Initial bladder closure of the cloacal exstrophy complex: Outcome related risk factors and keys to success

Purpose

This study examines a large single-institution experience with cloacal exstrophy patients, analyzing patient demographics and surgical strategies predictive of bladder closure outcomes.

Methods

One hundred patients with cloacal exstrophy were identified. Complete closure history including demographics, operative history, and outcomes was available on 60 patients. Twenty-six patients with a history of failed initial bladder closure were compared to 34 with a history of successful initial bladder closure. Univariate logistic regression analysis was used to compare the two groups.

Results

Median follow up time after initial closure was 9 years (range: 13 months-29 years). A 1 cm increase in pre-closure diastasis resulted in a 2.64 increase in the odds of initial closure failure (p = 0.004). Protective strategies against failure included delaying closure (per month) (OR = 0.894, p = 0.009), employing pelvic osteotomies (OR = 0.095, p < 0.001), and applying external fixation (OR = 0.024; p = 0.001). Among patients who underwent osteotomy (31% of patients in the failed group, 82% in the successful group), a longer delay between osteotomy and closure (OR = 0.033; p = 0.005) was also protective against failure.

Conclusion

Patients with a large diastasis are more likely to fail initial closure. Delaying initial closure for at least 3 months, performing pelvic osteotomy, and using an external fixation device post-operatively are strategies that improve closure success.     

The failed bladder closure in cloacal exstrophy: Management and outcomes

PurposeCloacal exstrophy (CE) is the most severe presentation of the Exstrophy-Epispadias Complex (EEC) and is associated with an omphalocele, making the bladder and abdominal wall closure difficult. If the bladder closure fails, a secondary closure is necessary. The objective of this study is to identify patient or surgical factors associated with a successful secondary closure.MethodsThe institution's EEC database was reviewed for CE patients between 1975 and 2015. Inclusion criteria included a failed primary bladder closure with a secondary closure. Patient demographics, surgical factors and outcomes of the secondary bladder closure were reviewed.ResultsTwenty-four patients met inclusion criteria. 8/8 patients had a successful two-staged closure at the author's institution (100%); 2/16 patients had a successful closure at an outside institution (12.5%). Older median age at secondary closure was associated with outcome, p = 0.045. Pelvic osteotomy was associated with successful secondary closure, p = 0.013. Using Buck's immobilization with external fixation was associated with a higher proportion of successful secondary closures compared to Spica cast, p = 0.012.ConclusionSuccessful reclosure in CE patients is associated with the use of osteotomy as well as Buck's immobilization with external fixation. While successful reclosure can be achieved, it is often at the cost of multiple procedures and, therefore, all efforts should be expended to achieve a successful primary closure.Type of Study: Prognostic.Level of Evidence: III.     

Bone health in patients with cloacal exstrophy and persistent cloaca after bladder augmentation

Purpose

In this study, the prevalence of osteoporosis was evaluated in patients operated on for cloacal exstrophy or persistent cloaca and who had undergone bladder augmentation.

Methods

Seven patients operated on because of cloacal exstrophy and 3 patients operated on because of persistent cloaca were evaluated. Patients were clinically assessed. Areal bone mineral density for the lumbar spine (L1-L4), femoral neck, and whole body was measured with dual-energy x-ray absorptiometry. Acid-base balance, plasma 25-hydroxyvitamin D (vitamin D), and gonadal function were determined and correlated with dual-energy x-ray absorptiometry results.

Results

The patients were small in height (median, −2.2 SD). According to areal bone mineral density measurements, 40% of the patients had significantly reduced BMD. When the values were adjusted for patient size, only 1 patient had slightly reduced bone mass. Half of the patients had low vitamin D values. The height-corrected bone mineral content values showed a positive correlation with vitamin D values (P = .0072) but were not different in patients with and without hypogonadism (P = .33).

Conclusions

Patients with cloacal exstrophy or complicated cloaca have many risk factors for osteoporosis. However, this study showed good overall bone health in affected adolescents.     

