腹腔镜全结肠切除治疗家族性腺瘤性息肉病12例

目的探讨腹腔镜全结肠切除术治疗家族性腺瘤性息肉病(familial adenomatous polyposis,FAP)的可行性及安全性。方法回顾性分析2015年6月～2018年12月腹腔镜全结肠切除术治疗12例FAP的临床资料,4例癌变者行腹腔镜全结肠直肠切除、末段回肠造口术,6例直肠下段未扪及明显息肉者行腹腔镜全结肠切除、直肠次全切除、回肠J形储袋直肠吻合术,2例直肠下段有少量息肉者行腹腔镜全结肠切除及直肠次全切除、残留直肠黏膜剥脱、经直肠肌鞘内回肠肛管吻合术。结果 12例均顺利完成手术,无中转开腹,术后2～3天肛门排气,住院时间8～12 d。随访3～24(13±3)月,无近期并发症发生,除回肠造口4例外,其余8例术后控便较好,3个月后大便能控制在3～6次/日。结论腹腔镜全结肠切除术治疗FAP创伤小,恢复快,安全,有效。     

家族性腺瘤性息肉病12例临床分析

目的总结家族性腺瘤性息肉病(FAP)诊断治疗经验。方法回顾分析12例 FAP 的临床病理资料。结果男5例,女7例,平均32岁。良性5例,平均25岁。恶性7例,平均37岁。8例有家族史,其中4个家系中有7人死于大肠癌。病史2～20年,息肉数均>100个,最多达3～7个/cm~2,7例癌变。全部病例均施行了全结肠切除,并分别采用回肠造口、回肠储袋及保肛手术。未癌变者均存活。癌变者有3例术后半年内出现脑、肝或腹腔转移。结论 FAP 易癌变,且癌变发生早,应争取在癌变前行结直肠全切除。手术以直肠粘膜剥脱,回肠储袋,回肠肛管吻合术为首选。     

家族性结直肠腺瘤样息肉病58例报告

目的 总结家族性结直肠腺瘤样息肉病(FAP)的最佳手术方式及临床效果.方法 对58例FAP患者的临床资料进行总结.结果 58例中良性45例,癌变13例.全部患者均施行了全结肠切除,采用回肠造口8例,部分直肠切除和回直肠吻合16例,直肠切除、回肠贮袋肛管吻合4例,直肠切除、直肠黏膜剥脱经直肠肌鞘内回肠J形贮袋肛管吻合30例(随访1～5年,大便次数2～4次/d,肛门有控便能力,无息肉复发、癌变,均能参加重体力劳动).本组严重并发症5例.随访6个月～8年,良性患者术后均存活,6例术后5～7年发生癌变而再次行手术.13例癌变者中,4例术后6～13个月因肿瘤广泛转移死亡,另9例仍存活.排便功能以回肠造口术较差,保留直肠的术式较好.结论 治疗FAP,选择全结肠切除、保留直肠肌鞘、回肠贮袋、回肠肛管吻合术较合适.     

家族性腺瘤性息肉病12例临床分析

目的 总结家族性腺瘤性息肉病(FAP)诊断治疗经验。方法 回顾分析12例FAP的临床病理资料。结果 男5例，女7例，平均32岁。良性5例，平均25岁。恶性7例，平均37岁。8例有家族史，其中4个家系中有7人死于大肠癌。病史2～20年，息肉数均>100个，最多达3～7个／cm^2，7例癌变。全部病例均施行了全结肠切除，并分别采用回肠造口、回肠储袋及保肛手术。未癌变均存活。癌变有3例术后半年内出现脑、肝或腹腔转移。结论 FAP易癌变，且癌变发生早，应争取在癌变前行结直肠全切除。手术以直肠粘膜剥脱，回肠储袋，回肠肛管吻合术为首选。     

