[Treatment of Familial Adenomatous Polyposis and family screening].
BACKGROUND
To reduce the mortality associated to Familial Adenomatous Polyposis (FAP), screening of close relatives of patients with the disease is crucial.


OBJECTIVE
To analyze the results of the surgical treatment of patients with FAP, and to evaluate the family screening.


METHODS
Clinical records of patients operated in our institution since 1977, were reviewed analyzing surgical and pathological results, and follow up. In their family members, we evaluated and analyzed the performance of screening tests, former surgeries, history of disease-related cancer and mortality, all due to FAP.


RESULTS
Between January 1977 and August 2002, 15 patients were operated on. Of these, only 33% consulted on the setting of a familial screening. A proctocolectomy and terminal ileostomy was performed in 27% of patients; 20% had a proctocolectomy and ileal pouch, and 53% underwent a total colectomy with ileo-rectal anastomosis. Morbidity and mortality were 7% and 0%, respectively. Twenty percent had a colorectal cancer. During a median of 68 months follow-up, the disease-related survival was 92%; no cancer of the rectal stump was detected. Of the 122 family members identified, only 33% with clear indication of screening underwent a colonoscopy. Twenty-nine percent had a confirmed FAP and were operated: in 61% of them a colorectal cancer was found, and 91% of these died.


CONCLUSIONS
The results of the surgical treatment of FAP are satisfactory. Nevertheless, family screening should be improved to reduce the high rates of mortality revealed in the study of other family members.
Soto
Gonzalo
G
Departamento de Cirugía Digestiva, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago.
López-Köstner
Francisco
F
Zárate
Alejandro
A
Vuletin
Fernando
F
Rahmer
Alejandro
A
León
Francisca
F
Zúñiga
Alvaro
A
D011125:Adenomatous Polyposis Coli / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D017491:Genes, APC; D005820:Genetic Testing; D006801:Humans; D008297:Male; D008875:Middle Aged; D009154:Mutation; D010375:Pedigree; D016737:Proctocolectomy, Restorative; D011446:Prospective Studies; D016896:Treatment Outcome