Morphological patterns in children with ganglion related enteric neuronal abnormalities

Area of Science:

• Pediatric Surgery
• Gastroenterology
• Developmental Biology

Background:

• Hirschsprung's disease (HD) is a congenital disorder affecting the enteric nervous system, characterized by absent ganglion cells in the distal bowel.
• This absence disrupts normal bowel motility, leading to clinical symptoms of HD.

Purpose of the Study:

• To investigate and document the morphological patterns of enteric neuronal abnormalities in children presenting with Hirschsprung's disease in Pakistan.
• To analyze the prevalence of different ganglion-related abnormalities in this population.

Main Methods:

• A cohort of 92 children with clinical HD features were studied.
• Histopathological analysis of colorectal biopsies using Hematoxylin and Eosin staining.
• Statistical analysis using SPSS-17, including binomial chi-square tests for significance (p<0.05).

Main Results:

• Of 84 analyzed patients, 15.5% had normal ganglion cells, while 84.5% exhibited abnormalities.
• Hirschsprung's disease was diagnosed in 71.8% of cases, followed by hypoganglionosis (12.7%) and immaturity of ganglion cells (12.7%).
• Hypertrophic nerve fibers were significantly observed in HD, hyperganglionosis, and hypoganglionosis groups.

Conclusions:

• Hirschsprung's disease is prevalent in the Pakistani pediatric population, with other neuronal abnormalities also noted.
• The study highlights the association of hypertrophic nerve fibers with specific enteric neuropathies.
• Findings contribute to understanding the spectrum of enteric nervous system disorders in this region.