Management of Bladder Exstrophy in Adulthood: Report of 2 Cases

Abstract

Bladder exstrophy is a variant of the exstrophy-epispadias complex, management of which is best started in the neonatal period. In infancy primary reconstruction is increasingly performed with good results. Bladder exstrophy in adults is rare and causes difficulties due to upper tract dysfunction and malignant potential. To our knowledge only 1 case of bladder exstrophy in adulthood has been previously reported.' This patient was treated with a Kock pouch. We report on 2 adults with bladder exstrophy treated with cystectomy and a modified Mainz pouch. in infancy.3 When untreated, this condition has malignant potential.4 F'reviously ureterosigmoidostomy wm the form of urinary diversion performed in these patients. In 1987 Matsuda et a1 reported the first case managed by a continent pouch.' The Kock ileal neobladder overcomes the difEculties associated with ureterosigmoidostomy, that is upper tract infection, hydronephrosis, metabolic and electrolyte disturCASE REPORTS Case 1. S., a 22-year-old woman, was hospitalized for untreated bladder exstrophy. There was no history of intranatal complicating factors and siblings were normal. Examination revealed an exstrophic bladder but no other abnormality. The clitoris was bifid, the labia were separated and the vaginal introitus was normal. Laboratory investigations and excretory urography (Np) were normal. The patient underwent cystectomy and urinary diversion with a modified Mainz pouch.2 The abdominal wall defect was closed primarily. Postoperatively the wound healed well and the neobladder functioned in a continent manner. Microscopy showed hyperemic bladder mucosa with nonspecific focal inflammatory changes. At 18 months of followup the patient has normal urinary parameters and preserved urinary continence. Case 2. G. D., a 35-year-old woman, was hospitalized with disabling continuous wetting from an exstrophic bladder (fig. 1). Prenatal history was normal and there were no siblings. The patient had had 2 normal pregnancies. Examination revealed no other congenital anomalies. Laboratory investigations were within normal limits but blood urea nitrogen and serum creatinine were 85 (normal 15 to 40) and 4.1 mg.% (normal 0.6 to 1.5), respectively. Ultrasonography and IVP revealed bilateral grade I11 hydroureteronephrosis and poorly functioning kidneys. However, glomerular filtration rate was 37 ml. per minute, pH was 5.65 on acidification and there was minimal daily urinary protein loss. The patient underwent cystectomy. A continent ileocecal pouch was fashioned with the appendix used as an external conduit. Bilateral ureteral catheters remained in place during convalescence and rehabilitation due to compromised renal function preoperatively. The abdominal wall defect was closed primarily. The patient accepted neobladder care well. Histopathological examination revealed squamous metaplasia in the mucosa, hyperplastic epithelium with areas of atrophy and ulceration, acute and chronic inflammatory cells, and inflammatory granulation tissue. Within 6 months blood urea nitrogen and serum creatinine levels improved to 35 and 2 mg.%, respectively. Postoperatively IVP demonstrated minimal hydronephrosis and cystography showed adequate neobladder capacity (fig. 2).