Long-term follow up (37–69 years) of patients with bladder exstrophy treated with ureterosigmoidostomy: Uro-nephrological outcome

Abstract

Objective

To describe the urological and nephrological long-term outcome of patients born with classical bladder exstrophy treated with bilateral ureterosigmoidostomies in early childhood.

Patients and method

Out of 42 patients born with bladder exstrophy in Switzerland between 1937 and 1968, 25 participated in this study; seven had died, seven were lost to follow up and three refused consent. Assessment included chart review, clinical examination, and assessment of renal function and morphology.

Results

After a follow-up period of 37–69 years ((mean 50 years), 13 of the 25 participants (52%) had their ureterosigmoidostomy still in place. All others had different forms of urinary diversions. Fifteen (60%) patients had normal renal function or mild chronic kidney disease as assessed by estimated glomerular filtration rate. Three patients were on renal replacement therapy. MRI (n = 16) showed 10 morphologically normal kidneys. One patient suffered from adenocarcinoma of the colon, five had benign colonic polyps, one urethral papillary carcinoma and 18 no evidence of tumor.

Conclusion

The majority of our patients have normal or mildly impaired renal function and a well functioning ureterosigmoidostomy. This is remarkable, given the fact that ureterosigmoidostomies are considered to be refluxing high-pressure reservoirs at risk of renal injury and malignancy.

Introduction

Most infants with classical bladder exstrophy undergo early reconstructive surgery. The goals of this treatment are extremely ambitious, including preservation of normal renal function, urinary continence, volitional voiding, normal sexual function and good cosmesis. However urinary continence with normal voiding cannot be achieved reliably and success rates in recent large international case series vary widely from 21% [1] to 70% [2] and 83% [3]. Despite numerous surgical procedures related to staged reconstructions, many patients cannot achieve normal micturition and ultimately need some form of diversion to achieve continence [2], [4]. Furthermore, bladder neck reconstruction has inherent risks such as recurrent pyelonephritis [5], bladder stones and bladder perforation [3], [6], [7]. Realistically, the results achieved in most centers differ from the goals of modern reconstructive surgery substantially. Yet, the outcome after reconstruction is hardly ever compared to other options such as primary urinary diversion.

Today, only patients with failed exstrophy reconstructions or bladder plates considered hopeless for reconstruction, and patients with additional problems such as being born in developing countries, will occasionally be treated by primary diversion. Little is known about the long-term outcome and quality of life of patients born with bladder exstrophy treated with early ureterosigmoidostomy (USS) in a country with excellent medical care for all. The aim of this study is to analyze the urological outcome of patients born in Switzerland with bladder exstrophy, with special emphasis on renal function, continence and complications. The psychosocial and psychosexual outcome as well as sexual function and fertility are beyond the scope of this study, but will be described elsewhere.