EUPSA/ERNICA · Surgical treatment for Hirschsprung’s Disease: An ERNICA animation for parents and families
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Video3 min·Published Dec 2023Older

Surgical treatment for Hirschsprung’s Disease: An ERNICA animation for parents and families

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What the experts said20 expert statements
Hirschsprung's disease is also known as agangliosis of the colon
Clinical
Surgical removal of the affected part is usually performed 2 to 3 months after diagnosis, when the baby is strong enough and growing well
Clinical
Surgery is performed when there are no signs of enterocolitis (inflammation of the bowel)
Clinical
The surgery is performed under general anesthetic
Clinical
The aim of surgery is to remove the aganglionic bowel, which is the affected part with no ganglion cells present
Clinical
The transition zone should be removed, which is the area between the aganglionic part and the ganglionic bowel with normal ganglion cells
Clinical
The remaining bowel is connected to the anal canal in a pull-through procedure to restore fecal movement
Clinical
During the operation, tissue samples can be taken to confirm the presence of ganglion cells
Clinical
The procedure used to be done by open surgery with a large incision
Clinical
Nowadays, when possible, the procedure is done transanally via the anal canal, sometimes assisted by laparoscopy
Clinical
Different pull-through techniques exist including transanal pull-through, Swenson, Duhamel, and Soave procedures
Clinical
Surgical technique selection depends on the child, the length of affected bowel, surgeon preference, and hospital resources
Clinical
Preservation of the anal canal is crucial to maintain continence
Clinical
After surgery, the new connection between bowel and anal canal must be unobstructed and surgical wounds must heal well
Clinical
After surgery, patients may experience ongoing difficulties including constipation and lack of control over bowel movements
Clinical
Sometimes support with bowel management is necessary after surgery
Clinical
After surgery, individuals with Hirschsprung's disease may remain prone to bowel infections known as enterocolitis
Clinical
Structured regular follow-up care by a multidisciplinary team is essential for babies with Hirschsprung's disease
Guideline
Identifying complications or difficulties early is very important
Clinical
Post-operative symptoms can improve as children grow older
Clinical