Poliposis adenomatosa familiar: Presentación de un caso clínico

Susana Kohen; Nelia Hernández; Akiko Ono

Autores/as

Susana Kohen Universidad de la República, Facultad de Medicina, Hospital de Clínicas, Clínica de Nutrición y Digestivo Prof. Dr. Elbio Zeballos, Ex asistente
Nelia Hernández Universidad de la República, Facultad de Medicina, Hospital de Clínicas, Clínica de Nutrición y Digestivo Prof. Dr. Elbio Zeballos, Asistente
Akiko Ono Médico Gastroenteróloga

Palabras clave:

PÓLIPOS ADENOMATOSOS, PÓLIPOS DEL COLON, POLIPOSIS COLI ADENOMATOSA

Resumen

La poliposis adenomatosa familiar (PAF) es una enfermedad autosómica dominante caracterizada por la presencia de más de cien pólipos a nivel colorrectal.
Se presenta el caso clínico de una paciente colectomizada por PAF que en la evolución desarrolla complicaciones extracolónicas de la enfermedad (adenomas duodenales), cuyo control y tratamiento es objeto de discusión.

Citas

1) Sarre RG, Frost AG, Jagelman DG, Petras RE, Sivak MV, Mc Gannon E. Gastric and duodenal polyps in familial adenomatous polyposis:a prospective study of the nature and prevalence of upper gastrointestinal polyps. Gut 1987; 28:306-14.
2) Buchi K, Becker JM, Burt R. Duodenal polyposis and malignancy in a case of Familial Polyposis Coli. Am J Gastroenterol 1988; 83(9):985-7.
3) Church JM, McGannon E, Hull-Boiner S, Sivak MV, Van Stolk R, Jagelman DG, et al. Gastroduodenal polyps in patients with familial adenomatous polyposis. Dis Colon Rectum 1992; 35:1170-3.
4) Spigelman AD, Williams CB, Talbot IC, Domizio P, Phillips RKS. Upper gastrointestinal cancer in patients with Familial Adenomatous Polyposis. Lancet 1989; 30(2):783-5.
5) Büllow C, Vasen H, Järvinen H, Björk J, Bisgaard ML, Büllow S. Ileorectal anastomosis is appropiate for a subset of patients with familial adenomatous polyposis. Gastroenterology 2000; 119:1454-60.
6) Jagelman DJ, DeCosse JJ, Bussey HJR. Upper gastrointestinal cancer in patients with Familial Adenomatous Polyposis. Lancet 1988; 21(1):149-51.
7) Offerhaus GJ, Giardello FM, Krush AJ, Booker SV, Tersmette AC, Kelley NC, et al. The risk of upper gastrointestinal cancer in familial adenomatous polyposis. Gastroenterology 1992; 102:1980-2.
8) Nugent KP, Spigelman AD, Nicholls RJ, Talbot IC, Neale K, Phillips RK. Pouch adenomas in patients with familial adenomatous polyposis. Br J Surg 1993; 80:1620.
9) Iida M, Yao T, Ohsato K, Watanabe H. Diagnostic value intraoperaterative fiberoscopy for small-intestinal polyps in familial adenomatous coli. Endoscopy 1980; 12:161-5.
10) Sugihara K, Muto T, Kamiya J, Konishi F, Sawada T, Morioka Y. Gardner's syndrome associated with periampullary carcinoma, duodenal and gastric adenomatosis. Dis Colon Rectum 1982; 25:766.
11) Alarcon F, Burke C, Church J, van Stolk R. Familial adenomatous polyposis. Efficacy of endoscopic and surgical treatment for advanced duodenal adenomas. Dis Colon Rectum 1999; 42:1533-6.
12) Vasen H, Bullow S, Myrhoj T, Mathus-Vliegen L, Griffioen G, Buskens E, et al. Decision analysis in the management of duodenal adenomatosis in familial adenomatous polyposis. Gut 1997; 40:716-9.
13) Buecher B, Lehur P. La polypose duodénale dans la polypose adénomateuse familiale:quels risques, quelle prise en charge? Gastroenterol Clin Biol 1997; 21(12):967-77.
14) Cruz-Correa M, Hylind L, Romans K, Booker S, Giardello F. Long-term treatment with sulindac in familial adenomatous polyposis:a prospective cohort study. Gastroenterology 2002; 122:641-5.
15) Nugent KP, Farmer KCR, Spigelman AD, Williams CB, Phillips RKS. Randomized controlled trial of the effect of sulindac on duodenal and rectal polyposis and cell proliferation in patients with familial adenomatous polyposes. Br J Surg 1993; 80:1618-9.
16) Iida M, Itoh H, Masui T, Mibu R, Iwashita A, Fujishima M. Ileal adenomas in poscolectomy patients with Familial Adenomatous Coli/Gardner syndrome. Incidence and endoscopic appearance. Dis Colon Rectum 1989; 32:1034-8.
17) Bertoni G, Sassatelli R, Nigirsoli E, Tansini P, Roncucci L, Ponz de Leon M, et al. First observation of microadenomas in the ileal mucosa of patients with familial adenomatous polyposis and colectomies. Gastroenterology 1995; 109:374-80.
18) Thompson-Fawcett MW, Marcus VA, Redston M, Cohen Z, McLeod RS. Adenomatous polyps develop commonly in the ileal pouch of patients with Familial Adenomatous Polyposis. Dis Colon Rectum 2001; 44:374-53.
19) Wu JS, McGannon EA, Church JM. Incidence of neoplastic polyps in the ileal pouch of patients with familial adenomatous polyposis after restorative proctocolectomy. Dis Colon Rectum 1998; 41:552-7.
20) Parc YR, Olschwang S, Desaint B, Schmitt G, Parc RG, Tiret E. Familial Adenomatous Polyposis:prevalence of adenomas in the ileal pouch after restorative proctocolectomy. Ann Surg 2001; 233(3):360-4.
21) Van Duijvendijk P, Vasen HF, Bertario L, Bulow S, Kuijpers JH, Schouten WR, et al. Cumulative risk of developing polyps or malignancy at the ileal pouch-anal anastomosis in patients with familial adenomatous polyposis. J Gastrointest Surg 1999; 3:325-30.