”