Bladder atrophy is a rare congenital condition estimated to affect up to 1 in 25,000 live infants. The condition is the result of an abdominal wall defect secondary to an aberrant division of the cloacal membrane by the urerectal fold. The abdominal wall defect forms early in the fetal development and leads to abnormalities in the bladder, abdominal wall, pelvic floor, and bony pelvis. As a consequence of the altered pelvic anatomy, women with bladder atrophy are predisposed to developing pelvic organ prolapse. The objectives of this video are to describe the changes in the pelvic anatomy associated with the bladder atrophy and how these changes predispose to pelvic organ prolapse, and to discuss the challenges of reconstructive surgery in bladder atrophy patients. We will begin by demonstrating the anatomy of the bony pelvis associated with the bladder atrophy. Anteriorly, the pelvis and pubis are rotated outward by an average of 18 degrees. The pubic bones are shortened by about 30%, forming a diastasis of an average 4 cm. Posteriorly, the pelvic width is increased by 30%, the acetabulum are typically retroverted, and the iliac wings are externally rotated by an average of 12 degrees. The sacrum is also typically larger with 24% more surface area as compared to a normal pelvis. The pelvic floor musculature is affected by the deformation in the bone structure. Specifically, the levator ani muscles, comprising the strength of the pelvic floor, are significantly altered. The levator ani muscle complex is flattened and the muscle mass is decreased anteriorly. With the anus displaced, the anterior portion of the levator is shorter, leaving a gaping levator hiatus. In summary, the bony pelvis and pelvic musculature are significantly altered in bladder atrophy. The pelvis is foreshortened, widened, inferiorly and laterally rotated, with abnormalities in the sacrum, iliac wings, and pubic rami. The levator ani muscle complex is more flattened, outwardly displaced, and with decreased muscle mass anteriorly. In addition, the vagina may be shortened and stenotic, and cardinal utero-sacral ligaments have been described to be congenitally weakened. With the advances in surgical and medical management of this condition, women with bladder atrophy have normal life expectancy, sexual function, and achieve pregnancies. Prolapse may present in the teenage years, during pregnancy, as well as later in life. Conservative management with the pessary may be challenging in the absence of orthopedic reconstruction due to the pubic symphysis diastasis. Thus, a pelvic reconstructive surgeon may be faced with the challenge of providing a durable surgical repair for these patients. To illustrate the management of prolapse in patients with bladder atrophy, we present the following case. MG is a 66-year-old newly gravida who presented with stage 4 pelvic organ prolapse. Her past surgical history is significant for ureterosigmoidastomy, multiple abdominal surgeries for small bowel obstruction, as well as abdominal hysterectomy. Ureterosigmoidastomy is one of the correctional procedures for bladder atrophy. It involves a diversion of the ureters into the sigmoid colon. This procedure carries significant risks of nephritis, urinary and fecal incontinence, and colon cancer. Thus, currently, the procedure is not performed, and an ileal conduit is a preferred surgery. Of note, our patient did not suffer from incontinence and was able to spontaneously urinate via directing. Physical examination revealed several abdominal incisions and significant scarring in the area of pubic symphysis. The pubic bones separated by approximately 10 centimeters. The external genitalia included the bifid clitoris and the labia majora. On pelvic examination, a stage 4 pelvic organ prolapse was noted. As part of preoperative evaluation, a CT urogram was obtained. It demonstrated the insertion of the right and left ureters into the sigmoid colon. The patient was consented for abdominal sacrocolopexy and a vaginal rectocele repair. Intraoperative findings included dense bowel adhesions secondary to previous surgeries. As a result, an inadvertent enterotomy was made upon fascial incision. Enterotomy was repaired and side-to-side anastomosis was performed. Once adhesion analysis was performed and enterotomy repaired, we proceeded with sacrocolopexy. On examination of the sacral promontory, the right ureter was identified clearly traversing across the midline prior to its insertion into the sigmoid colon. The ureter coursed superior to the sacral promontory safely away from the operative field. The dissection over the sacral promontory was then carried out, paying close attention to the ureter as well as to other vital structures. The peritoneum overlaying the anterior vagina was then dissected to where the bladder would have been located. Dissection of the posterior vagina was performed as well. Two pieces of polypropylene mesh were then attached anteriorly and posteriorly using permanent suture with six stitches on each side. We then used two separate permanent sutures to affix the two pieces of mesh to the anterior longitudinal ligament, once again paying close attention to the ureter. The peritoneum was closed over the mesh using absorbable suture. Indigo carmine dye was administered as the surgeons closely observed the abdomen and the pelvis for the dye spill. In the absence of the bladder, cystoscopy is not feasible and therefore vigilant examination of the ureters, particularly when the anatomy is altered, is of utmost importance. Gaping vagina is common in bladder atrophy patients, even with a well-supported apex. The rectocele repair and levator myoraphy help achieve a normal vaginal caliber. Post-operative course was uncomplicated. Initially the patient required self-catheterization per rectum. Once bowel function fully recovered, she was able to urinate spontaneously. She was discharged on post-op day 10 and is doing well to date. Significant alterations in pelvic bone and muscular anatomy that are associated with bladder atrophy predispose the patients to pelvic organ prolapse. According to most experts, sacrocopopexy provides the most durable repair. Laparotomy maximizes the visualization of the anatomy likely to be altered secondarily to the condition itself and due to multiple previous surgeries. Laparoscopic and robotic approaches are appropriate in expert hands. Concurrent vaginal procedures may likely be necessary to achieve optimal results. A thorough review of the past surgical history, appropriate imaging, and a multidisciplinary approach is paramount to the successful outcome of reconstructive pelvic surgery in patients with rare congenital anomalies.

bladder atrophy

congenital condition

pelvic organ prolapse

abdominal wall defect

levator ani muscles