ArticlePediatric surgery international2019
Nationwide survey of outcome in patients with extensive aganglionosis in Japan.
Article in Pediatric surgery international, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed, 11 citations in OpenAlex.
- Associations ofBioscience reports · 2019Trial
- Risk factors of bowel perforation in neonates with Hirschsprung disease.Pediatric surgery international · 2025Article
- Prognostic factors for pediatric patients with severe intestinal motility disorders: a single institution's experience.Surgery today · 2025Article
- Bowel perforation in neonates with Hirschsprung disease: a case series and literature review.Pediatric surgery international · 2024Review
- Comment on a recent genetic study on the risk profile of Hirschsprung's disease from a pediatric surgeon's perspective.Annals of translational medicine · 2020Article
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Authors and funding
11 authors at 10 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeHirschsprung's disease-related short bowel syndrome (HDSBS) is characterized by aganglionosis that extends orally to 75 cm from Treitz's band. The condition is reported be associated with a high mortality rate of 50-80%. This retrospective study aimed to survey the current trends in HDSBS treatment in Japan.
methodsPatient data were extracted from the results of a nationwide survey we conducted, resulting in the retrospective collection of the data of 1087 HD patients from 2008 to 2012 in Japan.
resultsA total of 11 (0.9%) cases of HDSBS were noted. All patients underwent jejunostomy as neonates. Radical procedures performed in five patients (A-colon patch method in four, Duhamel's procedure in one). Ziegler's myotomy-myectomy and serial transverse enteroplasty (STEP) were performed in each patient as palliative procedures. No radical operations were performed in 4 of the 11 cases. The mortality rate was 36.4%. Four patients died, 1 patient who underwent STEP and 3 patients who received no radical procedures. The causes of death were sepsis due to enterocolitis or central intravenous catheter infection, and hepatic failure. All patients who underwent radical procedures survived and showed satisfactory outcomes.
conclusionHDSBS still showed a high mortality rate, although surgical approaches such as the A-colon patch method resulted in satisfactory outcomes.
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