Current clinical features in diagnosis and treatment for immaturity of ganglia in Japan: analysis from 10-year nationwide survey

Background: To identify the current clinical features in diagnosis and treatment for immaturity of ganglia (IG) in Japan, we retrospectively analyzed data for patients with IG from the nationwide surveys in Japan. This survey was performed by Japanese Study Group of allied disorders of Hirschsprung’s disease (ADHD). Methods: In primary research, data on totally 355 cases of ADHD were collected for 10 years (2001–2010). Fifteen patients were IG. All IG patients were confirmed by pathological examination. In secondary research, detail questionnaires were sent and collected. Results: Male/female ratio was 9/6 and mean birth weight was 2474 g. All cases (100 %) were onset in neonatal period. Primary symptoms were abdominal distention (86.7 %), vomiting (53.3 %), and late egestion of meconium (26.7 %). An abnormal distention of intestine was recognized in 86.7 % on X-ray, and microcolon was recognized in 58.3 % on contrast enema. Caliber change was recognized in 58.3 % on laparotomy. An enterostomy was made in 13 patients (86.7 %), and an ileostomy was made in 69.2 %. Pathological diagnosis was performed in 100 %. Enterostomy was closed in 100 %. Conclusions: Totally, 15 definitive cases of IG in 10 years were collected and analyzed. All cases were onset in the neonatal period and almost all underwent enterostomy, but no mortalities occurred.