Pelvic and lower extremity immobilization for cloacal exstrophy bladder and abdominal closure in neonates and older children

Introduction

Successful bladder closure in cloacal exstrophy (CE) is best accomplished through a multidisciplinary team and attention to pre- and postoperative technique. This study from a high volume exstrophy center investigates outcomes and complications of primary and reoperative bladder closures in patients immobilized with spica cast or patients with external fixation (EF) and skin traction.

A neo-urethral enteroplication for urinary continence in a case of cloacal exstrophy

A four-year-old female, born with cloacal exstrophy, returned for evaluation of her urinary incontinence two years after her initial repair. Her bladder had been turned in at her first operation and had a capacity of approximately 15 cc. Her ureters exited close to the reconstructed introitus. At reoperation, her bladder measured 3.5 cm in length and appeared to be well healed and of normal consistency. An anterior wall bladder flap was created which was based at the dome and was reflected 180 degrees cephalad. The bladder was then tubularized over a #10 french catheter to create a "neo-urethra" of 6 cm in length. Both ureters were reimplanted into an intestinal conduit, constructed 18 cm long, in a nonrefluxing fashion. The distal, oversewn, end of the conduit was sewn down into the pelvis, at the level of the bladder neck and posterior to the "neo-urethra." The end of the neo-urethra was anastomosed to the anterior wall of the intestinal reservoir, which was then plicated circumferentially around the neo-urethra in a manner very similar to the construction of a Nissen fundoplication for gastro-esophageal reflux. Pressure in the reservoir serves to keep the neo-urethra closed so that urine cannot escape until the neo-urethra is intubated. She is currently continent of urine with a successful program of clean intermittent catheterization. Her upper tracts remain undilated and are free of intestinal-ureteral reflux. Her urine is sterile.     

Achieving urinary continence in cloacal exstrophy: The surgical cost

Introduction

Cloacal exstrophy (CE) is a severe midline congenital abnormality that requires numerous surgical corrections to achieve an acceptable quality of life. Candidates for urinary continence undergo multiple procedures, most often continent bladder diversions, to become socially dry. Here, the authors investigate the number of genitourinary interventions that patients with CE undergo to attain urinary continence.

Female bladder exstrophy

Bladder exstrophy is one of the most challenging congenital urinary tract abnormalities. Apart from the open bladder the patient also has various other abnormalities, including urogenital, musculoskeletal and anorectal defects. The size of the exstrophic bladder varies from patient to patient. In the female the clitoris is bifid and the vagina is anteriorly placed. In this paper various aspects of female bladder exstrophy are reviewed in detail, including incidence, etiology, anatomy and clinical features, together with early, medium-term and long-term surgical management. The aim is to achieve a functional bladder closure, although some patients are better off with a urinary diversion. Considering the complexity of the urogenital problems and the surgical management thereof, all patients require lifelong follow-up.     

Gastrointestinal ramifications of the cloacal exstrophy complex: a 44-year experience

Purpose

Cloacal exstrophy is a rare and complex congenital anomaly requiring coordination among multiple pediatric subspecialties. There is currently no consensus regarding the fate and function of the hindgut, which plays an integral role in patients' long-term gastrointestinal health and genitourinary reconstruction.

Methods

A retrospective chart review was performed evaluating 77 patients with cloacal exstrophy treated during the previous 44 years at our institution.

Results

Seventy-seven patients with cloacal exstrophy were treated between 1965 and 2008. Sixty-five were white, 6 were African American, 3 were Asian, and 3 were Hispanic. Genotypes included 44 XY, 32 XX, and 1 XYY. Fifty-one were reared as females and 26 as males. The hindgut length was 2 to 5 cm in 11 patients, 6 to 10 cm in 18 patients, 11 to 15 cm in 6 patients, 16 to 20 cm in 7 patients, and greater than 20 cm in 2 patients. The hindgut length was unknown in 33 patients. Forty-seven patients had tubularization of the cecal plate with an end colostomy, and 30 patients had an ileostomy placed for bowel diversion purposes. Four patients had short gut syndrome. Thirty-one patients had genitourinary reconstruction, 12 using small bowel and 19 using colon. Eight patients had hindgut pull-through procedures.

