Acquired isolated hypoganglionosis as a distinct entity: results from a nationwide survey

Satoshi Obata, Koichiro Yoshimaru, Kosuke Kirino, Tomoko Izaki, Satoshi Ieiri, Atsuyuki Yamataka, Tsugumichi Koshinaga, Jun Iwai, Hitoshi Ikeda, Hiroshi Matsufuji, Yoshinao Oda, Tomoaki Taguchi

Research output: Contribution to journalArticle

Abstract

Purpose: Acquired isolated hypoganglionosis (A-IH) is a late-onset intestinal pseudo-obstruction disorder and shows different pathophysiological findings from congenital isolated hypoganglionosis (C-IH). In this study, we retrospectively examined five cases of A-IH and investigated the features of A-IH. Methods: Five cases of A-IH were extracted from a nationwide retrospective cohort study in 10 years, from which totally 355 cases of Allied Disorders of Hirschsprung’s Disease (ADHD) were collected. Results: Ages of onset were between 13 and 17 years in three cases, and 4 years and 4 months in ones. Initial symptoms were abdominal distension and/or chronic constipation in 4 cases, whereas one exhibited intestinal perforation. Affected lesions varied from case to case, extending various length of intestinal tracts. All cases underwent multiple operations (average: 4.6 times), such as enterostomy, resection of dilated intestines, and/or pull-through. Pathological findings showed the decreased numbers of ganglion cells and degeneration of ganglion cells, whereas the size of the plexus was normal. Currently, all cases were alive and almost all eat regular food without requiring parenteral feeding. Conclusion: A-IH is rare, but distinct entity characterized by different clinical courses and pathological findings from those of C-IH. The outcome is considered to be favorable after a resection of affected intestine.

Original languageEnglish
Pages (from-to)215-220
Number of pages6
JournalPediatric surgery international
Volume35
Issue number2
DOIs
Publication statusPublished - Feb 5 2019

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Ganglia
Intestines
Enterostomy
Intestinal Perforation
Intestinal Pseudo-Obstruction
Hirschsprung Disease
Parenteral Nutrition
Constipation
Cell Size
Age of Onset
Cohort Studies
Retrospective Studies
Cell Count
Food
Surveys and Questionnaires

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Surgery

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Acquired isolated hypoganglionosis as a distinct entity : results from a nationwide survey. / Obata, Satoshi; Yoshimaru, Koichiro; Kirino, Kosuke; Izaki, Tomoko; Ieiri, Satoshi; Yamataka, Atsuyuki; Koshinaga, Tsugumichi; Iwai, Jun; Ikeda, Hitoshi; Matsufuji, Hiroshi; Oda, Yoshinao; Taguchi, Tomoaki.

In: Pediatric surgery international, Vol. 35, No. 2, 05.02.2019, p. 215-220.

Research output: Contribution to journalArticle

Obata, Satoshi ; Yoshimaru, Koichiro ; Kirino, Kosuke ; Izaki, Tomoko ; Ieiri, Satoshi ; Yamataka, Atsuyuki ; Koshinaga, Tsugumichi ; Iwai, Jun ; Ikeda, Hitoshi ; Matsufuji, Hiroshi ; Oda, Yoshinao ; Taguchi, Tomoaki. / Acquired isolated hypoganglionosis as a distinct entity : results from a nationwide survey. In: Pediatric surgery international. 2019 ; Vol. 35, No. 2. pp. 215-220.
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AU - Izaki, Tomoko

AU - Ieiri, Satoshi

AU - Yamataka, Atsuyuki

AU - Koshinaga, Tsugumichi

AU - Iwai, Jun

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