Predisposing syndromes associated with an increased risk of Wilms tumor (WT) are responsible for 9-17% of all cases of the malignancy. Due to an earlier age at WT diagnosis and an increased incidence of bilateral and metachronous disease, management of syndromic WT warrants a distinct approach from that of non-syndromic WT. This review of English-language manuscripts about WT focuses on the most common syndromes, surveillance protocols and current treatment strategies. Highlighted syndromes include those associated with WT1, such as WAGR (Wilms-Aniridia-Genitourinary-mental Retardation), Denys-Drash syndrome (DDS), and Frasier syndrome, 11p15 defects, such as Beckwith-Wiedemann syndrome (BWS), among others. General surveillance guidelines include screening renal or abdominal ultrasound every 3-4 months until the age of 5 or 7, depending on the syndrome. Further, some of the predisposing conditions also increase the risk of other malignancies, such as gonadoblastoma and hepatoblastoma. With promising results for nephron-sparing surgery in bilateral non-syndromic WT, there are increasing reports and recommendations to pursue nephron-sparing for these patients who are at greater risk of bilateral, metachronous lesions. In addition to the loss of renal parenchyma from malignancy, many patients are at risk of developing renal insufficiency as part of their syndrome. Although there may be some increase in the complication rate, recurrence free survival seems equivalent. Some conditions require specialized approaches to adjuvant therapy, as their syndrome may make them especially susceptible to side effects.
INTRODUCTION AND OBJECTIVES: Anastomotic urethroplasty is an effective but occasionally controversial treatment for short bulbar urethral strictures. Non-transecting variations of anastomotic urethroplasty were created in part to address this controversy. The objective of this study is to assess current outcomes of anastomotic urethroplasty and compare outcomes of transecting and non-transecting techniques.METHODS: 171 patients with complete follow-up underwent anastomotic bulbar urethroplasty from September 2003 to May 2016. Patient age, stricture length, location, etiology, 90-day complications and semi-quantitative assessment of sexual dysfunction were recorded. The primary (objective) outcome was success defined as urethral patency >16Fr on routine follow-up cystoscopy. Secondary outcome measures included 90-day complications (Clavien !2) and de novo sexual dysfunction assessed at 6 months. Statistical comparison between transecting and non-transecting cohorts was made using Cox Regression Analysis and Chi-square when appropriate.RESULTS: One hundred and thirty patients underwent transecting anastomotic urethroplasty while 41 had a non-transecting anastomotic urethroplasty. Mean stricture length was 1.5AE0.5cm (range 1-3) with a mean patient age of 43.0AE18.0 years. 78.9% of patients failed prior endoscopic treatment (135/171) and 2.4% failed prior urethroplasty (4). Overall there was a 98.2% (168/171) success rate with a mean follow-up of 74.9(AE46.7) months. 7.0% (12/171) of patients experienced a 90-day postoperative complication of Clavien !2 including 2.9% wound-related complications (5), 1.8% scrotal hematomas (3), 1.8% UTI (3), and 0.6% urethral bleeding (1). 9.9% reported an adverse change in sexual function including 6.4% erectile dysfunction (11), 1.8% ejaculatory dysfunction (3), 1.2% painful erection (2), and 0.6% chordee (1). When comparing transecting and non-transecting technique success using Cox Regression analysis there was no difference in urethroplasty success (97.7% vs. 100%; p¼0.63) and no difference in postoperative complications (7.7% vs. 4.9%; p¼0.73) but patients undergoing transecting anastomotic urethroplasty were more likely to report an adverse change in sexual function (13.1%; vs. 0%; p¼0.013).CONCLUSIONS: Anastomotic urethroplasty remains a highly effective treatment for short-segment bulbar urethral strictures with relatively minimal associated morbidity. Newer non-transecting anastomotic urethroplasty techniques appear to compare favorably in the short-term and may reduce the risk of associated sexual dysfunction.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.