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

https://doi.org/10.19106/JMedSci005302202103

Andi Sinapati Palissei(1), Ahmadwirawan Ahmadwirawan(2), Muhammad Faruk(3*)

(1) Department of Surgery, Faculty of Medicine, Hasanuddin University, Makassar
(2) Division of Pediatric Surgery, Department of Surgery, Faculty of Medicine, Hasanuddin University, Makassar
(3) Department of Surgery, Faculty of Medicine, Hasanuddin University, Makassar
(*) Corresponding Author

Abstract


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.

Keywords


Chronic Constipation; Pull-through; Hirschsprung;

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DOI: https://doi.org/10.19106/JMedSci005302202103

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Journal of the Medical Sciences (Berkala Ilmu Kedokteran) by  Universitas Gadjah Mada is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Based on a work at http://jurnal.ugm.ac.id/bik/.