Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

short-segment/long-segment disease: without enterocolitis

1st line – bowel irrigation

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ACUTE
|
short-segment/long-segment disease: without enterocolitis
1st line – 

bowel irrigation

The initial treatment of both short-segment (rectosigmoid) disease and long-segment disease is similar, as the extent of disease is not known until confirmation of pathology.

All patients receive irrigations in the newborn period to manage abdominal distension prior to proceeding with surgery.[11] Guidance suggests 1 to 3 irrigations per day.[27] Irrigation may not be effective in patients with long-segment disease.[27]​​

A large tube (20-24 Fr) is introduced through the rectum, and small amounts of saline solution (10-20 mL) are instilled through the lumen of the tube in order to clear the lumen of the tube. The liquid rectal and colonic content is expected to drain through the lumen of the tube. The tube is then rotated in different directions and moved back and forth. The operator continues to instill small amounts of saline solution, allowing the evacuation of gas and liquid stool through the tube.

Irrigations must be differentiated from enemas. Enemas involve instilling a large volume of fluid containing different irritant ingredients into the rectum and colon in order to elicit a bowel movement. Enemas are not recommended in patients with Hirschsprung disease.

Plus – definitive surgery

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ACUTE
|
short-segment/long-segment disease: without enterocolitis
Plus – 

definitive surgery

Treatment recommended for ALL patients in selected patient group

Definitive surgery is usually performed within the first week of life. The procedure can also be delayed up to 2-3 months while the patient is primarily managed with irrigations.[1][67][68]​​

Three surgical techniques exist for definitive surgical management. All have in common the removal of the distal aganglionic segment with pull-through of the proximal normal ganglionic bowel.

Guidelines do not recommend any single pull-through technique over others for short-segment or long-segment disease; all three major surgical approaches have potential advantages and complications.[1]​​[4][27]​​

The Swenson operation is a full-thickness excision of the rectum and remaining aganglionic bowel.[63][71][72][73]​ The original description involved entering the abdomen through a Pfannenstiel, hockey-stick incision, followed by a full-thickness dissection of the aganglionic sigmoid and rectum.[83] This whole procedure can now be done through a transanal approach.[63][73][75][76]​ The abdominal incision can be avoided in many cases or replaced with laparoscopy. The occurrence of fecal and urinary incontinence, as well as erectile dysfunction, which was felt to be due to nerve injury provoked during aggressive rectal dissection, prompted the development of the Yancey-Soave and Duhamel procedures in an attempt to avoid those complications.

The Yancey-Soave procedure includes the resection of the mucosal layer of the distal bowel (endorectal resection) leaving intact a seromuscular cuff and pulling through the normal ganglionic colon inside the cuff.[77][78]​ Theoretically, this minimizes the risk of potential injury to important neighboring pelvic structures during rectal dissection.[69][79]

The Duhamel procedure involves normal (i.e., ganglionic) intestine (usually above the most dilated portion) being pulled through a presacral space that has been created by blunt dissection and connecting this lumen to the original rectum left in its anterior position.[80]​ It avoids the extensive pelvic dissection required in the Swenson operation by preserving the distal aganglionic rectum, dividing the bowel at the peritoneal reflection as distally as possible. The rectal stump is then closed and the normal ganglionic colon is pulled through a presacral path and anastomosed to the posterior wall of the rectum, above the pectinate line.[70] A wide window is created with a stapler between the posterior rectal wall and the anterior wall of the normal ganglionic bowel. The fact that the anal canal is not disturbed likely contributes to the very low incidence of fecal incontinence; however, the Duhamel “pouch” (aganglionic rectum) often becomes dilated which leads to severe constipation.

Guidelines recommend one dose of preoperative intravenous broad-spectrum antibiotics; consult local protocols.[27]

short-segment/long-segment disease: with enterocolitis

1st line – bowel irrigation + intravenous fluids + antibiotics

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ACUTE
|
short-segment/long-segment disease: with enterocolitis
1st line – 

bowel irrigation + intravenous fluids + antibiotics

The initial treatment of both short-segment (rectosigmoid) disease and long-segment disease is similar, as the extent of disease is not known until confirmation of pathology.

Hirschsprung-associated enterocolitis (HAEC) may occur with prolonged abdominal distension and fecal stasis. The stasis leads to bacterial overgrowth, which leads to bacterial translocation and secretory diarrhea. HAEC can result in hypovolemia, endotoxin-related shock, and sepsis, making prompt treatment crucial as it is the leading cause of death in Hirschsprung disease.[11][12][13]​ HAEC must be suspected clinically. Bowel irrigation with saline solution is an extremely valuable procedure for the emergency management of HAEC.[11]​ By decompressing the bowel, the procedure may dramatically improve a very sick infant.

All patients receive irrigations in the newborn period to manage abdominal distension prior to proceeding with surgery.[11]​ Guidance suggests 1 to 3 irrigations per day.[27]​ Irrigation may not be effective in patients with long-segment disease.[27]

A large tube (20-24 Fr) is introduced through the rectum, and small amounts of saline solution (10 to 20 mL) are instilled through the lumen of the tube in order to clear the lumen of the tube. The liquid rectal and colonic content is expected to drain through the lumen of the tube. The tube is then rotated in different directions and moved back and forth. The operator continues to instill small amounts of saline solution, allowing the evacuation of gas and liquid stool through the tube.

Irrigations must be differentiated from enemas. Enemas involve instilling a large volume of fluid containing different irritant ingredients into the rectum and colon in order to elicit a bowel movement. Enemas are not recommended in patients with Hirschsprung disease.

Initially patients should be kept nothing by mouth (NPO) until they begin to improve. Patients should receive intravenous fluids and antibiotics. Metronidazole is usually given. In older children, oral antibiotics can be given when they begin to improve and when they are no longer NPO.

