The incidence and outcome of allied disorders of Hirschsprung's disease in Japan: Results from a nationwide survey

Tomoaki Taguchi, Satoshi Ieiri, kina miyoshi, Kenichi Kouhashi, Yoshinao Oda, Akio Kubota, Yoshio Watanabe, Hiroshi Matsufuji, Masahiro Fukuzawa, Takeshi Tomomasa

Research output: Contribution to journalArticle

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Abstract

Background Allied disorders of Hirschsprung's disease (ADHD) have been proposed to be the concept of the functional obstruction of the intestine with the presence of ganglion cells in the terminal rectum. They are classified into two categories based on pathology: (1) abnormal ganglia, including immaturity of ganglia, hypoganglionosis (HG), and intestinal neuronal dysplasia; (2) normal ganglia, including megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), segmental dilatation (SD), internal anal sphincter achalasia (IASA), and chronic idiopathic intestinal pseudo-obstruction (CIIP). Some of these show poor prognosis, therefore, the establishment of criteria and appropriate treatment strategies is required. Methods The questionnaires were sent to the 161 major institutes of pediatric surgery or gastroenterology in Japan, in order to collect the cases of ADHD during 10 years from 2001 and 2010. Results In total, 355 cases were collected. They included 28 immaturity of ganglia, 130 HG (121 congenital, 9 acquired), 18 intestinal neuronal dysplasia, 33 MMIHS, 43 SD, three IASA, and 100 CIIP. Of the 95 institutes, 69 (72.6%) had their own criteria for ADHD. Criteria were based on clinical symptoms and signs, and conventional pathological examinations. Prognosis was poor in congenital HG, MMIHS, and CIIP, while the others showed good survival rates. Conclusion Almost all Japanese cases of ADHD in the past 10 years were collected. Congenital HG and CIIP showed relatively high incidence, whereas acquired HG and IASA were extremely rare in Japan. The criteria of each disorder were also collected and summarized. Prognosis was poor in congenital HG, MMIHS, and CIIP.

Original languageEnglish
Pages (from-to)29-34
Number of pages6
JournalAsian Journal of Surgery
Volume40
Issue number1
DOIs
Publication statusPublished - Jan 1 2017

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Intestinal Pseudo-Obstruction
Hirschsprung Disease
Ganglia
Japan
Esophageal Achalasia
Anal Canal
Incidence
Dilatation
Gastroenterology
Rectum
Signs and Symptoms
Intestines
Survival Rate
Surveys and Questionnaires
Pediatrics
Pathology
Megacystis microcolon intestinal hypoperistalsis syndrome

All Science Journal Classification (ASJC) codes

  • Surgery

Cite this

The incidence and outcome of allied disorders of Hirschsprung's disease in Japan : Results from a nationwide survey. / Taguchi, Tomoaki; Ieiri, Satoshi; miyoshi, kina; Kouhashi, Kenichi; Oda, Yoshinao; Kubota, Akio; Watanabe, Yoshio; Matsufuji, Hiroshi; Fukuzawa, Masahiro; Tomomasa, Takeshi.

In: Asian Journal of Surgery, Vol. 40, No. 1, 01.01.2017, p. 29-34.

Research output: Contribution to journalArticle

Taguchi, Tomoaki ; Ieiri, Satoshi ; miyoshi, kina ; Kouhashi, Kenichi ; Oda, Yoshinao ; Kubota, Akio ; Watanabe, Yoshio ; Matsufuji, Hiroshi ; Fukuzawa, Masahiro ; Tomomasa, Takeshi. / The incidence and outcome of allied disorders of Hirschsprung's disease in Japan : Results from a nationwide survey. In: Asian Journal of Surgery. 2017 ; Vol. 40, No. 1. pp. 29-34.
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abstract = "Background Allied disorders of Hirschsprung's disease (ADHD) have been proposed to be the concept of the functional obstruction of the intestine with the presence of ganglion cells in the terminal rectum. They are classified into two categories based on pathology: (1) abnormal ganglia, including immaturity of ganglia, hypoganglionosis (HG), and intestinal neuronal dysplasia; (2) normal ganglia, including megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), segmental dilatation (SD), internal anal sphincter achalasia (IASA), and chronic idiopathic intestinal pseudo-obstruction (CIIP). Some of these show poor prognosis, therefore, the establishment of criteria and appropriate treatment strategies is required. Methods The questionnaires were sent to the 161 major institutes of pediatric surgery or gastroenterology in Japan, in order to collect the cases of ADHD during 10 years from 2001 and 2010. Results In total, 355 cases were collected. They included 28 immaturity of ganglia, 130 HG (121 congenital, 9 acquired), 18 intestinal neuronal dysplasia, 33 MMIHS, 43 SD, three IASA, and 100 CIIP. Of the 95 institutes, 69 (72.6{\%}) had their own criteria for ADHD. Criteria were based on clinical symptoms and signs, and conventional pathological examinations. Prognosis was poor in congenital HG, MMIHS, and CIIP, while the others showed good survival rates. Conclusion Almost all Japanese cases of ADHD in the past 10 years were collected. Congenital HG and CIIP showed relatively high incidence, whereas acquired HG and IASA were extremely rare in Japan. The criteria of each disorder were also collected and summarized. Prognosis was poor in congenital HG, MMIHS, and CIIP.",
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T1 - The incidence and outcome of allied disorders of Hirschsprung's disease in Japan