家族性腺瘤性息肉病17例的诊断与外科治疗

目的：总结家庭性腺瘤性息肉病（FAP）的诊断和外科治疗经验。方法：回顾分析17例FAP的临床病理资料。结果：17例患者中男7例，女10例，平均年龄23．8岁，良性13例，平均年龄19．6岁，癌变4例，平均年龄37．8岁，13例有家庭史。家系调查发现1个家系至少有2例患者，最多可达7例，其中，6个家系中有7人已死于息肉癌变，全部患者均施行了全结肠切除，并分别采用回肠造口，回肠储袋，回肠肛管吻合等术式，随访2－20年，13例良性患者均存活。其中1例术后5年发生癌变，癌变者中1例术后5年发生肺部转移，行肺叶切除术后2年死亡，1例术后3年因广泛转移死亡，另2例为术后2年和6年，仍存活 。结论：FAP易癌变应尽早手术，手术宜选全结肠切除，直肠粘膜剥脱，回肠储袋，回肠肛管吻合术。     

腹腔镜手术治疗家族性腺瘤性息肉病临床分析

目的探讨家族性腺瘤性息肉病(familial adenomatous polyposis,FAP)的微创外科治疗疗效与安全。 方法回顾性分析2010年2月至2017年3月于北京协和医院行腹腔镜手术治疗的16例FAP患者的病例资料。 结果16例患者均完成腹腔镜手术,其中行全结直肠切除、回肠储袋肛管吻合术6例,全结肠直肠切除、回肠端式造口5例,全结肠切除、回肠直肠吻合术5例;无中转开腹,手术时间(227.9±52.7)min,平均术中出血量(98.1±90.7)ml。5例(31.2%)发生腺瘤癌变,4例(25.0%)伴高级别上皮内瘤变。14例患者获得长期随访,其中4例患者残余直肠新发息肉、3例患者术后发生腹腔硬纤维瘤。1例FAP合并肠癌患者术后1.5年因肝转移死亡。 结论腹腔镜手术治疗FAP安全可行;FAP患者术后需定期复查。     

溃疡性结肠炎的外科治疗16例分析

目的探讨溃疡性结肠炎的外科治疗方式。方法对16例行外科手术治疗的溃疡性结肠炎的临床资料进行回顾性分析。结果本组16例行外科手术治疗，占全部收治患者的20．5％（16／78）。手术原因包括保守治疗无效10例，肠梗阻3例，合并息肉可疑癌变3例。行全结直肠切除、回肠造口术8例，全结直肠全切除、回肠肛管吻合术4例，结肠全切除、回直肠吻合术2例，结肠部分切除2例。结论溃疡性结肠炎主要手术指征为内科治疗无效或合并肠梗阻及并发息肉可疑癌变者。全结肠切除、回肠造口术治疗较彻底，全结直肠切除、回肠贮袋肛管吻合术可改善排便控制功能，但吻合口溃疡发生率高。     

家族性腺癌性息肉病的手术选择(摘要)

家族性腺瘤性息肉病(FAP)是常染色体显性遗传性疾病,如不手术治疗终将发生癌变,本文报告23例,男12例,女11例,年龄19~62岁,35岁以下10例,有家族史13例,手术时有8例癌变。最小癌变年龄23岁。治疗:行全结肠直肠切除回肠造口术1例,只适用于中低位直肠有恶变或全结肠切除回肠直肠吻合术后直肠发生癌变无法保留肛门括约肌者。行全结肠切除回肠直肠吻合术12例,保留段直肠为6~8cm便于术后复查直肠。适应证为直肠下段腺瘤少并能长期随访。行升结肠切除升结肠直肠吻合术1例。行全结肠切除,直肠部分切除,直肠粘膜剥除共9例,其中回肠末端直接与肛管吻合4例,J—pouch肛管吻合4例,S-pouch肛管吻合1例。随访1~18年,平均97个月,脾曲癌变1例术后3年死亡。回肠直肠吻合术12例中有4例直肠腺瘤再发多次手术切除,其中1例直肠腺瘤第3次再发未接受手术切除而致癌变,经局部切除。术后近期排便功能回肠直肠吻合组优于直肠粘膜剥除组。回肠贮袋有无远期排便功能无差别。作者认为应根据病变的范围、程度、有无恶变、能否长期随访、医生的经验等选择手术治疗方法。只要适应证选择合适即能取得良好的疗效。     

