Treating Children with Persistent Symptoms After Surgery for Hirschsprung Disease

There could be several reasons children have problems with soiling after surgery, including:

• Lack of bowel sensation: A child may be unable to feel that the rectum is full of stool or they are unable to detect the difference between gas and stool.
• Abnormal sphincter function: Abnormal sphincter function can occur if the sphincter was excessively stretched during the pull-through procedure or during a previous surgery.
• Encopresis: Encopresis can occur as a result of lack of sensation that the rectum is full or extreme constipation, resulting in an overflow of the bowels.

Unfortunately, sphincter weakness and abnormal bowel sensation cannot usually be repaired with surgery, which is why symptoms may persist afterwards. But, they can be treated successfully with a bowel management program.

After a clear diagnosis of the problem has been made, our colorectal specialists will develop specific and individualized protocols to address soiling that may include the following therapeutic approaches:

• Biofeedback training: Biofeedback training is a mind-body technique that helps patients gain control over certain bodily functions using visualization, meditation, breathing or relaxation techniques.
• Pelvic physical therapy: Anal sphincter and pelvic floor exercises can help strengthen the muscles surrounding the bowel and improve its function, reducing episodes of fecal incontinence.
• A constipating diet (for loose stools): May include a diet rich in grains and fiber.
• Laxatives/enemas: Laxatives and enemas can help relieve symptoms, but should only be used when necessary. In some cases, laxatives, stool softeners and enemas may make the problem worse because the child needs to feel the sensation caused by a full rectum.

The causes of bowel obstruction symptoms in children who have undergone surgery for Hirschsprung disease may be anatomical or caused by another underlying condition.

Bowel obstruction symptoms include:

• Abdominal distention
• Bloating
• Borborygmi (stomach growling)
• Vomiting
• Ongoing severe constipation
• Difficulty passing stools without the assistance of rectal stimulation or irrigations

Diagnosing Causes of Bowel Obstruction Symptoms

There can be many reasons children continue to experience bowel obstruction symptoms after surgery. Specialists in Colorectal and Pelvic Reconstructive Surgery have a specific method to diagnosing whether or not the underlying cause is anatomical, caused by another underlying physical disorder or a functional problem.

Their diagnostic protocol includes checking for:

• Anatomical problems: Anatomical problems, or abnormalities of the physical structure are usually related to the initial surgery, including:
• Anastomotic stricture, or the narrowing of the surgical connection between two structures in the GI tract.
• A twist or kink in the segment of the colon or rectum where the pull-through has been performed.
• An excessively dilated Duhamel pouch
• Scar tissue that develops post-surgery that may need to be surgically removed.
• Other underlying physical disorders: If there appears to be no anatomical issues causing the symptoms, a rectal biopsy may be performed to check for abnormal innervation (the abnormal connection of nerve fibers) or any other possible problems.
• Functional problems: Functional problems may be an underlying factor including:
• The absence of the rectoanal inhibitory reflex (RAIR): In children with Hirschsprung disease, the sphincter does not relax in response to a full rectum. The absence of the RAIR can sometimes cause obstructive symptoms. Most children ‘grow out’ of the problem by the age of five.
• Motility disorders: Motility disorders are characterized by abnormal movements of the gastrointestinal tract.
• Stool-holding behavior: Many children of toilet-training age with Hirschsprung disease engage in holding their stool due to anxiety. The absent RAIR often leads to painful hard stools, which creates a vicious cycle.

Management and Treatment for Children with Symptoms of Bowel Obstruction

Treatment for bowel obstruction symptoms include:

• Surgery: A re-do of the pull-through surgical procedure may be performed to correct any existing anatomical problems or underlying disorders such as abnormal innervation.
• Botulinum toxin injections: In the event that a repeat biopsy comes back normal, the next step would entail injecting botulinum toxin into the anal sphincter, allowing the sphincter to relax which results in improved symptoms. Use of botulinum toxin has largely replaced internal sphincterotomy or myectomy, the traditional operative approach for internal sphincter achalasia.

Hirschsprung associated enterocolitis (HAEC) is an infection or inflammation of the bowel that can occur in children both before and after their surgical procedure. There are several factors that cause HAEC, including:

• An intestinal blockage
• A bacterial or viral infection
• Other anatomical or underlying conditions caused by Hirschsprung disease

Symptoms of HAEC include:

• Bloating
• Severe diarrhea
• Vomiting
• Fever
• Lethargy
• Poor feeding
• Rectal bleeding

Management and Treatment of HAEC

HAEC can range from moderate to severe, and in some cases, potentially be life- threatening. HAEC can come on suddenly and will require immediate treatment and care. Treatment for HAEC will depend on the grade (severity of the condition).

Grade I HAEC:
Treatment for Grade I HAEC can be done on an outpatient basis and may include:

• Hydration with Pedialyte or another electrolyte-rich solution
• Oral metronidazole (an antibiotic used to treat certain bacterial and parasitic infections)
• A regimen of saline enemas to wash the lining of the bowel to help eliminate liquid stool or gas
• Probiotics

Grade II HAEC:
Treatment for Grade II HAEC entails treatment in the hospital with intravenous fluids. Nothing is given by mouth. When there is significant abdominal distention, nasogastric decompression (the insertion of a plastic tube through the nose, past the throat and down into the stomach) may be done to drain the contents of the stomach.

Other treatments include:

• Oral metronidazole
• Other broad-spectrum intravenous antibiotics
• Close monitoring of the child clinically and with the use of advanced imaging to watch for the development of pneumoperitoneum (abnormal air or gas in the abdominal cavity)
• Probiotics

Grade III HAEC:
For more severe Grade III HAEC, all of the above treatments will be given and the child will likely be admitted to the intensive care unit for close observation. If pneumoperitoneum develops, immediate surgical intervention will be required.

As with any treatment and surgery there is always the possibility for symptoms to continue. Although surgical intervention can improve their quality of life, children with Hirschsprung disease may still experience soiling, obstructive symptoms or enterocolitis following a pull-through surgery. However, the prognosis for children with persistent symptoms is very good. By applying an organized and effective therapeutic approach to treatment and care, most children respond very well and many of these problems improve as the child gets older.