Conclusion

Gastrointestinal ramifications of the cloacal exstrophy complex include the occurrence of short gut syndrome and significant fluid and electrolyte derangements in patients receiving an ileostomy for initial intestinal management. This has caused a paradigm shift of initial intestinal management to tubularization of the cecal plate with end colostomy placement. This shift has eliminated the occurrence of short gut syndrome and enabled patients to be candidates for intestinal pull-through procedure if these patients are able to form solid stool, have a reasonable degree of pelvic neuromuscular development, and are able to comply with a bowel management program.     

Repeat pelvic osteotomy in patients with failed closure of bladder exstrophy: applications and outcomes

Background/Purpose

In patients with failed primary or secondary closure of bladder exstrophy, repeat osteotomy is useful in facilitating reconstruction. The clinical consequences of repeated surgical disruption of the pelvic ring have not been carefully described, however.

Methods

We reviewed our experience with exstrophy patients who had undergone repeat pelvic osteotomy (RPO) and analyzed patient history, complications, and orthopedic outcomes.

Results

Fifty-six patients who underwent RPO were identified. All had previously failed at least one attempted bladder closure. The patients underwent RPO at a mean age of 23.2 months. The mean time from initial osteotomy to RPO was 20.5 months. Anterior innominate or combined iliac/innominate approaches comprised 80% of RPO procedures. Of the patients, 95% had a normal gait after RPO; all 3 patients with an abnormal gait had osteotomy site nonunion, which was treated with bone grafting. Five patients had local fixator pin site infections, which were managed with local care and oral antibiotics, and 1 patient had late osteomyelitis requiring incision and drainage. No patient had femoral or sciatic nerve palsy after RPO at our institution.

Conclusions

Orthopedic complications after RPO are uncommon, and most patients have a normal gait postoperatively. Repeat pelvic osteotomy is useful in the complex reconstruction of failed exstrophy closures, and few cases fail reclosure when the reconstruction is combined with RPO.     

Fate of the uterus in 46XX cloacal exstrophy patients

Purpose

Genetically female cloacal exstrophy (46XX CE) patients develop complications later in life due to their abnormal uterine anatomy, resulting in various invasive gynecologic procedures. Furthermore, they have difficulty becoming pregnant, and if they do conceive, they are unlikely to carry the pregnancy to term. We performed this review to determine the rate of gynecological complications, the fate of the uterus, and the rate of pregnancy in 46XX cloacal exstrophy patients.

Methods

All charts for 46XX CE patients treated by us were reviewed following IRB approval. Patient age at last follow-up, surgical management of the uterus, uterine complications, and pregnancies were recorded.

Results

The charts of all 16 of our 46XX CE patients who survived past the neonatal period were reviewed. Two patients underwent hemi-hysterectomy (HH): 1 for an atretic hemi-uterus at birth, another for abnormal uterine insertion at 3 years. A third patient initially had HH for hydrometrocolpos leading to ureteral and colonic obstruction at 14 years but she required a completion hysterectomy a year later. Four patients underwent total hysterectomy (TH) at the outset: 2 neonates for a diminutive uterus with extreme disparity in the halves, another for uterine prolapse at 1 month of age, and a fourth for hematometrocolpos at 16 years of age. Six patients reached adulthood without requiring gynecologic intervention; one of these six is now being managed at another institution. Two patients are prepubertal and one was lost to follow-up. The only patient in the series who became pregnant miscarried at 11 weeks gestation.

