Hirschsprung Disease
Conditions
Keywords
Swenson Technique, Single-Port Laparoscopy, Pediatric surgery, Postoperative outcomes, Hirschsprung disease
Brief summary
Hirschsprung disease (HD) is a rare congenital disorder of the enteric nervous system, affecting approximately 1 in 5,000 live births. It is characterized by the absence of ganglion cells in the distal colon, leading to functional intestinal obstruction due to impaired peristalsis. Surgical treatment consists of resection of the aganglionic segment-most commonly rectosigmoid-followed by a colo-rectal, colo-anal, or ileo-anal anastomosis. Among colo-anal pull-through procedures, the Swenson technique was historically performed through an exclusively transanal approach, which carries a risk of sphincter injury correlated with operative duration. More recently, combined laparoscopic and transanal approaches have been developed to reduce this risk, although they may be associated with higher overall complication and reoperation rates. The Swenson procedure can be performed using a single-port laparoscopic approach, a technique that is sparsely described in the literature and rarely practiced in France. Single-port laparoscopy represents an emerging surgical technique that, despite increased technical complexity for surgeons, may further enhance postoperative recovery and cosmetic outcomes compared to conventional multiport laparoscopy. The objective of this study is to describe the outcomes of single-port laparoscopic Swenson pull-through in children with Hirschsprung disease and to compare them with outcomes obtained using more conventional approaches, namely exclusive transanal surgery or combined multiport laparoscopic and transanal approaches.
Interventions
TA-Swenson consist of total trananal endorectal pullthrough after a short submucosal dissection of the last part of the rectum. Laparoscopy-assisted Swenson pull-through consists of laparoscopic mobilization of the aganglionic colon with intraoperative level confirmation, followed by a transanal resection of the aganglionic segment and a primary coloanal anastomosis
Sponsors
Study design
Eligibility
Inclusion criteria
* Children under 16 years of age * Diagnosis of Hirschsprung disease confirmed by initial rectal biopsy and postoperative pathological examination * Primary Swenson coloanal pull-through performed at Angers or Caen University Hospital between January 1, 2010 and June 30, 2025
Exclusion criteria
* Parental or legal guardian opposition to the use of medical data for research purposes * Hirschsprung disease treated with other surgical techniques (Duhamel, Soave, De La Torre, TERPT) * Patients operated on in another hospital * Patients who underwent rectosigmoid resection for causes of constipation other than Hirschsprung disease
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| To compare the short-term effectiveness of different surgical laparoscopic approaches used for the Swenson technique. | From surgery to 12 months postoperatively | Short-term effectiveness, defined as spontaneous bowel transit without obstructive-spectrum events during the first postoperative year, including: * Constipation * Delayed resumption of oral feeding * Sphincter hypertonicity * Hirschsprung-associated enterocolitis |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| To compare intraoperativensurgical efficiency according to surgical approach | During surgery (day 0) | Surgical efficiency, defined as operative time for coloanal anastomosis completion without conversion, including the approach for intraoperative frozen-section biopsy. |
| To compare immediate postoperative recovery | At hospital discharge (average 5 days) | Recovery, defined as hospital discharge in good health, which includes: * Return of bowel function * Resumption of oral feeding * Absence of early postoperative complications, particularly infectious complications (surgical site infection, intra-abdominal infection, sepsis) |
| Short-term complications | From hospital discharge to 30 days after surgery | All early postoperative complications graded according to the Clavien-Madadi classification |
| Long-term complications | From 31 days after surgery up to the last clinical follow-up visit (mean follow-up: 5.5 years) | All late postoperative complications graded according to the Clavien-Madadi classification |
| Long-term continence outcomes | Assessed after toilet training age (≥3-4 years old) and up to the last clinical follow-up visit (mean follow-up: 5.5 years) | Assessed using the Krickenbeck score, which includes: * Bowel movements perception * Soining (graded 0 - absence to 3 - constant/social prolem) * Constipation (graded from 0 - absence to 3 - resistant to diet and laxatives) |
Contacts
University Hospital of Angers