Abstract
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.
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Acknowledgments
This study was supported by a grant from The Ministry of Health, Labor Sciences Research Grants for Research on intractable disease. The authors thank The Japanese Society of Pediatric Surgeons, The Japanese Society of Pediatric Nutrition, Gastroenterology and Nutrition, and The Japanese Study Group of Pediatric Constipation. They also thank Dr. Brian Quinn for reading the manuscript, and Ms. Masutomi and Ms. Yamazaki of Department of Pediatric Surgery, Kyushu University, for their help in processing the data.
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Ieiri, S., Miyoshi, K., Nagata, K. et al. Current clinical features in diagnosis and treatment for immaturity of ganglia in Japan: analysis from 10-year nationwide survey. Pediatr Surg Int 31, 949–954 (2015). https://doi.org/10.1007/s00383-015-3774-0
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DOI: https://doi.org/10.1007/s00383-015-3774-0