T2 - Results from a nationwide survey

AU - Taguchi, Tomoaki

AU - Ieiri, Satoshi

AU - miyoshi, kina

AU - Kouhashi, Kenichi

AU - Oda, Yoshinao

AU - Kubota, Akio

AU - Watanabe, Yoshio

AU - Matsufuji, Hiroshi

AU - Fukuzawa, Masahiro

AU - Tomomasa, Takeshi

PY - 2017/1/1

Y1 - 2017/1/1

N2 - Background Allied disorders of Hirschsprung's disease (ADHD) have been proposed to be the concept of the functional obstruction of the intestine with the presence of ganglion cells in the terminal rectum. They are classified into two categories based on pathology: (1) abnormal ganglia, including immaturity of ganglia, hypoganglionosis (HG), and intestinal neuronal dysplasia; (2) normal ganglia, including megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), segmental dilatation (SD), internal anal sphincter achalasia (IASA), and chronic idiopathic intestinal pseudo-obstruction (CIIP). Some of these show poor prognosis, therefore, the establishment of criteria and appropriate treatment strategies is required. Methods The questionnaires were sent to the 161 major institutes of pediatric surgery or gastroenterology in Japan, in order to collect the cases of ADHD during 10 years from 2001 and 2010. Results In total, 355 cases were collected. They included 28 immaturity of ganglia, 130 HG (121 congenital, 9 acquired), 18 intestinal neuronal dysplasia, 33 MMIHS, 43 SD, three IASA, and 100 CIIP. Of the 95 institutes, 69 (72.6%) had their own criteria for ADHD. Criteria were based on clinical symptoms and signs, and conventional pathological examinations. Prognosis was poor in congenital HG, MMIHS, and CIIP, while the others showed good survival rates. Conclusion Almost all Japanese cases of ADHD in the past 10 years were collected. Congenital HG and CIIP showed relatively high incidence, whereas acquired HG and IASA were extremely rare in Japan. The criteria of each disorder were also collected and summarized. Prognosis was poor in congenital HG, MMIHS, and CIIP.

AB - Background Allied disorders of Hirschsprung's disease (ADHD) have been proposed to be the concept of the functional obstruction of the intestine with the presence of ganglion cells in the terminal rectum. They are classified into two categories based on pathology: (1) abnormal ganglia, including immaturity of ganglia, hypoganglionosis (HG), and intestinal neuronal dysplasia; (2) normal ganglia, including megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), segmental dilatation (SD), internal anal sphincter achalasia (IASA), and chronic idiopathic intestinal pseudo-obstruction (CIIP). Some of these show poor prognosis, therefore, the establishment of criteria and appropriate treatment strategies is required. Methods The questionnaires were sent to the 161 major institutes of pediatric surgery or gastroenterology in Japan, in order to collect the cases of ADHD during 10 years from 2001 and 2010. Results In total, 355 cases were collected. They included 28 immaturity of ganglia, 130 HG (121 congenital, 9 acquired), 18 intestinal neuronal dysplasia, 33 MMIHS, 43 SD, three IASA, and 100 CIIP. Of the 95 institutes, 69 (72.6%) had their own criteria for ADHD. Criteria were based on clinical symptoms and signs, and conventional pathological examinations. Prognosis was poor in congenital HG, MMIHS, and CIIP, while the others showed good survival rates. Conclusion Almost all Japanese cases of ADHD in the past 10 years were collected. Congenital HG and CIIP showed relatively high incidence, whereas acquired HG and IASA were extremely rare in Japan. The criteria of each disorder were also collected and summarized. Prognosis was poor in congenital HG, MMIHS, and CIIP.

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