Authors: Carlijn T de Betue Doeke Boersma Matthijs W Oomen Marc A Benninga Justin R de Jong
Publish Date: 2011/09/23
Volume: 170, Issue: 12, Pages: 1591-1595
Abstract
Chronic intestinal pseudoobstruction syndrome CIPS is a severe motility disorder of the gastrointestinal tract that presents with continuous or recurrent symptoms and signs of intestinal obstruction without evidence of a structural lesion occluding the intestinal lumen Mechanical obstruction might occur in these patients as well but is typically difficult to distinguish from an exacerbation of CIPS We report two pediatric cases in which mechanical obstruction by volvulus mimicked an exacerbation of CIPS requiring surgical intervention Conclusion Awareness of the possibility of true mechanical obstruction in CIPS patients during an exacerbation episode is needed as this is a severe condition and usually requires surgical interventionChronic intestinal pseudoobstruction syndrome CIPS is a rare severe gastrointestinal motility disorder characterized by recurrent or continuous signs and symptoms suggesting mechanical bowel obstruction in the absence of a lesion that occludes the intestinal lumen 3 14 15 Generally no curative treatment is available and morbidity and mortality are high Although the absence of mechanical obstruction is imperative for the initial diagnosis of CIPS true mechanical obstruction is a possible complication In CIPS patients this condition is particularly hard to recognize because the presentation is indistinguishable from common CIPS symptoms Here we report two cases of mechanical obstruction caused by volvulus in children with CIPSAn 8yearold boy diagnosed at 3 years of age with hypoperistaltic dysmotility of intestines and bladder as a variant of CIPS was admitted to our tertiary pediatric hospital Extensive histopathological examination showed no underlying disease In the past he had undergone two operations of the bladder size reduction and valve resection and biopsies of the bowel Neurological symptoms were absent and no other signs of involvement of mitochondrial disorders were observedAt admission the boy presented with abdominal pain abdominal distension increased gastric retention and decreased defecation frequency Initially these symptoms were interpreted as an exacerbation of CIPS and conservative treatment with gastric decompression by nasogastric tube was started During hospitalization the boy clinically deteriorated with increasing gastric retention abdominal distension and severe crampy abdominal pain There was no spontaneous defecation even after colonic irrigation At physical examination the abdomen was painful and progressively distended A plain abdominal radiograph showed extremely dilated bowel loops The ineffectiveness of the conservative treatment and meanwhile clinical deterioration were suggestive for mechanical obstruction Therefore we proceeded to surgical interventionVolvulus in an 8yearold boy with chronic intestinal pseudoobstruction syndrome An 8yearold boy with chronic intestinal pseudoobstruction syndrome presented with deteriorating symptoms of mechanical obstruction without improvement on conservative treatment At laparotomy an extremely dilated transverse colon with a diameter of about 20 cm was found as a result of transverse colonic volvulusA 2yearold boy with CIPS and a highoutput ileostomy for which no specific etiologic factor had been found was admitted to our hospital CIPS had been diagnosed in the first few months following premature birth and the patient had since been dependent of total parenteral nutrition TPN A split ileostomy had previously been constituted for bowel decompression Mitochondrial neurogastrointestinal encephalomyopathy MNGIE syndrome had been considered as underlying disorder However ophthalmoplegia polyneuropathy and other neurological symptoms were absent urine thymidine was normal and on MRI no leukoencephalopathy was seen Because MNGIE syndrome was therefore deemed unlikely MNGIE gene analysis was not performedAt this time he presented at the emergency department with a 3day course of abdominal pain progressive abdominal distension fluctuating febrile temperature coughing and malaise The symptoms were first thought to be part of an exacerbation of CIPS However the ileostomy production had decreased over the past few days which was in contradiction with the usual output of 3 L/day At physical examination an ill child was seen with a need for oxygen support and a painful and severely distended abdomen Plain abdominal radiography showed strongly dilated bowel loops Suspicion of a mechanical obstruction arose and gastric decompression by nasogastric tube was not awaited because of the severity of illness of the patient At laparotomy dilated fluidfilled bowel loops were seen which were caused by a volvulus of the small intestine The cause seemed to be torsion of the fluidfilled loops of decompensated bowel The volvulus was derotated and the intestine was drained with a suction device via the ileostomy The postoperative course was uneventful
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