Conclusion

Out of 13 post-pubertal patients 6 have retained the entire uterus and another 2 had a hemi-hysterectomy. One patient who became pregnant miscarried at 11 weeks gestation. We believe it is appropriate to avoid ablative genital surgery as far as possible and for these patients to become pregnant after detailed discussion with physicians experienced in the care of 46XX CE patients.     

Vaginal reconstruction using urinary bladder flap in a patient with cloacal malformation

We report a case of vaginal reconstruction using a flap from urinary bladder in a young girl. This girl was born with cloacal malformation and hemivaginas connected to the urinary bladder. Repeated urinary tract infection and vesicoureteral reflux were noted. At the age of 9 months, she received posterior sagittal anorectoplasty for rectum pull-through, but the urogenital part was not corrected. She had repeated urinary tract infection. Detrusor areflexia and large bladder volume were demonstrated by cystometry. Cystoscopy showed a common channel longer than 3 cm. Urogenital reconstruction was performed at 14 months of age. A part of the urinary bladder wall, which was connected to the vaginas, was used to lengthen the vagina so that the latter was able to pull down to the perineum. The patient received vaginal dilatation and intermittent catheterization after the surgery.     

Overview of bladder exstrophy: a third world perspective

Introduction

Bladder exstrophy is an uncommon condition, and the ramifications for patients living in a Third World environment are extensive.

Patients and Methods

This was a retrospective study of 57 patients, managed between 1983 and 2006. The review looked at the home environment, clinical findings, treatment provided, and short-term outcome.

Results

Of the 57 patients, 19 were born in metropolitan and 38 in rural hospitals. Twenty-four patients (42%) were referred on the first day of life, 21 (37%) within the first week, and 12 patients (21%) were older than 1 week of life. Associated congenital anomalies were seen in 18 patients (32%). Twelve patients died in hospital, the remaining 45 were discharged. Thirty three patients returned for review; their ages ranged from 2 to 17 years (median, 3 years). The overall mortality was 42%. On follow-up, urinary incontinence was the main complaint, which greatly affected the child's schooling and social life.

Conclusion

Bladder exstrophy is an uncommon condition. The associated congenital abnormalities together with the impoverished environment result in a poor prognosis. Antenatal screening, early referral, and establishing urinary continence are factors that will improve the outcome in children with this condition in a Third World environment.     

Augmentation ureterocystoplasty in bladder exstrophy: 5-year follow-up in two cases

OBJECTIVE: To report two cases of bladder exstrophy managed successfully by augmentation ureterocystoplasty (UCP) together with bladder neck surgery and continent diversion. PATIENTS: Two boys, age 5 and 1 years respectively, had augmentation UCP. The left refluxing megaureter was used in the first case together with bladder neck reconstruction. In the second patient, bilateral obstructed magaureters were used in tandem together with bladder neck division. Both patients had appendicovesicostomy according to Mitrofanoff. RESULTS: In follow-up for over 5 years, both patients are continent with improved upper urinary tracts and normal or stable renal function. They are managed by clean intermittent catheterization (CIC) via the continent stoma with bladder capacities of 220 cc and 150 cc, respectively. Cystogram showed no vesicoureteric reflux (VUR). The procedures were considered to be successful although one patient was on oxybutinin because urodynamics showed high intravesical pressures. CONCLUSIONS: Augmentation UCP should be considered in patients with bladder exstrophy when a suitable megaureter is available. This may be combined with simultaneous bladder neck reconstruction together with a continent diversion.     

Partial umbilical exstrophy with cecal patch and intact hindgut entering a cloaca: a new variant

Variants of cloaca and exstrophy are difficult to explain and manage. We describe a female neonate having partial umbilical exstrophy with a cecal patch in the bladder and an intact distal hindgut that was expressed as a cloaca. The baby was managed by ileostomy and bladder closure without separating the cecal patch from the bladder. The possible embryological origin of this abnormality is discussed, and the literature is reviewed.     

