Chronic intestinal pseudo-obstruction syndrome in pediatric patients

The aim of this study was to report the presentation and outcome of 22 cons ecutive children (13 female) who presented with a syndrome of chronic intes tinal pseudo-obstruction with or without urinary tract involvement. We anal yse the main clinical and histopathological features and discuss therapeuti c 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 ag e and in 6 other patients diagnosis was made between the ages of 1 and 12 y ears. There was a family history in 4 patients. Investigations showed diffu sely 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 occurre d in 15/21 and megaureter in 2/21. Full thickness biopsies (n = 22) reveale d involvement of muscle layers in 8, and abnormal myenteric plexus on histo chemistry in 13. In 1, the biopsies were inconclusive. Recurrent urinary tr act infections occurred in all with structural urinary tract abnormality an d most had bacterial overgrowth. Severe recurrent episodes of obstruction w hich required parenteral nutrition (PN) occurred in all patients. Drugs wer e unhelpful and decompression ileostomies or colostomies were performed in 20/22. Five children died from sepsis (n = 3) or sudden death. Eleven patie nts remain partially or totally dependent on PN despite decompression ileos tomy 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 fe d. Despite careful histological study pointing to 2 main forms, myopathy an d neuropathy, the etiology of primary intestinal pseudoobstruction syndrome s remains unknown, It may present antenatally while most of the time the gu t and the urinary tract are diffusely involved. The condition has a high mo rbidity with a percentage requiring long-term PN. Although the mortality ra te is high (23 %), careful treatment of urinary tract infections and bacter ial overgrowth, decompression surgery and judicious use of PN allows surviv al to adult life.