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The problematic Soave cuff in Hirschsprung disease: manifestations and treatment
Belinda H Dickie1, Keith M Webb1, Balgopal Eradi1
1Colorectal Center for Children Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue ML 2023, Cincinnati, OH 45229, USA.
Insights
Resecting the Soave cuff after Hirschsprung disease surgery can resolve functional bowel obstruction. This procedure significantly reduces enterocolitis and improves bowel emptying in affected children.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Colorectal Surgery
Background:
- Hirschsprung disease (HD) surgery, particularly the Soave pull-through procedure, can lead to persistent obstructive symptoms in some children.
- These symptoms may stem from a functional obstruction caused by the Soave cuff, a remnant of the surgical procedure.
Purpose of the Study:
- To investigate the hypothesis that the Soave cuff can cause functional obstruction following Hirschsprung disease surgery.
- To evaluate the efficacy of Soave cuff resection in improving bowel emptying and alleviating obstructive symptoms.
Main Methods:
- A retrospective review of patients who underwent reoperation for obstructive symptoms after a Soave pull-through for HD between 2008 and 2012.
- Analysis focused on patients with an obstructing Soave cuff, excluding those with other causes of obstruction like strictures or residual aganglionic segments.
Main Results:
- Seventeen patients had a Soave cuff as the sole identified cause of obstruction; common pre-operative symptoms included enterocolitis and constipation.
- Post-operatively, cuff resection reduced enterocolitis episodes to zero and halved the need for irrigations for distension.
- Significant improvements in bowel emptying were observed, with many patients achieving voluntary bowel movements or becoming enema-independent.
Conclusions:
- A problematic Soave cuff can cause functional obstruction, leading to recurrent enterocolitis, constipation, or failure to thrive in HD patients.
- Physical examination and contrast enemas are useful diagnostic tools for identifying cuff-related obstruction.
- Surgical reoperation involving Soave cuff resection offers a significant improvement in bowel function for affected children.
Purpose:
Following a Soave pull-through for Hirschsprung disease (HD), some children struggle with obstructive symptoms. We hypothesized that these symptoms could result from a functional obstruction of the pull through caused by the Soave cuff, and that cuff resection might improve bowel emptying.
Methods:
We reviewed patients referred to our center from 2008 to 2012 with obstructive problems following a Soave pull-through for HD (CCHMC IRB # 2011-2019). Only patients with an obstructing Soave cuff were analyzed. Patients with other reasons for obstruction (anastomotic stricture, transition zone, aganglionic segment) were excluded.
Results:
Thirty-six patients underwent reoperation at our center for obstructive symptoms after an initial Soave pull-through. Seventeen of these patients had a Soave cuff only as the potential source of obstruction. Pre-operative symptoms included enterocolitis (10), constipation (6), and failure to thrive (1). Nine patients (53%) required irrigations to manage distension or enterocolitis pre-operatively. 14/17 patients (82%) had a palpable cuff on rectal exam. Eight patients (47%) had radiographic evidence of a cuff demonstrated by distal narrowing (4) or a prominent presacral space (4). Four children (23%) underwent excision of the cuff only. Thirteen (76%) had removal of the cuff and proximally dilated colon [(average length 7.2cm) (12 performed transanally, and five needed laparotomy as well.)] Post-operatively, episodes of enterocolitis were reduced to zero, and need for irrigation to treat distension was reduced by 50%. Nine patients have voluntary bowel movements, and five are clean on enemas. 3/6 patients with pre-operative constipation or impaction now empty without enemas. (Follow up 1-17months, mean 7months.)
Conclusions:
Recurrent enterocolitis, constipation, or failure to thrive can indicate a functional obstruction due to a Soave cuff when no other pathologic cause exists. Physical exam or contrast enema can identify a problematic cuff. Reoperation with cuff resection can dramatically improve bowel emptying.
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