Hirschsprung’s disease: epidemiology, diagnosis, and treatment in a retrospective hospital-based study

• Main Authors: Andi Sinapati Palissei, Ahmadwirawan Ahmadwirawan, Muhammad Faruk
• Format: Article
• Language: English
• Published: Universitas Gadjah Mada 2021-04-01
• Series: Journal of the Medical Sciences
• Subjects: chronic constipation; pull-through; hirschsprung
• Online Access: https://jurnal.ugm.ac.id/bik/article/view/57309
• ISSN: 0126-1312; 2356-3931

Hirschsprung’s disease (HD), or congenital megacolon, is a disease characterized by the absence of ganglion cells in the myenteric (Auerbach’s) and submucosal (Meissner’s) plexuses of the intestine, causing maximum obstruction in neonates. The purpose of this study was to investigate patient characteristics, clinical presentation, investigations, surgical treatment, and outcome. This study is a retrospective study of HD cases. Data obtained from medical records at the institution. Of the 109 with a positive rectal biopsy diagnosis, 91 were patients with this disease. There were more cases in boys than girls with a ratio of 1.37: 1. The patients were grouped into 4 age groups: neonates 29 cases (26.61%); infants/toddlers/young children 55 cases (50.46%); children over 5 years16 cases (14.69%); and teenagers 9 cases (8.24%). The neonates generally presented with abdominal distension, green vomiting, and a history of delayed meconium release, while the toddler, child, and adolescent groups experienced constipation and abdominal distension. Furthermore, from 37 patients (33.94%) that got barium enema examination, the most common transition zone was observed in the rectosigmoid (17 patients, or 45.9%). In addition, The Duhamel procedure was the most frequent pull-through procedure found in our cohort which was performed in 40 patients (36.70%). In conclusion, HD is mostly found in male infants/toddlers/young children with abdominal distention and chronic constipation as common symptoms. The barium enema shows the rectosigmoid as the most common transition zone, while the Duhamel pull-through procedure is the primary definitive operative action performed.