[Twenty years diagnostic competence center for Hirschsprung's disease in Basel].

Abstract

BACKGROUND:

The experience gained by the Basel Hirschsprung Competence Center over 20 years is presented.

MATERIALS AND METHODS:

A total of 19,365 rectal mucosal biopsies were investigated in the 20 years between 1987 and 2006. All biopsies of rectal mucosa originated from 6,615 children aged between 1 week and 4 years. Biopsies were collected in teaching hospitals all over Germany and transported on dry ice by Intercity Courier Service. Serial sections of frozen tissue were made using a cryostat. Enzyme histochemical staining was performed.

RESULTS:

A total of 935 cases of Hirschsprung's disease (14%) were observed (769 cases of classical Hirschsprung's disease, 68 total colon aganglionosis, 98 ultrashort rectum aganglionosis). Total colon aganglionosis was found in 1.0% and the frequency of ultrashort Hirschsprung' disease was 1.4%. The quality of the histological results was confirmed by a second independent investigator. There were neither false positive nor false negative diagnoses. Enzyme histochemical staining results were readable within 2 h. Acetylcholinesterase, which is significantly increased in Hirschsprung's disease, was used for nerve fiber staining. Succinic and lactic dehydrogenases and nitric oxide synthase served as confirmatory proof of aganglionosis (elective nerve cell staining of the submucous plexus).

CONCLUSION:

Among 100 children with chronic constipation an average of 12 children were diagnosed with Hirschsprung's disease. Of these 2% showed total colon aganglionosis or ultrashort Hirschsprung's disease. Enzyme histochemical diagnosis of Hirschsprung's disease proved 100% reliable and time saving.

• Authors: E Bruder, W A Meier-Ruge
• Journal: Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen (Chirurg) Vol. 81 Issue 6 Pg. 572-6 (Jun 2010) ISSN: 1433-0385 [Electronic] Germany
• Vernacular Title: Zwanzig Jahre diagnostisches Hirschsprung-Kompetenzzentrum in Basel.
• PMID: 20458567 (Publication Type: English Abstract, Journal Article)

Chemical References

• Coloring Agents
• Acetylcholinesterase

Topics

• Acetylcholinesterase
• Benchmarking (standards)
• Biopsy
• Child, Preschool
• Coloring Agents
• Constipation (etiology, pathology)
• Female
• Hirschsprung Disease (diagnosis, epidemiology, pathology)
• Histological Techniques
• Humans
• Immunoenzyme Techniques
• Infant
• Infant, Newborn
• Intestinal Mucosa (innervation, pathology)
• Male
• Nerve Fibers (pathology)
• Neurons (pathology)
• Quality Assurance, Health Care (standards)
• Rectum (innervation, pathology)