VACTERL association in anorectal malformation: effect on the outcome

Anorectal malformations (ARM) can commonly occur in association with other congenital anomalies or as part of the combined anomaly. The present study aims to assess the outcome of patients with ARM and patients with ARM in VACTERL association. A 12-year retrospective analysis of all patients treated at a single tertiary children's institution with a diagnosis of ARM. We identified and compared patients with ARM to those with ARM in VACTERL association (3 or more anomalies). Data were collected for both groups to assess type of ARM, urinary incontinence (UI), constipation, soiling, dietary/laxative treatment, bowel management (BM) and surgical complications. Type of lesion and clinical outcomes were classified according to Krickenbeck International classification. Patients lost to follow-up, dead or not yet toilet-trained (or < 4 years old) were excluded. One hundred ninety-eight patients were identified, 174 enrolled in the study. Lesions were classified for each study group (VACTERL- vs VACTERL+) as perineal fistulas (36.4 vs 9.7 %, p = 0.0028), rectourethral fistulas (prostatic and bulbar) (23.1 vs 38.7 %, p = ns), rectovesical fistulas (3.5 vs 9.7 %, p = ns), rectovestibular fistulas (19.6 vs 22.6 %, p = ns), cloacal malformations (4.9 vs 9.7 %, p = ns), no fistula (4.9 vs 3.2 %, p=), others (7.7 vs 6.4 %, p = ns). The frequency of both dietary/laxative treatment and BM, as well as surgical complications were significantly higher in patients with VACTERL. The coexistence of VACTERL anomalies negatively affects not only the surgical outcome but also the bowel functioning. Therefore, a dedicated follow-up is strongly recommended. Further studies are needed to assess if this has an impact on the quality of life of these patients.