236 New name, new criteria, old disease: paediatric intestinal pseudoobstruction (PIPO). (11th October 2021) - Pavic, Ana Mocic
Abstract : Introduction: Pediatric intestinal pseudoobstruction (PIPO) is a rare and complex gastrointestinal motility disorder affecting mainly the small intestine and the colon resulting in intestinal failure. Due to severe and reccurent episodes of pseudo-obstruction, children are unable to meet their nutritional needs enterally and might even need parental nutritional support. In 2018, diagnostic criteria were proposed by the European Society for Pediatric Gastroenterology, Hepatology and Nutrition with a goal to improve diagnosis, management and treatment while decreasing morbidity and mortality in these patients. Case Report: We present a case of female infant who was referred to the tertiary center for further medical evaluation due to recurrent abdominal distention and subileus at the age of 6 months. Prenatally, the child was diagnosed with megacystis, later evaluated as hypotonic urinary bladder without vesicouretheral reflux and with normal renal function. At four months, she experienced the first intestinal obstruction characterized with severe abdominal distention, stool absence and inability to tolerate enteral feeds. Mechanical obstruction was excluded by radiologic studies. Therefore she was transferred to our center for further diagnostic work-up. In accordance to the guidelines, in our center, rectal biopsy was performed to exclude Hirschsprung's disease. During initial evaluation, the patient experienced a new episode of distention and not tolerating oral feeds. Radiologic images during the episode showed distended bowel loops throughout the abdomen (Figure 1). She was treated by withholding enteral feed, intravenous fluids and prokinetics. Because of the progressive course of the distension she underwent exploratory laparotomy with formation of decompressive ileostomy at the age of 7 months (Figure 2). Full thickness biopsy were also performed at various site of small and large intestine and showed histological elements of intestinal neuronal dysplasia. In her further clinical course she experienced 3 more episodes treated conservatively, she was not able to tolerate full enteral feeds so we initiated parenteral nutrition to maintain adequate growth. In accordance with disease course so far, our patient met 2 out of 4 listed criteria for pediatric pseudointestinal obstruction, persistently dilated loops of small intestine with air fluid and inability to maintain adequate nutrition and growth via enteral feed. Conclusion: To conclude, we present a difficult case of PIPO, with early onset of abdominal distention, need for parenteral nutrition, and bladder involvement with uncertain prognosis. Nevertheless, early recognition of disorder leads to optimal management including formation of venting stoma, prevention of unnecessary repeated surgeries and better nutritional management.