家族性息肉病的外科治疗

家族性息肉病是一种遗传性肿瘤性息肉，癌变率甚高，应及时手术治疗。本文对２１例家族性息肉病患者的诊治进行了分析，并提出了合理的手术方式。     

家族性腺瘤性息肉瘤的外科治疗

为了提高家族性腺瘤性息肉瘤的外科治疗效果，对１６例ＦＡＰ患者的外科治疗情况进行了回顾性总结。１６例中除１例行全结肠直肠切除，回肠造口外，其余１５例均行全结肠直遥切除、回肠贮袋代管吻合术。     

全结肠切除术治疗家族性多发腺瘤性息肉病六例

目的 探讨全结肠直肠切除回肠储袋肛管吻合术治疗家族性多发腺瘤性息肉病的效果.方法 回顾性分析2005年6月至2012年6月行全结肠切除术治疗家族性多发腺瘤性息肉病6例的临床资料.结果 6例患者均施行了全结肠直肠切除,回肠储袋肛管吻合术,同时作临时回肠造口术,均3个月后还纳;手术后无严重并发症发生.6例随访6个月至7年,2例患者肿瘤复发,1例死亡,5例仍存活,1例再次行肿瘤切除.6例术后能正常工作和生活,排便功能好.结论 回肠储袋肛管吻合术治疗家族性多发腺瘤性息肉病、切除彻底,术后控便能力比较好,不影响工作和生活,是治疗家族性多发腺瘤性息肉病比较好的手术方式,手术宜早.     

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。     

家族性腺瘤性息肉病

家族性腺瘤性息肉病晏仲舒，廖国庆家族性腺瘤性息肉病（ＦＡＰ）为常染色体显性遗传病，外显率达９０％以上［１］，按出生人口计其发病率约为１／５０００～１／１７０００［１，２］，如不治疗，最终１００％会发展成大肠癌。虽然由ＦＡＰ所致的在肠癌仅占１％～５％，...     

应用吻合器手术治疗家族性腺瘤样息肉病21例分析

目的探讨3种吻合器在治疗家族性腺瘤样息肉病的临床应用价值。方法回顾性分析华中科技大学同济医学院附属同济医院2000年1月至2005年12月间采用吻合器手术治疗家族性腺瘤样息肉病21例的临床资料。结果21例病人均采用3种吻合器手术,行全结肠切除、直肠超低位前切除、回肠J形贮袋肛管吻合术,并预防性回肠造口。全组无手术死亡病人、无吻合口瘘、盆腔脓肿、吻合口狭窄等严重并发症发生。1例病人发生贮袋粘连,在直肠镜下分离治愈;2例吻合口附近发现息肉经内镜电灼切除。全组病人排便功能满意,排便次数控制在每天2～5次。结论采用3种吻合器进行结肠切除、直肠超低位前切除、回肠贮袋与肛管吻合术,并预防性回肠造口,手术安全、简捷省时、术后并发症较少,应作为首选手术方式,手术后应进行长期随访,及时发现和处理复发息肉。     

家族性腺瘤性息肉病

作者单位自１９８５年１１月至１９９６年１１月，共收治家族性腺癌性息肉病（ＦＡＰ）１２例，其中６７％有明确家族史，５８％单例息肉＞１００个，ＦＡＰ癌变率为１７％。同时报告一个三代２４口人中９人患ＦＡＰ的典型家系，其ＦＡＰ癌变率６７％。作者分析了ＦＡＰ的遗传倾向与ＦＡＰ分布的关系，指出通过先证者（Ｉｎｄｅｘｃａｓｅ）普查ＦＡＰ患者的重要性。作者强调ＦＡＰ有明确的癌变遗传基础。所以ＦＡＰ的癌变与息肉大小无关，而是一种特定区域性的必然的癌变时相。对于ＦＡＰ外科手术方式，作者提倡无会阴部痛苦的全结肠、直肠切除，永久性回肠造口术。作者对本组病例的遗传倾向、息肉癌变特点及外科手术方式进行了分析讨论。     

