José Figueroa Díaz, MD
Manuel Pizarro-Mondesir, MS
Christopher Batista-Fernández, MS
Marcos Pérez-Brayfield, MD

University of Puerto Rico, Medical Sciences Campus, School of Medicine, Department of Urology, San Juan, Puerto Rico
Universidad Central del Caribe School of Medicine, Bayamón, Puerto Rico

Introduction

The bladder exstrophy-epispadias complex is one of the most severe congenital anomalies affecting the kidney and urinary tract. This condition occurs in about 1 in 50,000 live births. The primary objective of bladder exstrophy (BE) repair includes preserving renal function, achieving urinary continence, ensuring adequate sexual function, and satisfactory aesthetic results.

Methods

We present the case of a three-month-old male born with bladder exstrophy. Management options include immediate surgical correction during the newborn period or delaying surgery. Given the patient’s weight and size, we opted to delay the surgical intervention. The patient was scheduled for surgical intervention at 3 months of age, with a weight of 4 kg.

Results

The patient had an epispadic obliterated urethra and an orthotopic meatus. Cystoscopy revealed a normal orthotopic urethra with adequate bladder entry and an hourglass bladder deformity. An 8 Fr feeding tube was inserted through the urethra into the bladder. Dissection of the bladder and transection of the intersymphyseal ligaments allowed for adequate mobilization. The anterior bladder wall defect was extended to the bladder dome, and bilateral ureteral orifices were cannulated with 5 Fr feeding tubes. The bladder defect was repaired, and ureteral feeding tubes, along with an 8 Fr Foley catheter, were placed through the right anterolateral bladder wall. A neoumbilicus was created.

Conclusion

Classic bladder exstrophy is a rare urologic condition occurring in approximately one per 50,000 live births, and it is usually associated with epispadias. Surgical correction can be done during the newborn period or can be delayed. This patient had an unusual finding of a duplicated urethra, including a normal orthotopic urethra with a normal bladder neck. The rare variations of bladder exstrophy seen in this patient may represent an increased possibility of developing adequate bladder function and achieving continence without requiring surgical interventions in the future.