The problematic Soave cuff in Hirschsprung disease: manifestations and treatment.

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.