In the past, it was not uncommon that exstrophy patients experienced renal failure early in life, secondary to reflux and recurrent infections. The rates of renal dysfunction and scarring have been reported as high as 25 % in adults with exstrophy [7, 8]. In the modern era of exstrophy repair in regions near centers of excellence, most patients are closely followed and renal replacement therapy is a rarely needed and studies show minimal effect on renal function; however, it is important to continue to monitor renal function annually [9]. There remains no standard for follow-up of renal function, but most use routine serum creatinine and BUN as well as noninvasive imaging of the kidneys to follow renal function. A decrease in renal function may indicate reflux nephropathy, incomplete bladder/conduit/pouch emptying, or damage from repeat infections and should warrant a workup. This workup may include serum electrolytes, other methods of estimation of GFR, urodynamics, voiding cystourethrogram, and renal scanning when indicated.

It is essential to understand the type and timing of operations that have been performed throughout the patient’s lifetime. The exstrophy bladder is not the same as the normal bladder; therefore, it may not grow at the same rate as normal with age or have the same elastic properties that allow for low-pressure storage. As such, many of these individuals may require incorporation of a bowel segment to decrease intravesical storage pressures and increase capacity. After augmentation cystoplasty, renal deterioration can occur with normal bladder pressures secondary to a phenomenon described as volume-dependent obstruction, which is obstruction caused by exceeding a set volume in the bladder, which decreases the ability of the kidneys to drain [10].

Incorporation of bowel with bladder mucosa has been linked to increased risk of bladder cancer. Malignancy has been reported in the gastric portion of bladders augmented with stomach [11], which differs from small bowel and colon augmentation, where malignancy is traditionally not found on the bowel segment but in the bladder itself [12]. The etiology of malignancy in augmented bladders remains to be elucidated although these cancers typically present at a higher stage and have a shorter survival, when compared bladder cancers that occur in non-neurogenic and nonaugmented cancers [11]. Studies suggest that the lifetime risk of malignancy in these patients is as high as 4.5 %; however, many have coexisting risk factors [13]. Adults with bladder exstrophy have a 17.5 % risk of developing a malignancy, which is about 700 times greater than the normal population [14]. There is no conclusive evidence that bladder augmentation or the use of a separate bowel segment into the urinary system is an independent risk factor for malignancy, as nonaugmented neurogenic bladders also demonstrate elevated risk of malignancy. Annual or biennial screening 10–15 years after augmentation with cystoscopy, urine cytology, and renal ultrasounds is recommended for these patients to detect early lesions and decrease the chance to presenting with advanced cancer [11]. Recent studies suggest that surveillance of these bowel segments in patients with congenital anomalies may be increasing the cost of health care without much benefit to these patients. The sensitivity of cytology is unknown in these bladders, while the specificity reaches 90 %. Endoscopy at times reveals abnormal findings, which rarely are pathologically significant [15]. The authors recommend screening with endoscopy.

Some patients require a not only a small patch of bowel used in the reconstruction but may have a diversion that is completely made from bowel, either large or small. The terminal ileum is the primary site of vitamin B12 absorption; therefore, it is important to test B12 levels in patients where terminal ileum or large segments of bowel have been incorporated into a urinary reconstruction, 5 years after such a reconstruction.

One of the most important quality of life goals for the reconstruction of exstrophy patients is urinary continence. Many studies demonstrate continence rates as high as 90 % in patients with exstrophy [16, 17], although these studies come from centers of excellence in bladder exstrophy. In communities where specialized care of bladder exstrophy does not exist, this number is likely much smaller. Continent diversions can provide continence in most patients; however, these reconstructions require routine catheterization and are not preferred by children, adolescents, and parents [18]. Patients desire to achieve normal, spontaneous, and volitional voiding and avoid the use of catheters. The goal of bladder neck reconstruction is to emulate the normal sphincteric mechanism, which is disrupted in the exstrophy patient. Patient selection is key in bladder neck reconstructions. The most important selection criterion is a patient that desires continence. When the child starts to become aware, usually during school age years (5–7), that urinary incontinence is not normal and that others are not wearing pull-ups or diapers is the ideal time to start evaluating the child and also discussing the procedure with the patient. Any continence procedure should not be performed in patients who have not reached the emotional maturity or desire to achieve continence. Patients with high bladder capacities (almost normal per age), low post void residuals, and growing bladders are ideal for BNR procedure. The BNR takes away about 30 cm³ of capacity at the time of the procedure—so it is important that the patient have a large enough bladder after to be able to store for at least 2–3 h without leakage. This procedure creates a fixed obstruction that the patient’s bladder must overcome to empty; therefore, these patients must be motivated to empty regularly and strengthen pelvic floor muscles. Those that do not fit these criteria should be offered a continent diversion when they desire continence. Patients with failed previous closures and low bladder capacities have a lower chance of achieving continence with a bladder neck reconstruction; therefore, it is important to determine which patients are candidates for this procedure [16, 17, 19]. The ultimate result of the bladder neck reconstruction is to provide a constant level of resistance to the bladder outlet, which will allow for continence until the pressure of the fluid in the bladder exceeds that of the bladder outlet. The resistance of the bladder outlet however cannot exceed the detrusor pressure during voiding otherwise retention will result. Those that are not candidates for bladder neck reconstructions will need to pursue the continent diversion route when they are psychologically and socially ready.

Continence outcomes are reported in exstrophy patients however there is a lack of standardization in reporting, therefore making it difficult to compare outcomes between studies. In the majority of studies, continence is defined for the exstrophy patient as dry in between voids or catheterization at 3-h intervals [20]. Although high rates of continence are reported in the literature for exstrophy patients after reconstruction, most studies have short follow-up. Continence rates of 70 % in patients after successful primary repairs are reported at 5 years of follow-up; however, the remaining 30 % of patients either move on to continent diversions or continue to remain incontinent [21]. At five years of follow-up, 9 % of the patients had failed BNR and needed continent diversions. Longer-term outcomes remain to be elucidated for this group. Another study with a median follow-up of 12.5 years bladder neck reconstruction alone only yielded continence in 30 % of patients [22]. As the exstrophy population continues to be followed, it will be important to track their progress to understand the durability of continence and spontaneous voiding, nephrologic outcomes, and health-related quality of life measures in adult life.

Very few studies exist to understand what kinds of additional surgical interventions are required throughout the lifetime of an exstrophy patient to maintain continence. These interventions may range from small adjustments to the bladder neck to as radical as a complete change in the method of continence. A high percentage of patients with exstrophy by adult life will need bladder augmentations and will use catheters to achieve adequate bladder capacity and preserve continence rates [23]. Changes in continence in this patient population may indicate a change in bladder compliance, detrusor overactivity, an infectious process, a failure of the reconstruction at the level of the bladder neck, or a failure of the continence valve for catheterizable stomas, hypercontinence, or the presence of a foreign body (stone, suture, etc.). It is, therefore, important to evaluate for a change in continence by history at least annually. If a change in continence is elicited, a thorough examination of the bladder and appropriate diagnostic testing is performed.