Evidence map›Paper›PMID 42370961›Full record

SynthesisPediatric surgery international2026

Congenital hypoganglionosis: phenotype-based outcomes and evolution of diagnosis and management-a systematic review.

Hiroki Nakamura, Hiroshi Yamakawa, Nozomi Aoki, Rina Tanaka, Sakiko Yoshimoto, Tokiko Okunobo, Ryosuke Satake, Prem Puri, Takashi Doi

Abstract readSystematic Review
PubMed Publisher
In one paragraph

Synthesis in Pediatric surgery international, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed
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1 · What the graph read from it

What it found

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

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3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

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4 · The record

Corrections and comments

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5 · Who and what money

Authors and funding

9 authors.

Hiroki NakamuraDepartment of Pediatric Surgery, Kansai Medical University, Osaka, Japan.
Hiroshi YamakawaDepartment of Pediatric Surgery, Kansai Medical University, Osaka, Japan.
Nozomi AokiDepartment of Pediatric Surgery, Kansai Medical University, Osaka, Japan.
Rina TanakaDepartment of Pediatric Surgery, Kansai Medical University, Osaka, Japan.
Sakiko YoshimotoDepartment of Pediatric Surgery, Kansai Medical University, Osaka, Japan.
Tokiko OkunoboDepartment of Pediatric Surgery, Kansai Medical University, Osaka, Japan.
Ryosuke SatakeDepartment of Pediatric Surgery, Kansai Medical University, Osaka, Japan.
Prem PuriUniversity College Dublin, Dublin, Ireland.
Takashi DoiDepartment of Pediatric Surgery, Kansai Medical University, Osaka, Japan. doi.tak@kmu.ac.jp.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

purposeCongenital hypoganglionosis (CH) is a rare enteric neuropathy characterized by reduced and small ganglion cells in the myenteric plexus. We aimed to systematically review the clinical outcomes and diagnostic evolution of CH in children.

methodsA systematic review was conducted in accordance with PRISMA guidelines using PubMed and Web of Science (1978-2025). Of 396 identified records, 32 studies including 298 pediatric patients were analyzed. Disease extent was classified as diffuse/panintestinal, colon-limited, segmental, or unspecified.

resultsAmong 298 patients, 144 (48%) were classified as diffuse/panintestinal, 25 (8%) as colon-limited, 22 (7%) as segmental, and 107 (36%) were unspecified. Among patients with diffuse/panintestinal disease for whom mortality data were available (n = 105), 28 deaths (26.7%) were documented, whereas no deaths were reported in colon-limited cases (0/24). Most diffuse cases presented in the neonatal period. Diagnostic methods evolved over time, with increasing use of quantitative HuC/D staining after 2020. Surgical management also shifted from resection-based procedures to phenotype-based strategies, including stoma formation and intestinal rehabilitation.

conclusionDiffuse or panintestinal neonatal forms are associated with high mortality, whereas colon-limited disease is associated with favorable survival. Advances in diagnostic techniques have supported phenotype-based management. Extent-based stratification may improve outcomes in severe cases.

Indexed as

Hirschsprung DiseaseHumansInfant, NewbornPhenotypeCongenital hypoganglionosisEnteric neuropathyIntestinal motility disorderPediatric surgerySystematic review

Identifiers

PMID42370961

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.