Review of Isolated Hypoganglionosis and Presentation of a New Case Complicated With Sigmoid Volvulus

Study Overview
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Brief Summary
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Isolated hypoganglionosis is rare and constitutes 3-5% of all congenital neurological bowel diseases. Since hypoganglionosis is associated with symptoms such as chronic constipation or pseudo-obstruction, it can be confused with Hirschprung's disease and it is important to make a differential diagnosis before treatment. Our aim in this study; In addition to sharing the rarely isolated hypoganglionosis and sigmoid volvulus in a 45-year-old male patient with mental retardation, the investigators present a systematic comprehensive review of hypoganglionosis.

Detailed Description
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Isolated hypoganglionosis is rare and constitutes 3-5% of all congenital neurological bowel diseases. Since hypoganglionosis is associated with symptoms such as chronic constipation or pseudo-obstruction, it can be confused with Hirschprung's disease and it is important to make a differential diagnosis before treatment. Our aim in this study; In addition to sharing the rarely isolated hypoganglionosis and sigmoid volvulus in a 45-year-old male patient with mental retardation, the investigators present a systematic comprehensive review of hypoganglionosis. Pubmed and Scopus databases were scanned. No restrictions were used in the written language. Only isolated cases of hypoganglionosis were added to the study. In the cases; author, year of publication, country of publication, age, gender, surgical or non-surgical treatments, treatment results, and mortalities were examined. When sigmoid volvulus is seen in children and adolescents, congenital neurological bowel diseases should be considered in the differential diagnosis. Hirschprung's disease must be ruled out by rectal biopsy. The histopathological investigation should be carried out for the diagnosis and determination of surgical margins in isolated hypoganglionosis.

Official Title
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Systematic Review of Isolated Hypoganglionosis and Presentation of a New Case Complicated With Sigmoid Volvulus

Conditions
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Pathology, Sigmoid Volvulus, Hypoganglionosis

Intervention / Treatment
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* Diagnostic Test: Pathology result


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: isolated hypoganglionosis our case of isolated hypoganglionosis Exclusion Criteria: hypoganglionosis with Hirschsprung's disease
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Intervention/Treatment |
| --- |
|Diagnostic Test: Pathology result|Pathology result of rectal biopsy|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Mortality | Mortality of sigmoid volvulus due to isolated hypoganglionosis | three months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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colon, constipation, derotation, obstruction, Hypoganglionosis, intestinal neuronal dysplasia