全大肠切除回肠贮袋肛管吻合术的远期疗效评价

目的　探讨全大肠切除回肠贮袋肛管吻合术治疗溃疡性结肠炎 (UC)和家族性腺瘤性息肉病 (FAP)的远期疗效。方法　 1985～ 2 0 0 2年间共对 6 1例患者行全结肠直肠切除回肠贮袋肛管吻合术 ,其中UC 2 5例、FAP 34例、其他 2例 ,应用S袋 2 5例、S J袋 13例、J袋 17例以及W袋 6例 ,术后随诊 1～ 18年、平均 8年 ,比较分析术后控便功能及并发症。结果　全部患者存活。并发症发生率为 16 % ( 10 / 6 1) ,其中UC患者 ( 2 4 % ,6 / 2 5 )高于FAP患者 ( 12 % ,4 / 34) ,W袋高于其他各袋 ,手法吻合 ( 2 0 % ,8/ 4 1)高于吻合器吻合 ( 10 % ,2 / 2 0 ) ,但差异均无显著性 (P >0 0 5 )。术后 2 4h大便次数平均为 4 2次。日间 84 % ( 4 3/ 5 1)、夜间 75 % ( 38/ 5 1)的患者排便能自控 ,6 % ( 3/ 5 1)的患者有明显的大便失禁。大部分患者对目前的生活质量表示满意。结论　全大肠切除回肠贮袋肛管吻合术治疗可维持UC与FAP患者肛管自控功能和可接受的大便次数 ,术后并发症较少 ,是较为理想的手术方式     

全结肠切除回肠"S"型储袋肛管吻合术治疗家族性腺瘤性息肉病

目的:探讨全结肠切除回肠“S“型储袋肛管吻合术治疗FAP的效果.方法:回顾分析1995至2005年进行的33例FAP全结肠切除术,对治疗效果进行分析.结果:33例患者中良性21例,癌变12例.全部患者均施行了全结肠切除,27例回肠“S“型储袋肛管吻合术,6例永久性回肠造口术; 手术后无严重并发症发生.33例均得到随访1～10年, 5例癌变病人因肿瘤复发、肝转移死亡.余者术后生存质量满意,排便功能良好. 结论:全结肠切除回肠“S“型储袋肛管吻合术治疗FAP息肉切除彻底、术后控便能力理想,该术式是治疗FAP较为理想的手术方式.     

家族性腺瘤性息肉病的诊断与治疗

目的 总结家族性腺瘤性息肉病（ＦＡＰ）的诊断和治疗经验。方法 回顾性分析１３例ＦＡＰ的临床病理资料。结果 本组１３例患者,男５例,女８例,平均年龄３１．５岁。其中良性５例,平均年龄２５岁;癌变８例,平均年龄３６岁。８例有家族史者源于６个家系,３代中共发现１９例患者其中４个家系中有７例２死于大肠癌。本组患者病史２－２０年,每例息肉数均超过１００个。最多达３－９个／ｃｍ＾２。全部病例均施行了全结肠切除     

全大肠切除直肠肌鞘内回肠造袋肛门吻合术治疗家族性腺瘤性息肉病

家族性腺癌性息肉病（ＦＡＰ）为一种常染色体显性遗传病，如不手术治疗终将发生癌变。现已公认本病的最佳术式为全大肠切除，直肠粘膜剥除，回肠造袋肛门吻合术。作者近９年内施行此手术１１例。患者年龄为１９～６５岁。５例在３０岁以内。平均随访１～９年，效果满意。除２例切口感染外无其它并发症。每日大便４～６次，能恢复工作。手术时５例已有恶变，其中１例有单个肝转移灶同时行局部切除。手术要点为：肌鞘宜短，充分止血并放置引流，无张力吻合，保护性回肠造口。作者认为除直肠内仅少数息肉，患者能坚持随访者外，均宜采用本术式。     