家族性息肉病的外科治疗

家族性息肉目前最有效的治疗是预防性手术，手术方式多种。本文综述近年来各种手术及其临床效果的报道。     

腹腔镜辅助下全大肠切除、回肠储袋与直肠肌管吻合术治疗家族性腺瘤性息肉病

目的 探讨腹腔镜辅助下全大肠切除、回肠储袋与直肠肌管吻合术在治疗家族性腺瘤性息肉病(familial adenomatous polyposis,FAP)中的价值.方法 回顾性统计分析7例FAP患者行腹腔镜辅助下全大肠切除、回肠储袋与直肠肌管吻合术的手术、术后恢复情况及随访资料,并与同期开腹手术患者相比较.结果 腹腔镜手术患者手术切口平均长度为4.32 cm,明显短于开腹手术,差异有统计学意义;术后排气时间平均为1.45 d,短于开腹患者,两组间差异无统计学意义;住院时间平均为11.95d,明显短于开腹手术患者,差异有统计学意义.术后6个月随访资料显示回肠储袋与直肠肌管吻合术安全可靠,较回肠储袋肛管吻合术后患者排便次数明显减少,肛管静息压和最大收缩压明显增高.结论 与原有回肠储袋肛管吻合术相比,腹腔镜辅助全大肠切除、回肠储袋直肠肌管吻合术不仅手术创伤小,手术方式简单,手术难度低,而且减少了可能发生的盆底肌肉和肛门括约肌损伤,更好地保留患者控排便功能.     

Rectal and Pouch Recurrences After Surgical Treatment for Familial Adenomatous Polyposis

Background  Familial adenomatous polyposis (FAP) is a genetic disease characterized by multiple adenomatous colorectal polyps and different extracolonic manifestations (ECM). The present work is aimed to analyze the outcome after surgical treatment regarding complications and cancer recurrence. Methods  Charts from patients treated between 1977 and 2006 were retrospectively analyzed. Clinical and endoscopic data, results of treatment, pathological reports and information about recurrence were collected. Results  Eighty-eight patients (41 men [46.6%] and 47 women [53.4%]) were assisted. At diagnosis, associated colorectal cancer (CRC) was detected in 53 patients (60.2%), whose average age was higher than those without CRC (40.0 vs. 29.5 years). At colonoscopy, polyposis was classified as attenuated in 12 patients (14.3%). Surgical treatment consisted in total proctocolectomy with ileostomy (PCI, 15 [17.4%]), restorative proctocolectomy (RPC, 27 [31.4%]), total colectomy with ileal-rectum anastomosis (IRA, 42 [48.8%]), palliative segmental resection (1 [1.2%]) and internal bypass (1 [1.2%]). Two patients were not operated on due to religious reasons and advanced disease. Complications occurred in 25 patients (29.0%), more commonly after RPC (48.1%). There was no operative mortality. Local or distant metastases were detected in six (11.3%) patients with CRC treated to cure. During the follow-up of 36 IRA, cancer developed in the rectal cuff in six patients (16.6%), whose average age was higher than in patients without rectal recurrence (45.8 vs. 36.6 years). Five of them have had colonic cancer in the resected specimen. Among the 26 patients followed after RPC, cancer in the ileal pouch developed in 1 (3.8%). Conclusions  (1) Within the present series, FAP patients presented a high incidence of associated CRC and diagnosis was generally established after the third decade of life; (2) operative complications occurred in about one third of the patients, being more frequent after the confection of an ileal reservoir; (3) rectal cancer after IRA was detected in 16.6% of patients and it was associated with greater age and previous colonic carcinoma; (4) both continuous and long-term surveillance of the rectal stump and ileal pouch are necessary during follow-up.     

大肠家族性腺瘤性息肉病癌变的治疗

目的:分析大肠家族性腺瘤性息肉病的临床特点并探讨正确的手术治疗方法。方法:分析1985年1月～2002年8月我院收治的19例家族性腺瘤性息肉病癌变病例。结果:19例病人均发生癌变,6例有大肠癌家族史;肿瘤大小平均为5cm;Dukes分期:A期3例,B期7例,C期7例,D期2例;9例发生淋巴结转移;随访17例,2例死亡。结论:手术治疗仍为主要的治疗方案,应采用全结肠切除,回肠、直肠或肛管吻合,J形储袋是应推荐的手术方案。     

Prevalence of Adenomas and Carcinomas in the Ileal Pouch After Proctocolectomy in Patients with Familial Adenomatous Polyposis

Purpose  Restorative proctocolectomy has become the most common surgical option for patients with familial adenomatous polyposis (FAP). However, adenomas may develop in the ileal pouch mucosa over time, and even carcinoma in the pouch has been reported. Our aim was to evaluate the prevalence, nature, and etiology of ileal pouch and nonpouch adenomas and carcinoma in patients with FAP. Patients and methods  This was a retrospective study of 31 FAP patients with Kock’s continent ileostomy (Kock; n = 8), ileorectal anastomosis (IRA; n = 7), and ileal pouch–anal anastomosis (IPAA) (n = 16). All patients were followed with a standardized protocol including chromoendoscopy and biopsies of visible polyps in the ileal pouch and nonpouch mucosa. Results  Sixteen of 24 pouch patients (Kock and IPAA) developed adenomas in the ileal pouch mucosa, and all patients with IRA developed adenomas in the rectal mucosa. The prevalence of ileal adenomas was significantly higher in pouch patients than in IRA patients (P = 0.002). Only one patient with Kock showed adenoma in the prepouch area. Two cases of adenocarcinomas and one case of advanced adenoma were found in the ileal pouch mucosa. Conclusion  Our results show a high frequency of adenomas in the ileal pouch mucosa, with evolution into carcinoma in some patients. Regular endoscopic surveillance of the pouch is recommended at a frequency similar to that for the rectal mucosa after IRA in pouch patients with FAP.     

