Japanese clinical practice guidelines for allied disorders of Hirschsprung's disease, 2017

Abstract

Clinical practice recommendations for allied disorders of Hirschprung's disease are given for each CQ, along with an assessment of the current evidence. We hope that the information will be helpful in daily practice and future studies.

Massive atonic bleeding during cesarean delivery in a patient with chronic idiopathic intestinal pseudo‐obstruction: A case report and literature review

“…A full thickness intestinal biopsy revealed normal ganglion cells. Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) was diagnosed 1 . Genetic testing was declined by both parents.…”

“…In Japan, chronic idiopathic intestinal pseudo‐obstruction (CIIP) is classified as allied disorders of Hirschsprung's disease, 1,2 with similar symptoms and indicators to Hirschsprung's disease despite the presence of ganglion cells in the rectum 1 . CIIP patients present with bowel obstruction, gastrointestinal dilatation and chronic constipation without any mechanical cause.…”

“…CIIP patients present with bowel obstruction, gastrointestinal dilatation and chronic constipation without any mechanical cause. CIIP is diagnosed when intestinal ganglion cell abnormality and secondary chronic intestinal pseudo‐obstruction (CIPO) are excluded 1 . Outside Japan, CIIP is considered a myopathy among ‘primary’ or ‘idiopathic’ CIPO or a severe form of familial visceral myopathy (Table 1).…”

“…Outside Japan, CIIP is considered a myopathy among ‘primary’ or ‘idiopathic’ CIPO or a severe form of familial visceral myopathy (Table 1). 1–3 …”

Clinical practice guidelines for allied disorders of Hirschsprung's disease

“…In this issue of the Journal, Muto et al have defined this group of diseases as "allied disorders of Hirschsprung's disease"; outlined the newly established Japanese clinical practice guidelines for diagnosis; and provided appropriate treatment strategies for each disease. 6 The guidelines were developed using the methodologies in the Medical Information Network Distribution System (MINDS) and a unique systematic review approach in order to evaluate the small number of cases. The aim of the MINDS project is to help medical practitioners to fully utilize the information related to evidence-based medicine in their practice.…”