Guideline sources The following summarized guidelines for the evaluation and management of allied disorders of Hirschsprung's disease are prepared by our editorial team based on guidelines from the Ministry of Health, Labour and Welfare of Japan (MHLW 2018). 1 Calculator Calculator Diagnostic criteria for chronic idi Diagnostic criteria for megacysti Guidelines 1. Screening and diagnosis Diagnosis: suspect the diagnosis of megacystis microcolon intestinal hypoperistalsis syndrome in patients with observed microcolon and megalocystis confirmed on cystography, CT, or ultrasound. https://web.pathway.md/diseases/rec0NgqXVyqnFzyMM 1/4 6/23/23, 2:18 AM Allied disorders of Hirschsprung's disease Pathway B Differential diagnosis: differentiate mechanical obstruction and secondary intestinal pseudo- obstruction from chronic idiopathic intestinal pseudo-obstruction in adult patients. B 2. Classification and risk stratification Prognosis: expect long-term survival with appropriate treatment in patients with isolated hypoganglionosis and megacystis microcolon intestinal hypoperistalsis syndrome with appropriate treatment. B Show 2 more 3. Diagnostic investigations History and physical examination: elicit clinical history and perform physical examination to determine the duration of symptoms and bowel obstruction conditions in patients with suspected chronic idiopathic intestinal pseudo-obstruction. B Diagnostic imaging: Obtain diagnostic imaging to identify intestinal dilatation, air-fluid levels, and exclude mechanical obstruction in patients with suspected chronic idiopathic intestinal pseudo-obstruction. B Obtain gastrointestinal series in neonatal patients with suspected megacystis microcolon intestinal hypoperistalsis syndrome presenting with symptoms of bowel obstruction, such as abdominal distension and vomiting, and without organic obstruction. B 4. Diagnostic procedures Biopsy: perform a full-thickness biopsy of the gastrointestinal tract, especially the small intestine and sigmoid colon, for a definitive diagnosis of isolated hypoganglionosis. B Show 3 more 5. Medical management Pharmacologic therapy: Insufficient evidence to support the use of any medication for the management of patients with isolated hypoganglionosis. I Insufficient evidence to support the use of any of the following medications for the management of patients with chronic idiopathic intestinal pseudo-obstruction: prucalopride cisapride antibiotics (erythromycin). I https://web.pathway.md/diseases/rec0NgqXVyqnFzyMM 2/4 6/23/23, 2:18 AM Allied disorders of Hirschsprung's disease Pathway 6. Nonpharmacologic interventions Nutrition: Offer enteral and parenteral nutrition for nutritional management of patients with isolated hypoganglionosis, megacystis microcolon intestinal hypoperistalsis syndrome, and chronic idiopathic intestinal pseudo-obstruction. B Recognize that adequate nutritional support is required for a long period of time in many patients with isolated hypoganglionosis, megacystis microcolon intestinal hypoperistalsis syndrome, and chronic idiopathic intestinal pseudo-obstruction. B Probiotics: insufficient evidence to support the use of Japanese herbal medicine (daikenchuto) for the management of patients with megacystis microcolon intestinal hypoperistalsis syndrome or chronic idiopathic intestinal pseudo-obstruction. I Herbal products: insufficient evidence to support the use of probiotics for the management of patients with megacystis microcolon intestinal hypoperistalsis syndrome or chronic idiopathic intestinal pseudo-obstruction. I 7. Therapeutic procedures Antegrade continence enema: consider performing antegrade continence enema and antidromic enema to improve defecation in patients with chronic idiopathic intestinal pseudo-obstruction. C 8. Surgical interventions Intestinal decompression: consider performing upper jejunostomy in patients with isolated hypoganglionosis. C Show 4 more Radical surgery: consider avoiding radical surgery in patients with isolated hypoganglionosis. D Show 2 more Small bowel transplantation: Consider performing small bowel transplantation in patients with isolated hypoganglionosis in the following cases: discontinuation of parenteral nutrition is difficult even after the maximum use of the native intestinal tract under intestinal rehabilitation the number of central venous access routes is low repeated episodes of sepsis progressive hepatopathy. C Show 4 more References https://web.pathway.md/diseases/rec0NgqXVyqnFzyMM 3/4 6/23/23, 2:18 AM Allied disorders of Hirschsprung's disease Pathway 1. Mitsuru Muto, Hiroshi Matsufuji, Tomoaki Taguchi et al. Japanese clinical practice guidelines for allied disorders of Hirschsprung's disease, 2017. Pediatr Int. 2018 May;60 5 400 410. Open https://web.pathway.md/diseases/rec0NgqXVyqnFzyMM 4/4