Clinical characteristics of familial adenomatous polyposis and management of duodenal adenomas

The aim of this study was to evaluate the clinical characteristics of patients with familial adenomatous polyposis (FAP) undergoing surgical treatment over a 10-year period and specifically to evaluate the incidence and clinical outcome of patients treated for duodenal adenomas. Patients with FAP who underwent surgical treatment for colonic polyposis at the University of Louisville from January 1992 to July 2002 were investigated. Surgical treatment included colectomy and ileal J-pouch-anal anastomosis (IPAA) or completion proctectomy with or without IPAA in those who had previously undergone subtotal colectomy elsewhere. All patients underwent screening gastroduodenoscopy at 3-year intervals beginning at the time of diagnosis or referral. Postoperative morbidity, mortality, and functional outcome were evaluated, as well as the occurrence of extracolonic manifestations and results of treatment for duodenal adenomas when required. Fifty-four patients were included in the study (mean age 28 ± 2 years). Twentyseven of them (50%) underwent colectomy and IPAA as the initial operation. Twenty-seven patients had previously undergone subtotal colectomy. Eight of these 27 patients had cancer in the rectum, of which three were T4 and one was T2N1 cancer. Twenty-two patients underwent a completion proctectomy and three required abdominoperineal resection. Twenty of the 54 patients developed duodenal adenomas. The mean age of diagnosis of duodenal disease was not significantly different from that of patients who were still free of duodenal polyps (40 ± 11 vs. 34 ± 12 years). Seven of these 20 patients underwent local excision of duodenal polyps (either endocopically or transduodenally); four of these patients developed recurrent disease. Six patients underwent pancreaticoduodenectomy for duodenal adenomas with severe dyplasia. These patients experienced an increased number of bowel movements, from five per day (range 4 to 8) to 10 per day (range 6 to 15). One patient required pouch excision and end ileostomy to control diarrhea. Our data demonstrate the following: (1) patients with FAP who have undergone prior subtotal colectomy and ileorectal anastomosis have a high risk of developing advanced cancer in the rectal stump; (2) duodenal adenomas are common in patients with FAP and may occur at an early age; (3) screening duodenoscopy should be initiated at the time of diagnosis of FAP; (4) local excision of duodenal adenomas is associated with a high risk of local recurrence; and (5) even though pancreaticoduodenectomy is the treatment of choice for advanced duodenal adenomas, this procedure may adversely affect pouch function in some patients.     

Ampullary carcinoma in familial adenomatous polyposis: Report of a case

We report the case of a 48-year-old man with familial adenomatous polyposis (FAP) who was found to have ampullary carcinoma 6 years after undergoing total colectomy. The patient was examined periodically after colorectal surgery except for duodenoendoscopy because he had also previously undergone distal gastrectomy with gastrojejunal anastomosis for a duodenal ulcer at 27 years of age. An ampullary lesion was suspected on blood chemistry and detected by computed tomography scan and ultrasonography although the patient showed no clinical symptoms. A pancreatoduodenectomy was performed and the histological examination revealed well-differentiated adenocarcinoma with no metastasis to the regional lymphnodes. Long-term periodic surveillance of the upper gastrointestinal tract including the papilla of Vater is therefore important for FAP patients who have undergone prophylactic colectomy.     

腹腔镜全结肠切除术在家族性腺瘤性结肠息肉病及溃疡性结肠炎中的临床应用

目的:探讨腹腔镜全结肠切除术在家族性腺瘤性结肠息肉病(familial adenomatous polyposis,FAP)及溃疡性结肠炎(ulcerative colitis,UC)治疗中的应用价值。方法:回顾分析2009～2012年为12例患者行腹腔镜全结肠切除术(FAP患者7例,UC患者5例)及18例传统开腹全结(直)肠切除术(FAP患者7例,UC患者11例)的临床资料。结果:腹腔镜组在切口长度、术中出血量、术后止痛药使用量、术后排气时间、拔除导尿管时间、术后住院时间等方面优于开腹组,住院总花费明显高于传统开腹手术。结论:腹腔镜全结肠切除术用于FAP及UC安全、可行,与传统开腹手术相比,具有患者创伤小、康复快等优点,手术效果不亚于传统开腹手术,具有较高的临床应用价值。     

