Introduction: The Exstrophy-Epispadias complex is a spectrum of rare congenital malformations that can involve multiple organ systems. It can range from a simple glandular defect to a complete multisystem malformation with cloacal exstrophy and musculoskeletal diastasis. We report on the case of an adult woman who was born with an exstrophy variant with bladder and urethral duplication in the sagittal plane. The aim of our study was to report our reconstructive approach in this rare case of bladder exstrophy with maximum use of available tissues
Methods: A 27-year old female patient was referred to our center for the complaint complete incontinence since birth. Upon first clinical examination, we discovered an exstrophic bladder with blind ending ureteral orifices. Further evaluation revealed a urethral meatus caudal to the exstrophic bladder plate. Cystoscopy revealed a second non-exstrophic bladder in the sagittal plane with complete incontinence due to a broad open bladder neck. This bladder contained 2 orthotopic ureters with normal retrograde urethrography. Vaginoscopy revealed a short and small vaginal channel and an arcuate uterus. Plain radiography of the pelvis showed a diastasis of 5 centimeters. CT scanning could not reveal any aberrant anatomy in the upper urinary tract. Genetic testing showed a normal 46XX karyotype.
A laparotomy incision is performed and the exstrophic bladder plate is dissected caudally up to the point where a ventral inlay in the non-exstrophic bladder can be performed as augmentation tissue. A Mitchell-type bladder neck reconstruction with fascia sling interposition is performed. Since we do not know if this non-exstrophic bladder will be capable of emptying or if the bladder neck reconstruction will remain patent, we chose for the interposition a continent Mitrofanoff-type vesicostomy. Genital reconstruction with re-anastomosis of the urethral meatus between the inner labia is performed. The patient was thought self-catheterization through the appendiceal channel. At this point the patient can hold around 250 ml in the bladder. She catheterizes 5 times a day and once at night. She is completely dry.
Results: We present our reconstructive technique in a step-by-step-video guide. Our main focus was to achieve continence by maximal use of available tissues with the best possible quality of life for the patient.
Conclusions: No surgical technique will be suitable for all these varying anomalies. Therefore, reconstructive surgeons need to tailor their approach based on available tissues and functional goals.