Laparoscopic restorative proctocolectomy with and without previous subtotal colectomy

Introduction  Laparoscopic techniques have been applied to the procedure of restorative proctocolectomy (RPC). The aim of this study was to compare the outcomes of patients after laparoscopic ileal pouch-anal anastomosis (IPAA) with restorative proctocolectomy (RPC) and without previous colectomy [restorative proctectomy (RP)] and to highlight some technical tips.
Method  Data were collected prospectively from all patients who underwent laparoscopic IPAA from July 2006 to December 2007.
Results  Thirty-six patients underwent IPAA either with total proctocolectomy ( n  = 25) or after previous emergency colectomy ( n  = 11). Postoperative morbidity occurred in 22% of patients. The overall median operative time was 210 (range 120–325), 240 (170–325) and 180 (120–240) min for RPC and RP respectively ( P  < 0.05). The median length of stay for all patients was 6 (3–26), 6 (4–26) and 4 (3–13) days for RPC and RP respectively ( P  < 0.05). There was no correlation between BMI, age, use of immunosuppressive agents and length of stay. The operative procedure was facilitated by the use of specific devices at particular stages of the operation.
Conclusion  Laparoscopic IPAA is not only safe and feasible for the virgin abdomen but also for patients with a previous emergency colectomy through a midline laparotomy incision.     

The Ileal Pouch Anal Anastomosis: To Divert or not to Divert? The Case for Diversion

The ileal pouch anal anastomosis is a high-risk anastomosis with a high rate of complications. Because of this proximal diversion has often been done when performing this operation. Recently the practice of proximal diversion has been questioned, noting the high rate of complications associated with the stoma and stomal reversal. However, the cumulative data does suggest that an undiverted ileal pouch anal anastomosis has a higher leak rate and is associated with increased pelvic sepsis. Because of the severity of this complication proximal diversion is still advocated in the majority of cases. This paper was originally presented as part of the SSAT/SAGES Joint Symposium entitled, “The Gastrointestinal Anastomosis: Evidence vs. Tradition; The Colon Anastomosis: Diversion or Primary Anastomosis”, at the SSAT 49th Annual Meeting, May 2008, in San Diego, California. The other articles presented in this symposium were Koruda MJ, “Diverting Ostomy with Pouch Procedure Causes More Morbidity Than It Prevents,” and DuBose J, “Colonic Trauma: Indications for Diversion vs. Repair.     

Ileal pouch anal anastomosis in pediatric familial adenomatous polyposis: A 24-year review of operative technique and patient outcomes

Background

Total proctocolectomy with ileal pouch anal anastomosis (IPAA) is the operative procedure of choice for familial adenomatous polyposis (FAP) patients. We review 24 years of operative experience and outcomes in pediatric patients with FAP.

Methods

Patients with FAP, age < 20 years, presenting to a single institution between 1987 and 2011 were included. Operative technique and outcomes were reviewed retrospectively. Primary outcomes included postoperative complications (30 days), long-term bowel function, and polyp recurrence at the anal anastomosis.

Results

95 patients with FAP underwent IPAA. Mean age at IPAA was 15.5 years with a mean follow-up of 7.6 years. 29 patients underwent 1-stage IPAA, 65 patients had a two-stage IPAA, and 1 patient underwent a 3-stage procedure. 67 patients had an open procedure, 25 underwent a laparoscopic approach, and more recently 3 patients underwent single incision laparoscopic IPAA. Patients with 1-stage IPAA demonstrate better long term bowel control vs. 2-stage IPAA patients (10.7% vs. 36.0% occasional incontinence, p = 0.018). However, 1-stage IPAA patients suffered increased short-term complications, such as anastomotic leak (17.2% vs. 0%, p = 0.002) and reoperation (20.7% vs. 4.6%, p = 0.02) compared to 2-stage IPAA. Anal anastomosis polyp recurrence occurred in 22.7% of 1-stage patients and 10.0% of 2-stage patients. Short-term complications, polyp recurrence, or long-term continence were equivalent between open and laparoscopic cases.

