Transanal-only Swenson-like pull-through for late diagnosed Hirschsprung disease

Abstract

Hirschsprung disease (HD) is an obstructive colonic process usually diagnosed in the neonatal period. A small subset of cases are diagnosed late, present with severe constipation without enterocolitis and have low rectosigmoid disease. A transanal-only pull-through is a well-described approach but in the newborn period risks a situation whereby the transition zone is higher than the sigmoid. We present our experience with the unique patient population of older HD patients in whom the transition zone was reliably reachable via a single-stage transanal approach, performed in prone position. Patients between 2 and 6 years of age with a rectal or sigmoid transition zone and minimal proximal colonic dilation can undergo a primary transanal pull-through surgical approach.

Adopting the Swenson-like technique for patients with Hirschsprung disease in Vietnam

“…The anastomotic leak and perianal abscesses occurred in patients with delayed diagnosis in children over the age of 2 years with a significant size mismatch noted prior to the anastomosis. In pediatric HD patients diagnosed after the age of 2 years, Rentea et al [ 17 ] suggest that single-stage transanal-only pull-through should only be performed in pediatric patients with a low transition, specifically from below the sigmoid colon and minimally dilated colonic diameter. In our study, 9 (6.5%) cases were diagnosed after 2 years of age, however, the colon in most cases was dilated (Fig.…”

“…The rate of enterocolitis after surgery in pediatric patients outside the neonatal age in our study was 17.9%, which is comparable to current literature [ 10 , 17 , 19 ]. In the cohort of patients with enterocolitis, there were 4 (2.8%) anastomotic strictures, 1 (0.7%) Trisomy 21, and 4 (2.8%) other genetic abnormalities.…”

“…Levitt et al popularized the "Swenson-like" technique in transanal pull-through by demonstrating its safety and feasibility with very low complication rates [ 10 ]. As a result, many pediatric surgeons gradually became more empowered to apply this surgical technique [ 17 , 18 ]. With promising results internationally, we suspected that we could also adopt this technique in Vietnam successfully, with the anticipation that our postoperative outcomes would be improved from previous techniques which were not standardized across our institution.…”

“…When first adopting the transanal “Swenson-like” technique at our institution, we were initially concerned about the risk of intraoperative injury to adjacent structures such as the urethra or vagina. However, we carefully followed the instructions of the technical documentation [ 10 , 20 ], and similar to published reports [ 10 , 17 , 18 ], we had no cases of urethral or vaginal injury. We found that the circumferential sutures at the rectum not only provided uniform traction on the rectum but also aided in making the first rectal mucosal incision.…”

Image of the Month: Decision-Making in Surgery for Late Onset Hirschsprung Disease

“…In deciding on a surgical approach for patients with HD, the age of the patient should be factored into the decision-making, in addition to location of transition zone, extent of colonic dilation, as well the patient's nutritional status. 2,3 In the case presented, the contrast enema demonstrates a low transition zone at the rectosigmoid junction with dilation of the sigmoid colon extending to the descending colon. His history also suggests malnutrition and failure to thrive, not uncommon in HD, but much rarer in functional constipation and colonic motility disorders.…”