Abstract
BACKGROUND: The present case of a colorectal adenocarcinoma at the anastomotic site of a colorectal anastomosis after childhood surgery for Hirschsprung disease is a rare report of such pathology. Possibly, the altered anatomy after Duhamel procedure may pose a risk for carcinogenesis in those patients. The previously surgically opened plane of the mesorectal fascia, which is usually dissected during total mesorectal excision in rectal cancer, and the impossibility to differentiate from colon or rectal cancer in this case influence oncologic treatment strategies. Furthermore, the case highlights functional aspects of lower anterior resection syndrome before and after rectal cancer surgery with a coloanal anastomosis and how this influences quality of life. CASE PRESENTATION: We report a rare case of a 54-year-old male with a history of Hirschsprung disease, treated with a Duhamel procedure during childhood, who developed colorectal cancer at the site of the colorectal anastomosis. The Duhamel procedure, a common surgical technique in Hirschsprung disease, involves creating a deep colorectal anastomosis with a retained rectal stump. The tumor, a moderately differentiated adenocarcinoma, was treated with total mesorectal excision and intersphincteric resection with a hand-sewn coloanal anastomosis and a loop ileostomy. Postoperative complications included paralytic ileus and urinary retention, but the ileostomy was successfully reversed. Despite increased LARS (lower anterior resection syndrome) scores postoperatively, the patient adapted well, reporting minimal impact on quality of life. In the short term of 1.5 years post-surgery, the patient is disease-free. CONCLUSION: This case highlights the need for awareness of potential colorectal cancer development in patients with a history of Hirschsprung disease and emphasizes the importance of individualized management and close surveillance strategies.