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Volume : 43 Issue : 1 Year : 2024
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pelviperineology. 2021; 40(2): 96-102 | DOI: 10.34057/PPj.2021.40.02.006

Diagnosis and pathophysiology of Hirschsprung’s disease

1Department of Pediatric Radiology of the 1st State Hospital, Minsk, Belarus

Objective: :The aim of this research was to study the X-ray findings of Hirschsprung’s disease (HD) based on analysis of radiographs and videos published on the Internet and our own research, in order to increase the accuracy of X-ray diagnosis HD.
Materials and Methods: :An analysis of 56 radiographs and 2 videos of patients with a histologically confirmed diagnosis of HD was performed, including 25 radiographs from our own practice and 31 radiographs and 2 videos from articles published in PubMed and PMC. For comparison, cases without HD were compared, including radiographs from the author’s practice, where HD was excluded based on manometric detection of rectoanal inhibitory reflex (RAIR). For X-ray analysis, a contrast marker of known diameter was used, which was located near the anus.
Results: :In addition to the signs already described in the literature (the transition zone, microcolon, retention of contrast on post evacuation film, and the rectosigmoid ratio <1), we propose new anatomical signs of HD, i.e. the absence of an anterior displacement of the anal canal axis, an expansion of the retro-rectal space, and symptom of “frozen” segmentation. In addition, we suggest novel physiological diagnostic signs, i.e. the absence of the RAIR and defecation reflex.
Conclusion: :The combination of these signs makes it possible to diagnose HD. Shortening of the anal canal, with a noticeable displacement of it towards the pubis, as well as the presence of RAIR and wide opening of the anal canal during rectal emptying indicate the absence of HD. The use of these signs increases the accuracy of the HD diagnosis. An algorithm of diagnostics is offered.

Cite This Article

LEVIN M. Diagnosis and pathophysiology of Hirschsprung’s disease. 2021; 40(2): 96-102

Corresponding Author: LEVIN M.

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