家族性腺瘤性息肉病手术方式的探讨

目的 探讨家族性腺瘤性息肉病(FAP)最佳手术方式。方法 总结1980～2000年各种手术治疗65例FAP的临床资料。结果 其中16例采用全大肠切除经肛门外直肠粘膜剥脱直肠肌鞘内回肠肛管吻合术，随访5年，大便次数3～5次／日，肛门有控制功能，无息肉复发、癌变，体重增加，均能参加重体力劳动，无并发症。结论 该术式是治疗FAP较为理想的手术方式。     

Pancreatic acinar cell carcinoma in familial adenomatous polyposis

Pancreatic carcinoma is rarely associated with familial adenomatous polyposis (FAP). Ten patients with this association have been reported in the literature. However, detailed data were available in only a few cases. The first case of pancreatic acinar cell carcinoma associated with FAP is reported. A 51-year-old man who had undergone total colectomy and ileoproctostomy for colonic polyposis 29 years previously was admitted with a cancer in the residual rectum. Preoperative examinations revealed a tumor in the head of the pancreas. Pancreaticoduodenectomy and very low anterior resection of the rectum were performed. Histologically, the tumor of the pancreas was acinar cell carcinoma. In patients with FAP, we recommend careful surveillance of not only the gastrointestinal tract but also of other organs such as those of the pancreaticobiliary system and the endocrine organs.     

Laparoscopic-assisted operation for familial adenomatous polyposis patients-two case reports.

OBJECTIVE: We describe herein the results of 2 laparoscopic operations to treat patients with familial adenomatous polyposis (FAP). METHODS: Two female FAP patients, aged 32 and 29 years old, were treated with restorative proctocolectomy and total colectomy with ileorectal anastomosis (hand-assisted laparoscopic surgery), respectively. RESULTS: The operative time was 360 minutes for the restorative proctocolectomy and 150 minutes for the total colectomy with ileorectal anastomosis. The blood loss was 500 cc for the restorative proctocolectomy and minimal for the total colectomy patient. The return of bowel movements took 3 days for each patient, and no complication occurred. Patients were discharged on the 15th and 7th postoperative days. CONCLUSION: A laparoscopic approach for restorative proctocolectomy or total colectomy with ileorectal anastomosis is safe and technically feasible, and provides good cosmesis.     

RECTAL CANCER FOLLOWING COLECTOMY AND ILEORECTAL ANASTOMOSIS FOR FAMILIAL ADENOMATOUS POLYPOSIS

Background : Familial adenomatous polyposis (FAP) has historically been treated by colectomy and ileorectal anastomosis (IRA). Preservation of the rectum allows the subsequent development of cancer in the rectum. The risk of rectal cancer following ileorectal anastomosis in the Australian population has not been published to date. Methods : An audit of the Familial Adenomatous Polyposis Registry of Western Australia was undertaken to assess patients who had undergone colectomy and ileorectal anastomosis. Fifty-five patients ranging in age from 13 to 65 years were studied. Results : Seven patients (13%) developed cancer of the rectum with a median follow-up of 10 years (range: 1–31 years). Median interval to diagnosis of carcinoma of the rectum following colectomy and IRA was 10 years. All patients who developed cancer in the retained rectum had rectal polyps. Colon cancer was present in the initial colectomy specimen in 13 patients (of these, five patients developed rectal cancer). Flat polyps were noted in five patients. Four patients with flat polyps developed cancer of the rectum. Conclusions : Total colectomy and IRA should be considered as part 1 of a staged procedure in the patient with FAP. With the exception of the patient with no evidence of rectal polyps, completion proctectomy should be undertaken within 10 years of the initial colectomy.