Conclusion

Single-stage IPAA in children with FAP is associated with better bowel control but increased anastomotic leak, reoperative rate, and polyp recurrence. In experienced hands, laparoscopic IPAA is equivocal to open IPAA.     

Gender of the patient may influence perioperative and long‐term complications after restorative proctocolectomy

Aim Gender‐related differences in preoperative characteristics and early and long‐term outcome for patients undergoing ileal pouch anal anastomosis (IPAA) have not previously been well studied. Method All male and female patients undergoing IPAA at a single centre between 1983 and 2008 were compared for perioperative variables and long‐term outcome. Statistical tests were used as appropriate. A multivariate analysis was performed to evaluate the effects of gender on pouch failure. Results Female patients (n = 1495) were younger than male patients (n = 1912) (P < 0.001). Surgery type and pouch configuration were similar, although male gender was associated with a higher use of ileostomy (P < 0.001) and a higher incidence of 30‐day anastomotic separation (P = 0.001). During a median follow up of 9.9 (female) and 9.3 (male) years, female patients were more likely to develop bowel obstruction (20.8 vs 16.7%, P = 0.02) and pouch‐related fistula (10.9 vs 7.6%, P = 0.001). Women had a higher number of daily bowel movements than men (P = 0.001), and more frequently had urgency (P = 0.001), daily seepage (P = 0.01) and pad use (P < 0.001). A higher percentage of female patients reported dietary (P < 0.001) and work (P = 0.022) restrictions and lower mental component of the Short‐Form 36 quality of life score (P = 0.018). On multivariate analysis of perioperative variables, female gender was associated with pouch failure (P = 0.05). Conclusion The gender of the patient seems to be associated with specific differences in preoperative variables and postoperative outcomes for patients undergoing IPAA.     

Predictors for acute and chronic pouchitis following restorative proctocolectomy for ulcerative colitis

Objective  This study was undertaken to evaluate the cumulative incidence, onset and risk predicting factors for acute and chronic pouchitis. Method  A consecutive series of patients (n = 210), who underwent restorative proctocolectomy (RPC) and had a minimum follow‐up of 12 months was reviewed. The cumulative incidence and onset of pouchitis was determined. Univariate analysis, followed by logistic regression analysis was used to evaluate the association of various demographic, clinical and histopathologic variables with the subsequent development of acute and chronic pouchitis. Results  A total of 198 patients were included. The mean follow‐up was 64 months (range, 12–180). Sixty‐four patients (32%) developed pouchitis, 35 acute and 29 chronic. The first episode of pouchitis occurred within the first year in 70% of cases. The presence of backwash ileitis (OR, 2.6; P = 0.015), primary sclerosing cholangitis (PSC; OR, 2; P = 0.018) and the duration of follow‐up (OR, 1.1; P = 0.043) were associated with a higher incidence of pouchitis. The duration of follow‐up was the only variable associated with acute pouchitis (P = 0.007). The presence of backwash ileitis and PSC were independent risk factors for chronic pouchitis (OR, 5.9; P < 0.001; OR, 2.8; P = 0.001 respectively). Conclusion  Pouchitis is a heterogeneous disease which tends to occur early after restoration of gastrointestinal continuity. Patients with backwash ileitis and/or PSC are at considerable risk of developing chronic pouchitis. The strong association between backwash ileitis, PSC and chronic pouchitis suggests a common link in their pathogenesis.     

Anal Function During Pregnancy and Postpartum After Ileal Pouch Anal Anastomosis for Ulcerative Colitis

Purpose Total proctocolectomy and ileal pouch anal anastomosis (IPAA) is now a common procedure for ulcerative colitis (UC). Moreover, it has been reported that fertility is preserved in women who undergo this procedure, many of whom can experience normal delivery.Methods We assessed the perinatal outcomes and changes in anal function in four women, aged from 27 to 35 years, who gave birth after undergoing IPAA for UC. Pregnancies were confirmed 15–111 months after IPAA, three women had full-term pregnancies, and one gave birth after 31 weeks gestation.Results Cephalic delivery with a vacuum extractor was used for one patient, and another underwent cesarean section. The other two patients experienced normal delivery. None of the patients needed artificial insemination and none had any symptoms of intestinal obstruction during their pregnancy. After delivery, two patients suffered a transient increase in bowel frequency and soiling, which gradually resolved.Conclusions Although transient anal dysfunction occurred in the puerperal period, there were no changes in anal function during pregnancy. Therefore, there are no factors directly prohibiting pregnancy and vaginal delivery in women who undergo IPAA for UC.