The Surgical Treatment Options for Familial Adenomatous Polyposis: A Clinic Experience

INTRODUCTION: Familial adenomatous polyposis (FAP) is treated by abdominal colectomy with ileorectal anastomosis (IRA), restorative proctocolectomy with ileal J-pouch anal anastomosis (IPAA), and total proctocolectomy with ileostomy. This study investigated question is what type of surgery is better.
METHODS: Twenty FAP patients, who underwent surgery in a single hospital between 2004 and 2014, were included. Patients were classified in 3 groups according to the operation procedures. Demographic, surgical, pathological, and outcome data were analyzed from hospital records retrospectively.
RESULTS: Overall 20 patients were included, 15 index patients, and 5 relatives diagnosed by screening. Median age of patients with or without colorectal cancer was 37 and 20 years respectively. Four patients were treated by IPAA, 4 by IRA, and 12 by proctocolectomy. All patients presented with 100 or more colonic polyps. The median follow-up time was 38 months. During the follow-up period, 8 (40.0%) patients developed recurrent or metastatic disease. There were; distant metastasis in 4 patients, rectal cancer in three (who underwent IRA), desmoid tumor in one. Adenoma developed in one rectal remnant, ileal pouch adenoma or adenoma in ileostomy was not seen in any patient. Four patients died 3 of them because of metastatic disease and the other one died because of a huge desmoid that caused renal failure.
DISCUSSION AND CONCLUSION: Abdominal colectomy with IRA was not a good option for classic FAP patients who presented with colorectal cancer preoperatively, but it should be noticed that this study consists of small number of patients.

Ailesel Adenomatöz Polipoziste Cerrahi Seçenekler: Klinik Deneyimimiz

GÝRÝÞ ve AMAÇ: Ailesel adenomatöz polipoziste (AAP) cerrahi tedavi, abdominal kolektomi-ileorektal anastomoz (IRA), restoratif proktokolektomi-ileal J poþ anal anastomoz (IPAA) ve total proktokolektomi- kalýcý ileostomiyi içerir. Bu çalýþma ile hangi tip cerrahi yöntemin en iyi seçenek olduðu irdelendi.
YÖNTEM ve GEREÇLER: Ailesel adenomatöz polipozis tanýsýyla 2004-2014 yýllarý arasýnda ayný merkezde cerrahi giriþim uygulanmýþ 20 hasta çalýþmaya alýndý. Hastalar uygulanan ameliyat türüne göre 3 gruba ayrýldý. Demografik özellikler, ameliyat bulgularý, patoloji sonuçlarý ve klinik seyirlerine iliþkin veriler geriye dönük olarak hastane kayýtlarýndan elde edildi.
BULGULAR: Yirmi hastada AAP tanýsý kolonoskopi ve patoloji raporuna dayanýlarak konuldu. Bunlarýn 15’inde kanser geliþmiþken 5 hastada kolonoskopik taramada kanser henüz geliþmeden taný konuldu. Kanser geliþen ve geliþmeyen hastalarýn ortanca yaþý sýrasýyla 37 ve 20 yaþ. Dört hastaya IPAA, 4 hastaya IRA ve geri kalan 12 hastaya total proktokolektomi kalýcý ileostomi uygulandý. Hastalarýn hepsinde 100 ve üzerinde kolonda polip vardý. Hastalar ortanca 38 aya takip edildi. Bu takip esnasýnda 8 hastada (%40.0) tekrarlayan veya metastatik hastalýk geliþti. Bunlar; 4 hastada uzak metastaz, 3 hastada rektum kanseri (hepsine daha önce IRA yapýlmýþ) ve bir hastada ise desmoid tümör þeklindeydi. Ayrýca IRA yapýlmýþ 1 hastada rektumda adenom geliþti. Ileal poþ adenomu ya da ileostomide adenom geliþimi izlenmedi. Dört hasta hastalýða baðlý kaybedildi. Bunlardan 3’ü metastatik hastalýk geliþimine baðlýyken kalan 1 hastada dev desmoid tümörün neden olduðu böbrek yetmezliði ölüm sebebiydi.
TARTIÞMA ve SONUÇ: Ameliyat öncesi kanser tanýsý konulmuþ klasik AAP hastalarýnda cerrahi yöntem olarak abdominal kolektomi-IRA seçimi iyi bir seçenek olmayabilir. Ancak çalýþmanýn az sayýda hastayý kapsadýðý da göz önünde bulundurulmalýdýr.