Prune-belly综合征(PBS)(附二例报告)

本文报道2例Prune-belly综合征,有腹壁、骨、肌肉以及尿生殖器官的多发畸形。采用输尿管乙状结肠、乙状结肠直肠吻合Roux-Y尿流改道,并修补和加强了腹壁和骨盆底。另1例作了肾盂输尿管的成形术。2例均获得满意效果。     

Sigmoidorectal (Mainz II) pouch for continent urinary diversion in bladder cancer

BACKGROUND: We aimed to assess the feasibility, safety, and outcome of the sigmoidorectal (Mainz II) pouch for urinary diversion in patients with invasive bladder cancer. METHODS: Twenty-nine patients (25 men and four women), aged 65-76 years, who had undergone radical cystectomy and the sigmoidorectal pouch procedure for invasive bladder cancer were included in this study. Postoperative evaluations included metabolic testing, standard laboratory screening, renal ultrasonography, pouchography, and intravenous urography or pouchoscopy when indicated. RESULTS: The median operative time was 175 min. Urine leakage was encountered in two patients (6.8%), deep vein thrombosis in one (3.4%), and ileus necessitating surgery in another one. Two patients developed pyelonephritis due to ureterocolonic stricture, which was treated with antegrade balloon dilatation. No local relapses of bladder cancer were found. All patients were continent during the day, but one patient was occasionally incontinent during the night. In the long term, six patients (20.6%) developed metastatic disease, and five patients (17.2%) died because of cancer-related causes. Overall survival was 100, 96 and 60% at 1, 2 and 3 years after the operation, respectively. The mean survival was 36.8 +/- 1.9 months, which was statistically significantly associated with the M stage (P < 0.001), but not with the T (P = 0.091) or N (P = 0.081) stages. CONCLUSIONS: The sigmoidorectal (Mainz II) pouch seems to be a feasible, safe and effective method for continent urinary diversion. It is able to provide good quality of life, and ensure good overall survival rates.     

A modified sigma-rectum pouch (Mainz pouch II) technique: analysis of outcomes and complications on 220 patients

OBJECTIVE: To evaluate the safety and efficacy of a modified technique for a sigma-rectum pouch (Mainz pouch II). PATIENTS AND METHODS: Between October 1994 and March 2003, a modified Mainz pouch II was constructed in 220 patients (153 men and 67 women, mean age 56.7 years) in a multicentre prospective study. The median follow-up (177 patients, 81%) was 21 (1-84) months. RESULTS: There were no peri-operative deaths; there were early complications in 24 patients, including prolonged ileus in six, pyelonephritis in 17, unilateral ureterohydronephrosis in 11, bilateral ureterohydronephrosis in two, and incipient renal failure in five. Late complications included ureteric implantation-site stenosis (unilateral in eight patients and bilateral in three). In all, 92 patients (52%) needed oral alkalizing medications and potassium supplementation because of hyperchloraemic metabolic acidosis. All but three patients (99%) were continent by day and at night. The mean (sd) voiding frequency was 4.2 (1.6) voids by day and 2.1 (0.5) at night. CONCLUSION: The Mainz pouch II is a simple and reproducible surgical technique, with good results in terms of mortality, morbidity, continence and overall quality of life. The main limitation is malignant transformation of the uretero-intestinal anastomosis. For selected cases, the technique is a good alternative to other types of continent urinary diversion.     

改良Sigma直肠膀胱术14例临床分析

目的：介绍并评价改良Sigma后术的可控性尿流改道临床疗效。方法：14例膀胱、前列腺肿瘤患者接受该手术。取25cm左右乙状结肠折叠后全层切开,再缝合成低袋,低压袋顶端固定在骶岬处,两输尿管末端合并吻合并外翻形成乳头,从低压袋上方引入再植。结果：术后发生直肠阴道瘘1例,经横结肠造口过渡后自愈;1例左肾积水伴上尿路感染,经输尿管顺行扩张后恢复正常,该患者术后3个月发生严重低血钾,经补充枸橼酸钾后治愈。平均随访22.5个月,本组未见尿失禁、上尿路感染。3例肿瘤复发,2例肉瘤复发患者于术后6个月后死亡。结论：改良Sigma 手术费时短,对肠管扰动小,操作简便,术后尿控满意,无上尿路积水、感染及明显酸碱电解质紊乱,明显提高了患者生活质量。     

膀胱全切输尿管结肠,结肠结肠吻合术

近十年来我院首先开展了膀胱全切后再将乙状结肠切断，近端乙状结肠与远端乙状结肠行端侧吻合，再将双输尿管吻合在紧缩的乙状结肠袢上。行尿流改道手术３０例，得到随访者２３例，５年以上生存１３例，除２例逆行感染治愈余者均无逆行感染，无水电解质紊乱和肛门尿失禁等。     

