{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,6]],"date-time":"2026-07-06T23:58:02Z","timestamp":1783382282528,"version":"3.54.6"},"reference-count":19,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T00:00:00Z","timestamp":1698019200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"JSPS KAKENHI","award":["20K08942"],"award-info":[{"award-number":["20K08942"]}]},{"name":"JSPS KAKENHI","award":["23K08037"],"award-info":[{"award-number":["23K08037"]}]},{"name":"Takeda Japan Medical Office","award":["20K08942"],"award-info":[{"award-number":["20K08942"]}]},{"name":"Takeda Japan Medical Office","award":["23K08037"],"award-info":[{"award-number":["23K08037"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biomolecules"],"abstract":"<jats:p>No standard diagnostic method or surgical treatment for congenital isolated hypoganglionosis (CIHG) has been established. This study aimed to analyze the clinical outcomes of patients with CIHG and identify the best surgical interventions provided thus far. Data on surgical interventions in 19 patients were collected between 1992 and 2020, including the type of enterostomy, type of revision, and length of the intestines. Ganglion cells in the myenteric plexus were enumerated using Hu C\/D staining. The ratio of the length of the small intestine to its height was defined as the intestinal ratio (IR). The outcomes were assessed using the stoma output, growth parameters including the body mass index (BMI), and parenteral nutrition (PN) dependency. All patients required a diverting enterostomy. The IR ranged from 0.51 to 1.75 after multiple non-transplant surgeries. The stoma types were tube-stoma, end-stoma, Santulli-type, and Bishop\u2013Koop (BK)-type. Patients with Santulli- or BK-type stomas had better BMIs and less PN dependency in terms of volume than those with end-stomas or tube-stomas. Two patients with BK-type stomas were off PN, and three who underwent an intestinal transplantation (Itx) achieved enteral autonomy. The management of CIHG involves a precise diagnosis using Hu C\/D staining, neonatal enterostomy, and stoma revision using the adjusted IR and Itx if other treatments do not enable enteral autonomy.<\/jats:p>","DOI":"10.3390\/biom13101560","type":"journal-article","created":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T01:41:21Z","timestamp":1698025281000},"page":"1560","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Historical Cohort Study of Congenital Isolated Hypoganglionosis of the Intestine: Determining the Best Surgical Interventions"],"prefix":"10.3390","volume":"13","author":[{"given":"Yohei","family":"Yamada","sequence":"first","affiliation":[{"name":"Department of Pediatric Surgery, Keio University School of Medicine, Tokyo 160-8582, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teizaburo","family":"Mori","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, National Center for Child Health and Development, Tokyo 157-8535, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nobuhiro","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Keio University School of Medicine, Tokyo 160-8582, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takumi","family":"Fujimura","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, National Hospital Organization Saitama National Hospital, Saitama 351-0102, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5443-0175","authenticated-orcid":false,"given":"Motohiro","family":"Kano","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, National Center for Child Health and Development, Tokyo 157-8535, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mototoshi","family":"Kato","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Keio University School of Medicine, Tokyo 160-8582, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masataka","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, National Center for Child Health and Development, Tokyo 157-8535, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoki","family":"Shimojima","sequence":"additional","affiliation":[{"name":"Department of Surgery, Tokyo Metropolitan Children\u2019s Medical Center, Tokyo 183-8561, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshihiko","family":"Watanabe","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Tokai University School of Medicine, Kanagawa 259-1193, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2644-1846","authenticated-orcid":false,"given":"Takako","family":"Yoshioka","sequence":"additional","affiliation":[{"name":"Department of Pathology, National Center for Child Health and Development, Tokyo 157-8535, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yutaka","family":"Kanamori","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, National Center for Child Health and Development, Tokyo 157-8535, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatsuo","family":"Kuroda","sequence":"additional","affiliation":[{"name":"Kanagawa Children\u2019s Medical Center, Yokohama 232-0066, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2987-5081","authenticated-orcid":false,"given":"Akihiro","family":"Fujino","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Keio University School of Medicine, Tokyo 160-8582, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,10,23]]},"reference":[{"key":"ref_1","first-page":"171","article-title":"Disorders of the innervation of the large intestine--classification and diagnosis. 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