Varga G., Pacík D.
Department of Urology, Faculty of Medicine, Masaryk University and Faculty Hospital Brno
Received after revision April 2009
Complete duplication of the urinary bladder and the Urethra
Urodynamics
Pelvic
Pain syndrome
Varga G.
Department of Urology
Faculty of Medicine
Masaryk University and Faculty Hospital Brno
Jihlavská 20
625 00 Brno
Czech Republic
A complete duplication of the urinary bladder and the urethra is a rare congenital anomaly which is often associated with other congenital malformations. There are only few articles in the literature on this rare topic. This anomaly is presented in early age. The authors present the case of a 52-year-old woman in whom this anomaly was detected by laparoscopy from gynaecological indication. After 10 years from the diagnosis of the anomaly she is referred to the Department of Urology.
PID - pelvic inflammatory disease
IVP - intravenous urogram
USG - ultrasonography
ATB - antibiotic
CT - computer tomography
A complete duplication of the urethra and the bladder (CDBU) is a rare congenital anomaly which is often associated with other congenital malformations [2, 4, 7, 8, 10, 11, 12]. There are few articles in the literature on this rare topic [2-13]. A child with such an anomaly should be followed up immediately after the diagnosis to prevent possible complications. Anatomical disorders of the bladder are also associated with functional disorders, which is the potential cause of complications related to this congenital anomaly.
A 52-year-old woman was referred to the Department of Urology from the regional Gynaecology Department where she had been an inpatient for colpitis and had undergone extraction of an intrauterine device (IUD).
In 1996, when she was 43 years old, a duplication of the bladder was diagnosed by laparoscopy for a gynaecological indication. No other malformations were detected. At that time she was experiencing pelvic pain syndrome (PID) manifested by chronic idiopathic abdominal and pelvic pain and recurrent colpitis. These problems led to the indication for laparoscopy. Apparently, the first manifestation of the anomaly was recurrent interstitial bilateral nephritis at the age of 20 years. In spite of the late detection of the anomaly, additional screening including urological examination was not performed at the time. In May 2006, ten years after the discovery of the double bladder, a urological examination was completed during hospitalisation at the Department of Gynaecology as a consequence of painful voiding.
Abdominal ultrasonography (USG) confirmed the duplication of the bladder and showed cystolithiasis in the left bladder. An accidental finding from the abdominal USG was liver haemangioma. A subsequent intravenous urogram (IVP) showed normal excretory function of both kidneys with filling of the two separate bladders, each with one normally located ureter, but on the right side with a saccular dilatation indicating ureterocele.
According to the IVP cystogram, the bladder appeared duplicated rather than as a septum bladder, and the differential diagnosis was focused on this aspect. The urine culture confirmed significant bacteriuria of Streptococcus alpha haemolyticus and Enterococcus species, and antibiotic therapy was started. In the results provided by the Regional Health Institute, there was no mention of a duplication of the urethra. This anomaly was diagnosed by physical examination (Figure 1). The renal scan did not show passage obstruction. The voiding cystourethrogram showed a complete duplication of the bladder in the sagittal plane, with two stones in the left bladder where post-voiding residuum was significant (Figures 2, 3, and 4). However, vesicoureteral reflux was not confirmed by x-ray examination.
After repeated therapy for bacteriuria, cystoscopy was performed under epidural anaesthesia, which showed trabeculisation of the left bladder and ureteric plica with diverticulum at its proximal end. At this time, three calculi of 1.5 - 2.5 cm were detected in the left bladder. Using the intravenous indigo carmine, the ureteric orifice was detected just inside the proximal region of the ureteric plica. The cystoscopic view from the right bladder was dominated by pseudodiverticula, and the ureteric orifice was seen in one of these. The bilateral retrograde pyelography indicated in terms of IVP findings and for clinical purpose showed free passage on the right side, but there was a hypotonic ureter with a cut in the pelviureteral junction on the left side and an image of hydronephrosis. The cystolithiasis was fragmented by electrohydraulic lithotripsy and completely evacuated. In the postoperative period, fever and a positive urine culture were observed. After repeated targeted antibiotic therapy and extraction of the catheters, the patient had urine retention in her left bladder. The patient was discharged from the hospital on the 4th postoperative day after therapy with a permanent catheter, which was extracted during the check-up. The post-voiding residuum was 10 ml in the right bladder and 45 ml in the left bladder. The positive urine culture from the urine of the catheterised left bladder persisted, although the patient did not report any symptoms. During the checkup, we performed a computed tomography (CT) scan of the kidneys and the pelvis, which showed small cysts of 6 mm to 8 mm in the kidneys, and a polycystosis of the liver of 10 mm to 60 mm. The CT of the pelvis confirmed the double bladder and showed thickening of the bladder wall due to a chronic cystitis. We also completed a separate urodynamic examination of both bladders (Figures 5 and 6), whose results are shown in Table 1. During voiding, a low detrusor pressure (Pdet) and a high abdominal pressure (Pabd) were seen in the left bladder, suggesting a low detrusor contractility with an insufficient evacuation bladder ability. The voiding curve was irregular and flat, but the bladder outlet obstruction (BO) was not confirmed.
The residual urine caused by the evacuation failure increases the risk of recurrent infections and formation of bladder stones. The urodynamic parameters of the right bladder, in comparison with the left bladder, were acceptable, and full evacuation was well-kept.
Except for the liver cysts, no other malformations were detected in the abdominal cavity and in the pelvis. At present, the patient is without symptoms, the urine culture of the separately catheterised urethras is free of bacteria, and the post-voiding residuum of 50 ml in the left bladder is acceptable. The evacuation function of the right bladder is complete. The patient is periodically checked as an outpatient.
The urinary bladder and the urethra evolve from the endodermal element of the cloaca during embryogenesis. From the cloaca, the allantoideus duct (allantois) runs in the ventrocranial direction. Laterally in the cloaca, the Wolf's ducts end and later form the ureters. In the 2nd month of embryonic development, the urorectal septum divides the cloaca into a dorsal anorectal sinus and a ventral urogenital sinus. Later in embryonic development, the urinary bladder forms in the urogenital region, as well as the urethra and some of the genital organs. The caudal region of the urorectal septum later connects with the membrane of the cloaca in the 7th week of development. The membrane of the cloaca is divided into a ventral urogenital membrane and a dorsal anal membrane. The primitive urogenital orifice forms by the perforation of the ventral membrane. The membrane of the cloaca coalesces with the urorectal septum to create the perineum. The cranial region of the urogenital sinus forms the vesicourethral basement from which the urinary bladder evolves; a woman's urethra (or the prostate urethra in men) forms from the caudal region [1].
Failure of this developmental programme can lead to anatomical anomalies of the lower urinary tract [2, 8, 11]. The complete duplication of the urethra and the bladder in women is the result of a failure to form the vesicourethral basement of the urogenital sinus [1]. This anomaly, found more commonly in males than in females [12], is not only a latent "cosmetic" defect, but results in functional defects which may manifest clinically as pelvic pain disease (PID).
From the cases with CDBU reported in the literature, we illustrate the oldest patient in whom the anomaly was initially detected (Table 2).
This rare anomaly requires follow-up from the time of the first diagnosis because of the late complications that can result from bladder function failure, particularly evacuation of the bladder. In this time of neonatal ultrasound screening, this anomaly should not escape our notice in newborns.
