Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Hirschsprung disease is a congenital anomaly of the GI tract that results in a functional bowel obstruction.
In Hirschsprung disease, the ganglion cells don't make it all the way down distally, and the colon ends up not being able to contract.
More than 95% of neonates pass meconium within the first 48 hours of life.
Failure to pass meconium within the first 48 hours of life is typical of Hirschsprung's disease.
Hirschsprung disease has an incidence of about 1 in 5,000 children.
About 10% of children with Hirschsprung disease will have a positive family history.
The RET gene is a predisposing genetic condition associated with Hirschsprung disease.
Up to 10% of children with Hirschsprung's disease will have trisomy 21.
Only 1 to 2% of patients with trisomy 21 have Hirschsprung's disease.
Hirschsprung disease is associated with Wordenberg syndrome and congenital central hyperventilation (Andine's curse).
The three studies that should be considered for evaluating for Hirschsprung's disease include contrast enema, rectal biopsy, and possibly anorectal manometry.
The classic finding on contrast enema is a transition zone in the rectosigmoid, but that transition zone can be located anywhere within the bowel.
The transition zone is from contracted rectum (where nerve cells are absent) to dilated rectum (where normal nerve cells are present).
A rectosigmoid ratio of less than 1.0 is suggestive of Hirschsprung's disease.
In Hirschsprung's disease, the rectum is usually less dilated than the proximal colon, making the rectosigmoid ratio less than 1.
Inability to evacuate contrast is a finding suggestive of Hirschsprung disease.
In total colonic Hirschsprung's disease, a foreshortened or question mark colon can be seen on contrast enema.
Rectal biopsy is the true definitive diagnosis for Hirschsprung disease.
Typical features on rectal biopsy include absence of ganglion cells, presence of hypertrophic nerves, abnormal pattern of colon esterase staining, and an absence of calretinine staining.
To be considered an adequate rectal biopsy, it must be taken from the rectum at least 1 cm above the dentate line and must include both mucosa and submucosal layers.
Suction rectal biopsy technique is typically used for patients less than 6 months of age.
Full thickness rectal biopsy technique should be considered for patients older than 6 months or when a suction biopsy is inadequate.
Suction rectal biopsy is painless and commonly done at the bedside in neonates.
In anorectal manometry for Hirschsprung disease, there is a lack of the recto-anal inhibitory reflex (RAIR).
The recto-anal inhibitory reflex may be absent in other conditions besides Hirschsprung disease, and some children have a false positive test.
Any patient with an absent RAIR must undergo a rectal biopsy for confirmation of the diagnosis.
Children with Hirschsprung disease will need this condition managed for life, but the expectation is that they will live a normal life with close management and care.
NPO babies diagnosed with Hirschsprung disease are typically managed with irrigations, antibiotics if they show evidence of enterocolitis, and NPO or NG tubes if they're distended.
Older children with Hirschsprung disease are not amenable to just doing a primary pull through in many cases because the colon has become dilated.
Older children with Hirschsprung disease will start on an enema program, and some may need diversion more proximally to give the colon time to decompress.
The three goals of surgical management of Hirschsprung disease are: identify the extent of the aganglionic segment, resect that segment, and restore the bowel to its continuity.
The three procedures for Hirschsprung disease (Swensen, Suave, Duamel) all involve a transanal approach of removing the aganglionic colon and pulling down healthy colon and sewing it to the anus.
The Swensen technique is a full thickness dissection and anastomosis.
The Suave procedure is a mucosectomy where you leave a cuff of aganglionic bowel and bring the ganglionated bowel through that cuff of rectum and perform the anastomosis.
The Duamel procedure involves performing a pouch with an anastomosis of aganglionic and ganglionated bowel.
Early postoperative complications include diaper rash and excoriation that can often need to be treated like a burn.
Anastomotic leak is a rare but possible early complication after Hirschsprung surgery.
Hirschsprung's associated enterocolitis is the main early complication everyone needs to be aware of.
Hirschsprung's associated enterocolitis is poorly understood and likely is an inflammatory condition secondary to bacterial overgrowth.
A child with enterocolitis presents with abdominal distention, vomiting, fever, and change in bowel habits.
Enterocolitis must be recognized as potential enterocolitis and treated urgently.
Treatment for enterocolitis includes fluid resuscitation, digital rectal exam, and colonic irrigations.
Depending on the severity of enterocolitis, broad-spectrum antibiotics are sometimes added, and patients are usually started on metronidazole.
About 80% of kids with Hirschsprung's disease are constipated and will need some kind of management.
Assuming the operation has been done well and there are no transition zone or strictures, most patients with Hirschsprung disease are expected to do very well and be in kindergarten socially confident.
The first reports of Hirschsprung disease date back to the 17th century.