Nationwide survey of outcome in patients with extensive aganglionosis in Japan

Satoshi Obata, Satoshi Ieiri, Takashi Akiyama, Naoto Urushihara, Hisayoshi Kawahara, Masayuki Kubota, Miyuki Kono, Yuji Nirasawa, Shohei Honda, Masaki Nio, Tomoaki Taguchi

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Purpose: Hirschsprung’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. Methods: Patient 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. Results: A 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. Conclusion: HDSBS still showed a high mortality rate, although surgical approaches such as the A-colon patch method resulted in satisfactory outcomes.

Original languageEnglish
Pages (from-to)547-550
Number of pages4
JournalPediatric surgery international
Volume35
Issue number5
DOIs
Publication statusPublished - May 1 2019

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Japan
Short Bowel Syndrome
Hirschsprung Disease
Mortality
Colon
Jejunostomy
Enterocolitis
Surveys and Questionnaires
Liver Failure
Cause of Death
Sepsis
Catheters
Retrospective Studies
Newborn Infant
Infection

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Surgery

Cite this

Nationwide survey of outcome in patients with extensive aganglionosis in Japan. / Obata, Satoshi; Ieiri, Satoshi; Akiyama, Takashi; Urushihara, Naoto; Kawahara, Hisayoshi; Kubota, Masayuki; Kono, Miyuki; Nirasawa, Yuji; Honda, Shohei; Nio, Masaki; Taguchi, Tomoaki.

In: Pediatric surgery international, Vol. 35, No. 5, 01.05.2019, p. 547-550.

Research output: Contribution to journalArticle

Obata, S, Ieiri, S, Akiyama, T, Urushihara, N, Kawahara, H, Kubota, M, Kono, M, Nirasawa, Y, Honda, S, Nio, M & Taguchi, T 2019, 'Nationwide survey of outcome in patients with extensive aganglionosis in Japan', Pediatric surgery international, vol. 35, no. 5, pp. 547-550. https://doi.org/10.1007/s00383-019-04462-9
Obata, Satoshi ; Ieiri, Satoshi ; Akiyama, Takashi ; Urushihara, Naoto ; Kawahara, Hisayoshi ; Kubota, Masayuki ; Kono, Miyuki ; Nirasawa, Yuji ; Honda, Shohei ; Nio, Masaki ; Taguchi, Tomoaki. / Nationwide survey of outcome in patients with extensive aganglionosis in Japan. In: Pediatric surgery international. 2019 ; Vol. 35, No. 5. pp. 547-550.
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