Extracolonic Manifestations of Familial Adenomatous Polyposis After Proctocolectomy

Tulchinsky, Hagit; Keidar, Andrei; Strul, Hana; Goldman, Gideon; Klausner, Joseph M.; Rabau, Micha

doi:10.1001/archsurg.140.2.159

Cited by 34 publications

(27 citation statements)

References 29 publications

(29 reference statements)

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“…There are reports of adenomas arising in the anal canal after either mucosectomy or double-stapled anastomosis [52,66,[207][208][209]. The incidence of dysplastic polyps in the residual ATZ after double-stapled IPAA has been estimated to be up to 28-31% compared with 10-14% after endoanal mucosectomy, which is nearly less than half [52,66].…”

Section: Risk Of Neoplasia In the Anal Canalmentioning

confidence: 99%

Restorative proctocolectomy and ileal pouch-anal anastomosis for familial adenomatous polyposis revisited

et al. 2006

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Since restorative proctocolectomy (RPC) with ileal-pouch anal anastomosis (IPAA) removes the entire diseased mucosa, it has become firmly established as the standard operative procedure of choice for familial adenomatous polyposis (FAP). Many technical controversies still persist, such as mesenteric lengthening techniques, close rectal wall proctectomy, endoanal mucosectomy vs. double stapled anastomosis, loop ileostomy omission and a laparoscopic approach. Despite the complexity of the operation, IPAA is safe (mortality: 0.5-1%), it carries an acceptable risk of non-life-threatening complications (10-25%), and it achieves good long-term functional outcome with excellent patient satisfaction (over 95%). In contrast to the high incidence in patients operated for ulcerative colitis (UC) (15-20%), the occurrence of pouchitis after IPAA seems to be rare in FAP patients (0-11%). Even after IPAA, FAP patients are still at risk of developing adenomas (and occasional adenocarcinomas), either in the anal canal (10-31%) or in the ileal pouch itself (8-62%), thus requiring lifelong endoscopic monitoring. IPAA operation does not jeopardise pregnancy and childbirth, but it does impair female fecundity and has a low risk of impairment of erection and ejaculation in young males. The latter can almost completely be avoided by a careful "close rectal wall" proctectomy technique. Some argue that low risk patients (e.g. <5 rectal polyps) can be identified where ileorectal anastomosis (IRA) might be reasonable. We feel that the risk of rectal cancer after IRA means that IPAA should be recommended for the vast majority of FAP patients. We accept that in some very selected cases, based on clinical and genetics data (and perhaps influenced by patient choice regarding female fecundity), a stepwise surgical strategy with a primary IPA followed at a later age by a secondary proctectomy with IPAA could be proposed.

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Section: Risk Of Neoplasia In the Anal Canalmentioning

confidence: 99%

Restorative proctocolectomy and ileal pouch-anal anastomosis for familial adenomatous polyposis revisited

et al. 2006

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“…Although extra-colonic neoplasms are observed often in patients with FAP, desmoids and upper gastrointestinal malignancies are considered important as causes of death (Tulchinsky et al 2005;de Campos et al 2010). Gastric fundic gland polyps (FGPs), gastric cancer, duodenal adenoma, and ampullary adenoma complicate in patients with FAP (Kashiwagi and Spiegelman 2000).…”

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confidence: 99%

Clinical Characteristics of Gastric Cancer in Patients with Familial Adenomatous Polyposis

Shibata

Ogawa

Miura

et al. 2013

Tohoku J. Exp. Med.

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Familial adenomatous polyposis is an autosomal dominant hereditary disease leading to the development of numerous colorectal polyps with malignant potential. Extra-colonic neoplasms are observed often in patients with familial adenomatous polyposis, but clinical characteristics of gastric cancer associated with familial adenomatous polyposis are not well understood. We studied the clinical characteristics of five Japanese patients who developed gastric cancer after undergoing colectomy for familial adenomatous polyposis. Gastric cancer was found on gastroduodenal endoscopy performed during postoperative follow-up in all five patients. Mean ages at the time of colectomy and at the time of diagnosis of gastric cancer were 39.2 and 58 years, respectively. Importantly, gastric fundic gland polyps were undetectable in these five patients. The mean duration between colectomy and diagnosis of gastric cancer was more than 20 years in three of five patients (mean: 19 years and 1 month). Cancers were multiple in three of five patients. Two patients developed metachronous gastric cancer in the gastric remnant. All five patients presented with well to moderately differentiated adenocarcinoma; four of the five patients were stage IA. Characteristics of the gastric cancer with familial adenomatous polyposis include a long duration between occurrence of gastric cancer and colectomy, metachronous cancers, multicentric lesions, and a high rate of well to moderately differentiated adenocarcinoma. Long-term and periodic gastroduodenal surveillance endoscopy is recommended for patients with FAP who underwent colectomy.

