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

NCT ID: NCT04363125

Last Updated: 2020-04-28

Basic Information

• Recruitment Status: COMPLETED
• Total Enrollment: 310 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2019-01-01
• Study Completion Date: 2020-04-20

Brief Summary

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

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.

Conditions

Pathology; Sigmoid Volvulus; Hypoganglionosis

Study Design

• Observational Model Type: CASE_ONLY
• Study Time Perspective: OTHER

Interventions

Pathology result

Pathology result of rectal biopsy

Intervention Type: DIAGNOSTIC_TEST

Eligibility Criteria

Inclusion Criteria

• isolated hypoganglionosis
• our case of isolated hypoganglionosis

Exclusion Criteria

• hypoganglionosis with Hirschsprung's disease

• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Inonu University

OTHER

Sponsor Role: collaborator

Dr. Ersin Arslan Education and Training Hospital

OTHER_GOV

Sponsor Role: lead

Responsible Party

Ufuk Uylas

Principal Investigator

Responsibility Role: PRINCIPAL_INVESTIGATOR

Principal Investigators

ufuk uylas, Specialist

Role: PRINCIPAL_INVESTIGATOR

Dr. Ersin Arslan Training and Resource Hospital

Locations

Dr. Ersin Arslan Training and Resource Hospital

Gaziantep, , Turkey (Türkiye)

Countries

Turkey (Türkiye)

Other Identifiers

23042020

Identifier Type: -

Identifier Source: org_study_id