Vasen Hans F A, Ghorbanoghli Zeinab, de Ruijter Bastian, Trinidad Ro-Anne, Langers Alexandra M J, Peeters Koen C M J, Bonsing Bert A, Hardwick James C H
Department of Gastroenterology & Hepatology, Leiden University Medical Centre , Leiden , The Netherlands.
The Netherlands Foundation for the Detection of Hereditary Tumours , Leiden , The Netherlands.
Scand J Gastroenterol. 2019 Jun;54(6):733-739. doi: 10.1080/00365521.2019.1621930. Epub 2019 Aug 11.
Familial adenomatous polyposis (FAP) is characterized by the development of hundreds of colorectal adenomas in the second decade of life, and prophylactic colectomy is usually performed around age of 20. A common question is the appropriate timing of surgery and which endoscopic findings indicate surgery. All FAP patients known at Leiden University Medical Centre from 1985 onwards were included. The patients were then subdivided into those diagnosed before or after 2000. Patient information included age at diagnosis, colonic phenotype, age at surgery, pathological findings and the outcome of follow-up colonoscopies in whom surgery was postponed. The 72 FAP patients identified consisted of 33 patients diagnosed before (group A) and 39 after (group B) 2000. The median age at diagnosis for patients with classical FAP was 18 in groups A and B. All patients diagnosed before 2000 underwent colorectal surgery versus 68% of those diagnosed >2000. The median age at surgery for classical FAP patients was 19 and 24 years in groups A and B, respectively. In patients with intact colon, the number of adenomas gradually increased over many years. Although most adenomas remained <5 mm, the proportion of 5-15 mm adenomas slowly increased. Only one patient developed a high-grade adenoma. None of the patients developed CRC. Surgery today in FAP is performed less often and at a more advanced age. Our experience also suggests that surgery can be safely postponed in selected patients. The most important endoscopic indication for surgery is substantial number of large adenomas of >5-10 mm.
家族性腺瘤性息肉病(FAP)的特征是在生命的第二个十年发展出数百个结肠腺瘤,通常在20岁左右进行预防性结肠切除术。一个常见的问题是手术的合适时机以及哪些内镜检查结果提示需要手术。纳入了自1985年起在莱顿大学医学中心确诊的所有FAP患者。然后将患者分为2000年之前或之后确诊的两组。患者信息包括诊断时的年龄、结肠表型、手术时的年龄、病理结果以及推迟手术患者的后续结肠镜检查结果。确定的72例FAP患者中,33例在2000年之前确诊(A组),39例在2000年之后确诊(B组)。A组和B组中经典型FAP患者的诊断中位年龄均为18岁。2000年之前确诊的所有患者均接受了结直肠手术,而2000年之后确诊的患者中这一比例为68%。A组和B组中经典型FAP患者的手术中位年龄分别为19岁和24岁。在结肠完整的患者中,腺瘤数量在多年间逐渐增加。尽管大多数腺瘤仍小于5毫米,但5 - 15毫米腺瘤的比例缓慢上升。只有1例患者发展为高级别腺瘤。没有患者发生结直肠癌。如今FAP患者接受手术的频率降低且手术年龄更大。我们的经验还表明,在部分患者中手术可以安全推迟。手术最重要的内镜指征是大量直径>5 - 10毫米的大腺瘤。