Standing the test of time: long-term outcome of reconstruction of the exstrophy bladder

The surgical management of classic bladder exstrophy has evolved over time. Different techniques are used to address the challenge of reconstructing these patients. We review the long-term outcomes of bladder exstrophy treatment from the published literature with regard to urinary continence, voiding and secondary complications. Continence now can be achieved in up to 80% of children in specialist centres. Whether such success can sustained into adult life is uncertain. About 40% of adults are dry in the best hands. Up to 84% of children can void, but there is some evidence that this function is lost with time in 70%. The need for bladder augmentation is widely variable between series, reported in 0–70% of children. This reduces the ability to void spontaneously to about 50% of children. It brings with it the later risk of metabolic disturbance and stone formation. Adults with exstrophy have a 694-fold increase in the risk of bladder cancer by the age of 40 years.     

An unusual form of duplicate bladder exstrophy

Duplicate bladder exstrophy is a rare variant of the exstrophy/epispadic lesions in which an external patch of exstrophic bladder presents in addition to a closed bladder in a more normal anatomical position, diastasis of the pubis and rectus muscles, and often a form of epispadias. We describe an anomaly of this variant with a fistula to the closed bladder. Careful examination with fluoroscopy and cystoscopy is helpful in defining the anatomy in these variants.     

Reconstruction of bladder and urethra using ileocecal segment and appendix in patients with exstrophy-epispadias complex: the first report of a new surgical approach

Objective We aimed to describe our experience in administering an innovative surgical technique to treat pediatric cases of exstrophy-epispadias complex. Material and methods Between 1995 and 2004, seven consecutive patients (six males) with exstrophy-epispadias complex were treated using ileocecal segment for bladder augmentation and appendix for urethral reconstruction. In a single-stage operation, the exstrophied bladder was dissected- and a segment of cecum, ascending colon, terminal ileum, and the corresponding appendix were isolated. Using the opened colon to augment the bladder, the ileal segment was fashioned to skin as temporary stoma, and the appendix was laid in the urethral lumen as the neourethra following urethral demucosation. This technique was used as a secondary surgery in one case and as a primary surgery in six neonates. Evaluation of the urinary tract status was performed by cystograms and ultrasonograms. Results Renal function was saved in all cases and continence was achieved by clean intermittent catheterization every two hours either via the neourethra (n = 6) or through the temporary stoma (n = 1). The patients did not experience any metabolic complications in their follow-ups. Moreover, no one had vesicourethral reflux, dehiscence, or fistula. Conclusions The technique was deemed safe with acceptable outcomes even when secondary repair of previously failed operation was intended. Experiencing the technique in larger cohorts as well as longer follow-ups might be necessary to assess probable long-term complications.     

Impact of pelvic osteotomy on the incidence of inguinal hernias in classic bladder exstrophy

Background/purpose

The high prevalence of inguinal hernias in the bladder exstrophy population is well documented. The authors' aim is to determine whether pelvic osteotomy reduces the incidence of primary and recurrent inguinal hernias in patients with classic bladder exstrophy.

Methods

Using an institutionally-approved database, patients who underwent immediate or delayed primary bladder closure between 1974 and 2012 were identified and stratified by the use of pelvic osteotomy at the time of closure. Data were analyzed using Fisher's exact test and multivariate logistic regression analysis.

Results

One hundred thirty-six patients were identified with a median follow up of 8 years. The incidence of inguinal hernias following closure was 25% in the osteotomy group versus 46% in the non-osteotomy group (p = 0.017). Osteotomy was associated with a significant decrease in recurrence of inguinal hernias amongst patients who underwent previous repair (17% versus 47%, osteotomy versus non-osteotomy, p = 0.027) and the development of primary inguinal hernias in whom initial groin exploration was negative (20% versus 39%, p = 0.029). Osteotomy and female sex were associated with a decreased rate of inguinal hernia development after bladder closure while age at closure was not.

Conclusions

Pelvic osteotomy at the time of exstrophy closure decreases the likelihood of primary or recurrent inguinal hernia development.