Clinical trial NCT04363125: Review of Isolated Hypoganglionosis and Presentation of a New Case Complicated With Sigmoid Volvulus
Official title: Systematic Review of Isolated Hypoganglionosis and Presentation of a New Case Complicated With Sigmoid Volvulus
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Pathology
Sigmoid Volvulus
Hypoganglionosis
Keywords: colon
constipation
derotation
obstruction
Hypoganglionosis
intestinal neuronal dysplasia
Lead sponsor: Dr. Ersin Arslan Education and Training Hospital
Sponsor class: OTHER_GOV
Collaborators: name: Inonu University
First posted: 2020-04-27
Enrollment: 310
Sex: ALL
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: CASE_ONLY
Time perspective: OTHER
Sampling method: NON_PROBABILITY_SAMPLE
Study population: our case of isolated hypoganglionosis and published isolated hypoganglionosis studies patients.

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.

Eligibility criteria: Inclusion Criteria:

* isolated hypoganglionosis
* our case of isolated hypoganglionosis

Exclusion Criteria:

* hypoganglionosis with Hirschsprung's disease

Primary outcomes:
measure: Mortality
timeFrame: three months
Secondary outcomes:
Other outcomes:
Locations:
city: Gaziantep
country: Turkey
facility: Dr. Ersin Arslan Training and Resource Hospital
Study officials:
affiliation: Dr. Ersin Arslan Training and Resource Hospital
name: ufuk uylas, Specialist
role: PRINCIPAL_INVESTIGATOR
References: