Early jejunostomy creation in cases of isolated hypoganglionosis: verification of our own experience based on a national survey

Yoshio Watanabe, Wataru Sumida, Hidemi Takasu, Kazuo Oshima, Yutaka Kanamori, Keiichi Uchida, Tomoaki Taguchi

Research output: Contribution to journalArticle

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Abstract

Purpose: Isolated hypoganglionosis (IH) is a rare disease, with few well-established therapeutic strategies. This study aims to verify our preliminary therapeutic strategies developed to date in a comparison with data obtained from a nationwide survey of congenital-type IH. Methods: Of the 90 registered IH cases assessed in a survey of Japanese pediatric surgical departments, 40 patients who had initially undergone jejunostomy (JE) and 41 treated with ileostomy (IL) were analyzed. Thirteen patients with JE sites located less than 50 cm from the ligament of Treitz were defined as having undergone upper jejunostomy (UJE). Postsurgical plain abdominal X-ray findings and survival rates, estimated using the Kaplan–Meier method, were used to evaluate improvements following stoma creation. Results: Improvements in bowel obstruction were observed in significantly more UJE patients (9/13) than non-UJE patients [20/63 (22 JE and 41 IL cases); p = 0.01]. Furthermore, the JE patients demonstrated a significantly higher survival rate than the IL patients (p = 0.01). Following the completion of the 10-year follow-up period, three JE patients died after undergoing massive bowel resection. Conclusions: To manage IH successfully, patients should undergo JE less than 50 cm from the ligament of Treitz during the neonatal period. Properly managing the distal intestines is important for achieving long-term survival.

Original languageEnglish
Pages (from-to)1509-1512
Number of pages4
JournalSurgery today
Volume45
Issue number12
DOIs
Publication statusPublished - Dec 1 2015

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Jejunostomy
Ileostomy
Ligaments
Survival Rate
Surveys and Questionnaires
Rare Diseases
Intestines
X-Rays
Pediatrics

All Science Journal Classification (ASJC) codes

  • Surgery

Cite this

Early jejunostomy creation in cases of isolated hypoganglionosis : verification of our own experience based on a national survey. / Watanabe, Yoshio; Sumida, Wataru; Takasu, Hidemi; Oshima, Kazuo; Kanamori, Yutaka; Uchida, Keiichi; Taguchi, Tomoaki.

In: Surgery today, Vol. 45, No. 12, 01.12.2015, p. 1509-1512.

Research output: Contribution to journalArticle

Watanabe, Yoshio ; Sumida, Wataru ; Takasu, Hidemi ; Oshima, Kazuo ; Kanamori, Yutaka ; Uchida, Keiichi ; Taguchi, Tomoaki. / Early jejunostomy creation in cases of isolated hypoganglionosis : verification of our own experience based on a national survey. In: Surgery today. 2015 ; Vol. 45, No. 12. pp. 1509-1512.
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abstract = "Purpose: Isolated hypoganglionosis (IH) is a rare disease, with few well-established therapeutic strategies. This study aims to verify our preliminary therapeutic strategies developed to date in a comparison with data obtained from a nationwide survey of congenital-type IH. Methods: Of the 90 registered IH cases assessed in a survey of Japanese pediatric surgical departments, 40 patients who had initially undergone jejunostomy (JE) and 41 treated with ileostomy (IL) were analyzed. Thirteen patients with JE sites located less than 50 cm from the ligament of Treitz were defined as having undergone upper jejunostomy (UJE). Postsurgical plain abdominal X-ray findings and survival rates, estimated using the Kaplan–Meier method, were used to evaluate improvements following stoma creation. Results: Improvements in bowel obstruction were observed in significantly more UJE patients (9/13) than non-UJE patients [20/63 (22 JE and 41 IL cases); p = 0.01]. Furthermore, the JE patients demonstrated a significantly higher survival rate than the IL patients (p = 0.01). Following the completion of the 10-year follow-up period, three JE patients died after undergoing massive bowel resection. Conclusions: To manage IH successfully, patients should undergo JE less than 50 cm from the ligament of Treitz during the neonatal period. Properly managing the distal intestines is important for achieving long-term survival.",
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AU - Uchida, Keiichi

AU - Taguchi, Tomoaki

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