Primary options

metronidazole: children: 30 mg/kg/day orally/intravenously given in divided doses every 6 hours, maximum 4000 mg/day; neonates require lower doses, consult specialist for further guidance on dose

Consider – decompression by colostomy or ileostomy

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ACUTE
|
short-segment/long-segment disease: with enterocolitis
Consider – 

decompression by colostomy or ileostomy

Treatment recommended for SOME patients in selected patient group

Colostomy or ileostomy is necessary if a child is sick with intractable HAEC, bowel perforation, or abdominal distension unresponsive to irrigations.[27]

An ileostomy or right transverse colostomy is a safe and effective method for decompressing the colon. This is a particularly useful option in emergency situations, for instance if there are no pediatric pathologists available to define the exact level of the transition zone (where circumferential ganglion cells are identified). By using this location for the stoma, the risk of the error of opening the colostomy in an aganglionic area is much reduced. A particular advantage is that the left side of the colon remains untouched, allowing for a future resection of the aganglionic segment and pull-through of the normal ganglionic colon. The disadvantage is that it commits the surgeon to a 3-stage procedure.

Alternatively, a leveling colostomy can be performed. This is a colostomy placed at the start of the ganglionic portion of the colon, and where there are also no hypertrophic nerves. This obligates the surgeon to pull the colostomy down at the time of the definitive repair, depriving the patient of the protection of a proximal diversion. The advantage of this approach is that the child will require only a 2-stage procedure.

Plus – definitive surgery

Back
ACUTE
|
short-segment/long-segment disease: with enterocolitis
Plus – 

definitive surgery

Treatment recommended for ALL patients in selected patient group

Treatment of enterocolitis with irrigations, hydration, and antibiotics usually takes days or weeks - about a week in hospital and several weeks having the family perform irrigations at home before proceeding to definitive surgery.

Three surgical techniques exist for definitive surgical management. All have in common the removal of the distal aganglionic segment with pull-through of the proximal normal ganglionic bowel.

Guidelines do not recommend any single pull-through technique over others for short-segment or long-segment disease; all three major surgical approaches have potential advantages and complications.[1][4][27]

The Swenson operation is a full-thickness excision of the rectum and remaining aganglionic bowel.[63][71][72][73]​ The original description involved entering the abdomen through a Pfannenstiel, hockey-stick incision, followed by full-thickness dissection of the aganglionic sigmoid and rectum.[83] This whole procedure can now be done through a transanal approach.[63][73][75][76]​ The abdominal incision can be avoided in many cases or replaced with laparoscopy. The occurrence of fecal and urinary incontinence, as well as erectile dysfunction, which was felt to be due to nerve injury provoked by aggressive rectal dissection, prompted the development of the Yancey-Soave and Duhamel procedures in an attempt to avoid those complications.

The Yancey-Soave procedure includes the resection of the mucosal layer of the distal bowel (endorectal resection) leaving intact a seromuscular cuff and pulling through the normal ganglionic colon inside the cuff.[77][78] Theoretically, this minimizes the risk of potential injury to important neighboring pelvic structures during rectal dissection.[69][79]​​

The Duhamel procedure involves normal (i.e., ganglionic) intestine (usually above the most dilated portion) being pulled through a presacral space that has been created by blunt dissection and connecting this lumen to the original rectum left in its anterior position.[80]​ It avoids the extensive pelvic dissection required in the Swenson operation by preserving the distal aganglionic rectum, dividing the bowel at the peritoneal reflection as distally as possible. The rectal stump is then closed and the normal ganglionic colon is pulled through a presacral path and anastomosed to the posterior wall of the rectum, above the pectinate line.[70] A wide window is created with a stapler between the posterior rectal wall and the anterior wall of the normal ganglionic bowel. The fact that the anal canal is not disturbed likely contributes to the very low incidence of fecal incontinence; however, the Duhamel “pouch” (aganglionic rectum) often becomes dilated which leads to severe constipation.

If bowel diversion had been required, then once the child is well, a reconstruction can be planned. If the colostomy was a leveling colostomy, namely that it was placed proximal to the transition zone, then that colostomy can be pulled through and the distal aganglionic bowel resected. If the colostomy was placed more proximally, or if there was an ileostomy created, a pull-through can be performed using normal ganglionic colon proximal to the transition zone, and then the stoma can be closed at a third stage.

Guidelines recommend one dose of preoperative intravenous broad-spectrum antibiotics; consult local protocols.[27]

total colonic aganglionosis

1st line – ileostomy

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ACUTE
|
total colonic aganglionosis
1st line – 

ileostomy

Irrigations often do not work for patients with total colonic aganglionosis because it is difficult to reach the dilated small bowel. Patients will require colonic mapping biopsies and an ileostomy.[1][4]​​[11][59]​​

Plus – definitive surgery

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ACUTE
|
total colonic aganglionosis
Plus – 

definitive surgery

Treatment recommended for ALL patients in selected patient group

Definitive surgery (total or subtotal colectomy with ileoanal or coloanal anastomosis) is performed when stoma output is of a thicker consistency, which typically occurs at around one year old when the child has fully transitioned to solid foods.[1]

A 2024 expert consensus on the surgical management of TCA did not favor any single pull-through technique over others, and instead recommends that the technique should be chosen based on the experience of the operating surgeon.[59] All three major surgical approaches have potential advantages and complications.[1] The most commonly performed surgical procedures used to treat TCA include J pouch with ileoanal anastomosis (JIAA), straight ileoanal anastomosis (SIAA) and the Duhamel technique.[82]​​​

Guidelines recommend one dose of preoperative intravenous broad-spectrum antibiotics; consult local protocols.[27]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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