Modified ureterosigmoidostomy (Mainz II): a long-term follow-up

OBJECTIVE: To assess the long-term results in patients treated using a modified ureterosigmoidostomy (Mainz II). PATIENTS AND METHODS: Between 1994 and 1999, 17 patients had their lower urinary tract reconstructed by a ureterosigmoidostomy, modified by reconfiguring the rectum to make a low-pressure reservoir (Mainz II). All patients were followed on a standard protocol. Data were extracted from the database and from a review of the case-notes. In 12 patients the procedure was with a radical cystectomy for carcinoma. Five had a failed conventional ureterosigmoidostomy for bladder exstrophy and therefore proceeded to a Mainz II. The data on continence and complications were retrieved for a retrospective analysis; the mean (range) follow-up was 6.4 (4-8.6) years. RESULTS: Ten of those with bladder cancer and one in the revision group were continent. Two patients in the revision group had sufficiently severe nocturnal incontinence to require conversion to a colonic conduit. Seven of the 17 patients had hyperchloraemic acidosis, one had pyelonephritis and one had renal stones. There were no anastomotic neoplasms. CONCLUSION: The Mainz II has a good outcome if used as the primary procedure. In patients with an existing ureterosigmoidostomy who are incontinent, detubularization of the rectosigmoid alone is unlikely to restore continence.     

Modified Ureterosigmoidostomy

Objective To introduce an operation procedure and evaluate the coutinence diversion results of the modified ureterosigmoidostomy after radical cystectomy. Methods Fourteen cases of bladder cancer or prostate carcinoma were operated on with modified Sigma pouch from Feb, 1998 to Dec, 1999. A longitudinal incision about 25 cm on the sigmoid uall was done to form a low pressure pouch. The vertex of the new pouch was fixed to sacrum. Both ends of ureters were anastomosed side to side and to form a big nipple and inserted into the top of pouch for 2 to 3 centimeters. Results It took about sixty five minutes to create a new low pressure pouch after radical cystectomy. Early complication of was found in two cases postoperatively, and cured with temporary colonostomy. Hydronephrosis and hypokalemia in one patient were cured by percutaneous anterograde ureter dilatation with balloon and oral replacement of potassium salt. A~ patients displayed urinary continence. No symptomatic renal infection or hypercholoraemic acidosis occurred. Conclusion Modified ureterosigmoidostomy is a safe procedure of urinary diversion and provides a big volume, low intravesical pressure pouch. The patients are free from the troublesome urine-bag, intermittert catheterization , and upper urinary tracts are protected effectively. The quality of life is satisfied.     

An initial evaluation of pelvic floor function and quality of life of bladder exstrophy patients after ureterosigmoidostomy

Classic bladder exstrophy is characterized by displaced pelvic floor musculature and significant skeletal and genitourinary defects. A paucity of data exist evaluating long-term pelvic floor function in exstrophy patients after ureterosigmoidostomy. This study is an initial attempt to evaluate the prevalence of urofecal incontinence, pelvic organ prolapse, and overall quality of life in patients who have had ureterosigmoidostomies. Fifty-two individuals who underwent ureterosigmoidostomy between 1937 and 1990 were identified through the Ureterosigmoidostomy Association and the Johns Hopkins bladder exstrophy database and mailed questionnaires approved by the Institutional Review Board (Johns Hopkins). Data were analyzed with SigmaStat 3.0 (SPSS, Inc., Chicago, IL). Eighty-three percent of the subjects responded, with a mean age of 44.4 years (range, 14–73 years) and mean of 40.9 years (range, 14–65 years) after ureterosigmoidostomy. Prevalence of daily urinary and fecal incontinence was 48% (n = 20) and 26% (n = 11), respectively, whereas the prevalence of weekly combined urofecal incontinence was 63% (n = 27). The incidence of pelvic organ prolapse in this cohort was 48% (n = 20). In these patients, a significant risk of urofecal incontinence and pelvic organ prolapse exists. Long-term follow-up studies are needed to understand the role of pelvic floor musculature in this complex birth defect. Presented at the Forty-Sixth Annual Meeting of The Society for Surgery of the Alimentary Tract, Chicago, Illinois, May 14–19, 2005 (poster presentation).     

Colonic carcinoma after ureterosigmoidostomy

Urinary carcinogens promote late malignant transformation of the colon after a ureterosigmoidostomy. An unusual case is presented where, despite the early removal of the latter and hence cessation of urine flow, a colonic carcinoma developed at the site of previous anastomosis. The importance of surveillance of all patients who have undergone this procedure to avoid an iatrogenic cancer is emphasised.