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“…TULCHINSKY et al (17) estudaram 50 doentes com PAF pós-retocolectomia total, dos quais 41 tiveram seguimento endoscópico e, destes, 11 desenvolveram adenomas duodenais (26,8%). Esses autores apresentaram taxa de aderência ao seguimento de 82%, próxima a obtida neste estudo (88,7%).…”

Section: Discussionunclassified

“…A causa de mortalidade dos doentes com PAF que foram submetidos a retocolectomia, tem mudado substancialmente, sendo o tumor duodenal e o tumor desmóide as causas mais freqüentes de óbito nesta população (9,17) . SPIGELMAN et al (16) propuseram uma classificação para os pólipos duodenais de acordo com o número de pólipos encontrados, além do tamanho, histologia e grau de displasia.…”

Section: Discussionunclassified

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Polipose gastroduodenal em doentes com polipose adenomatosa familiar Pós-Retocolectomia

Leal

Ayrizono

Coy

et al. 2007

Arq. Gastroenterol.

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RESUMO -Racional -As manifestações extracólicas, como os pólipos gastroduodenais e o tumor do duodeno, são fatores que influenciam a morbimortalidade dos doentes com polipose adenomatosa familiar no seguimento pós-retocolectomia total. Objetivo -Investigar a freqüência destas alterações em doentes com polipose adenomatosa familiar e verificar a eficácia do rastreamento endoscópico. INTRODUÇÃOA polipose adenomatosa familiar (PAF) é doença autossômica dominante que afeta de 1 a 8 indivíduos a cada 10.000 habitantes, distribuídos igualmente em ambos os sexos e presente em todos os grupos étnicos. Geralmente o aparecimento dos pólipos ocorre na puberdade, sendo caracterizada pela presença de mais de 100 pólipos adenomatosos no cólon e reto (7,14,17) .Mesmo após a retocolectomia, a morbimortalidade é influenciada pelas manifestações extracólicas, como os tumores desmóides, pólipos gastroduodenais e os tumores duodenais, sendo os tumores desmóides e duodenais as principais causas de óbito pós-retocolectomia (8) .O rastreamento endoscópico pós-operatório para pesquisa de pólipos gastroduodenais tem mostrado incidência de 90% a 95% de pólipos, sendo que 5% desenvolvem câncer (4,5,10) . No entanto, este risco é 100 a 300 vezes maior que na população em geral, na qual o carcinoma duodenal é de ocorrência rara (3,11,12) . Os achados endoscópicos muitas vezes geram dúvidas a respeito das condutas a serem realizadas, não estando suas freqüências bem estabelecidas na literatura (6) .O objetivo deste estudo foi investigar a freqüência de pólipos gastroduodenais na população de doentes com PAF em seguimento de retocolectomia em hospital universitário, a ocorrência de neoplasia local decorrente da transformação maligna desses pólipos e o grau de aderência ao esquema de acompanhamento pós-operatório. MÉTODOS Trata

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Extracolonic Manifestations of Familial Adenomatous Polyposis After Proctocolectomy

Abstract: Hypothesis: Extracolonic manifestations have a major effect on the morbidity and mortality of patients with familial adenomatous polyposis following proctocolectomy.

Cited by 34 publications

References 29 publications

Restorative proctocolectomy and ileal pouch-anal anastomosis for familial adenomatous polyposis revisited

Restorative proctocolectomy and ileal pouch-anal anastomosis for familial adenomatous polyposis revisited

Clinical Characteristics of Gastric Cancer in Patients with Familial Adenomatous Polyposis

Polipose gastroduodenal em doentes com polipose adenomatosa familiar Pós-Retocolectomia

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