| Autor: |
Jeelani SM; Department of Pediatric Surgery, Aga Khan University Hospital, Karachi, Pakistan., Saleem A; Department of Surgery, Aga Khan University, Karachi, Pakistan., Dogar SA; Section of Paediatric Surgery, Department of Surgery, Aga Khan University Hospital, Karachi., Shariff AH; Department of Surgery, Aga Khan University, Karachi, Pakistan., Qazi SH; Paediatric Surgery, Department of surgery Aga Khan University, Karachi, Pakistann. |
| Abstrakt: |
Familial Adenomatous Polyposis accounts for <1% of all the colorectal cancer cases, with progression to colorectal cancer usually at >20 years of age. Endoscopy is essential for the diagnosis with definitive treatment involving prophylactic total colectomy. With current surgical advances, this is routinely being performed with the aid of laparoscopy. Due to resource limitations and non-availability of the screening programs in the developing world, such cases remain under diagnosed. Genetic testing is necessary for prognostication of both the index case and their at-risk family members. Thus, we present a rare case of an eight-year-old female, with an early onset progression to colorectal cancer with Familial Adenomatous Polyposis. We performed a prophylactic laparoscopic total procto-colectomy with ileo-anal anastomosis, which to our knowledge is the first ever-performed procedure in Pakistan. We conclude that progression to colorectal carcinoma in familial adenomatous polyposis can present at an earlier age than that reported in the literature. Laparoscopic total colectomy has similar outcomes than open surgical methods with better cosmetic results. |
