Yahya, Maya Mazuwin
(2006)
A review of hirschsprung's disease in Hospital Universiti Sains Malaysia 1999-2004.
Masters thesis, Universiti Sains Malaysia.
Abstract
Introduction
Hirschsprung' s disease is a developmental disorder of the enteric nervous system
causing congenital megacolon and the commonest cause of intestinal obstruction in the
neonatal period. Being the only Pediatric surgical unit until the end of 2005 Hospital
USM undertook all the references for Hirschsprung's disease in the upper east coast
region of Malaysia. The treatment of the disease started when a patient is suspected of
having the disease clinically. The patient would receive rectal irrigation until the
diagnosis is confirmed by rectal biopsy. When the diagnosis is confirmed the child
would have a colostomy created. When the child reached about one year old or weight
of 10 kilograms, the definitive procedure would be done. Until 2004, the procedure of
choice in the hospital was Duhamel procedure. After the definitive procedure, the
colostomy would be closed.
Objective:
The objective of the study is to review the children presenting with Hirschsprung's
disease to the hospital and described the demographics, mode and age of presentations,
diagnosis, operative treatment, complications and outcome of the patients.
Methodology:
The study was retrospective review study of the patients who were diagnosed and had
their definitive pull-through procedure in the hospital during a period of 5 years (from
February 1999 - February 2004). Patients who defaulted prior to the definitive
procedure were excluded.
Results:
There were 94 patients whose data was available for the review. There were 4: 1 male
to female ratio with age of presentation ranging from I day old and I3 years old with
73.4% presented within the age of 3 months. The most common of clinical presentation
was abdominal distension (87.2%) followed by vomiting (61.7%), poor feeding
(55.3%), delayed passage of meconium (47.9%) and chronic constipation (46.8%).
Seventeen percent (I 7%) of patients had other associated anomalies and 3.2% had a
family history. Sixty three percent (63.4%) were diagnosed with rectal suction biopsy.
Ninety five percent (95.7%) of patients had Duhamel type of pull-through procedure
with 3 had transanal pull-through and only one who had Soave. There was a mortality
recorded for the study and it was attributed to enterocolitis. The patient was also
having Down syndrome and congenital heart disease.
In the follow up period an average of 74% were free of complications of constipation,
enterocolitis, incontinent, redo operation. There was I 0.6% reoperation rate. Five
patients needed a complete redo of their definitive procedures.
Conclusion
The demographics finding of the study is quite similar to the patterns in the other parts
of the world. The presenting symptoms were also quite similar to other studies. The
definitive procedure of choice during the study time was Duhamel operation. The
operative outcome was safe and acceptable.
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