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Hirschsprung's disease is characterized by a lack of enteric nervous system ganglion cells (aganglionosis) in a variable extent of distal bowel. It is the commonest congenital bowel motility disorder and affected neonates usually present with distal intestinal obstruction in the first few days of life. Despite the common underlying pathology of Hirschsprung disease, it has varying presentations. Infants classically present with delayed passage of meconium, feeding intolerance, and bilious emesis. In fact, 90% of children with Hirschsprung will not have passage of stool within the first 24 h of life. Neonates and infants can also present with abdominal distension, failure to thrive, enterocolitis, or bowel perforation.
Hirschsprung's disease is characterized by a variable length of distal colonic aganglionosis. In approximately 80% of cases, it is short-segment, and only involves the rectosigmoid colon. Less commonly, it can extend proximal to the sigmoid colon (15%), include the entire colon (total colonic aganglionosis, 5%), or rarely, the entire intestine (total intestinal aganglionosis). The principles of the operation are to remove the aganglionic colon and connect the normally innervated bowel just above the anus, at a level which prevents further functional obstruction, but at the same time preserves fecal continence.
The surgical treatment of Hirschsprung's disease has evolved from the historical three-stage procedure to a single-stage technique. Since then, multiple series reported its safety, efficacy, and feasibility in the management of HSCR in the neonatal period. Swenson and Bill, Soave, and Duhamel are the most common procedures for Hirschsprung's disease. However, there is a heated debate about which technique gives the best short- and long-term outcomes. There are many surgical approaches to Hirschsprung's disease, including the transabdominal approach (TAB) and transanal endorectal pull-through (TERPT). The TAB includes 4 types: the Swenson, Duhamel, Rehbein, and Soave procedures. Both the Swenson and the Soave procedures have been adapted as transanal approaches.
Transanal access is based on the traditional surgical techniques performed previously in abdominal approach. This type of surgery is used for the treatment of small children. Transanal endorectal pull-through method performed with transanal access is characterized by low invasiveness of surgery and good results of treatment.
The Swenson procedure involves the removal of the entire affected site and end-to-end anastomosis of the normal colonic anal canal. In the Soave procedure, physiological saline is injected into the rectum after cutting through the rectal muscle layer in a circular manner, while keeping the mucosa intact to the dentate line level.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Swenson procedure | Active Comparator | full-thickness dissection is started 1 cm above the dentate line |
|
| Soave procedure | Active Comparator | circumferential incision is made 1cm above the dentate line in the rectal mucosa. Using blunt dissection, a submucosal plane is developed |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Swenson operation includes resection of the aganglionic segment and anastomosis with full thickness layer above the dentate. | Procedure | The Swenson operation: The patient is placed in either the prone or lithotomy position and full-thickness dissection is started 1 cm above the dentate line until ganglionated bowel is encountered. Fine silk suturing is performed circumferentially at the level of that point which would be used for traction for the distal end. Another circumferential suture was performed parallel 0.5 cm distances above the original one and used for traction for the proximal intestines. The full-thickness rectal wall is truncated between the above two circumferential sutures with cautery, avoiding damaging adjacent tissues when the abdominal cavity is open. The full thickness of rectum and sigmoid colon is mobilized out though the anus and the mesenteric vessels are carefully dissected and ligatured. The colon is divided until a few centimeters above the most proximal normal site. The distal rectum is pulled eversion and is dissected anteriorly 2.5-3.5 cm above the dentate line. |
| Measure | Description | Time Frame |
|---|---|---|
| Operative time. | Time taken to perform the surgery completely. | From enrollment to the 6 months postoperatively. |
| Measure | Description | Time Frame |
|---|---|---|
| Frequency of defecation | Number of fecal motions every day | From the time of enrollment to the 6 months after surgery |
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Inclusion Criteria:
Exclusion Criteria:
• Patients with severe systemic disease making anesthesia or surgery prohibitively risky (American society of Anesthesiologists (ASA) class IV.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Kafrelsheikh University Hospitals | Kafr ash Shaykh | Kafr el-Sheikh Governorate | 33516 | Egypt |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| Background | Dave, A., Allukian, M., Dickie, B. (2022). Hirschsprung Disease. In: Mattei, P. (eds) Fundamentals of Pediatric Surgery. Springer, Cham. https://doi.org/10.1007/978-3-031-07524-7_72 |
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The information included data of complicated cases.
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| Soave procedure: includes resection of the aganglionic segment and anastomosis with submucosal thickness layer above the dentate. | Procedure | For the operation, the patient is placed in a prone position with the pelvis elevated. As a first step to the transanal mucosectomy of the rectum, the anal canal is exposed, and a circumferential incision is made 1cm above the dentate line in the rectal mucosa. Using blunt dissection, a submucosal plane is developed placing multiple 5-0 silk traction sutures in the mucosa to facilitate its separation from the muscular wall. The submucosal plane is extended 6 cm. The next step is to prepare the muscular sleeve through which the normoganglionic colon would be pulled. At the same site at which mucosectomy is finished, a complete incision on the rectal muscle is made to reach into the perirectal tissue. To liberate the muscular sleeve, perirectal tissue is dissected, and smooth muscle fibers of the rectum are divided circumferentially. Through this procedure, the muscular sleeve could be liberated and returned to its original position. |
|
| ID | Term |
|---|---|
| D003248 | Constipation |
| ID | Term |
|---|---|
| D012817 | Signs and Symptoms, Digestive |
| D012816 | Signs and Symptoms |
| D013568 | Pathological Conditions, Signs and Symptoms |
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| ID | Term |
|---|---|
| D000714 | Anastomosis, Surgical |
| ID | Term |
|---|---|
| D013514 | Surgical Procedures, Operative |
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