there is a growing adult spina bifida population , with 7585% of children with spina bifida expected to survive into adulthood with advances in their care [ 1 , 2 , 3 ] .
bladder disturbances are common in spina bifida patients , affecting 2498% of patients [ 37 ] . without adequate urological management ,
the upper urinary tract can be adversely affected ; as many as 48% of spina bifida patients with untreated urologic problems have evidence of kidney damage [ 8 , 9 ] .
this makes early initiation of urological management key in the preservation of renal function . early and appropriate bladder management of spina bifida patients is essential not only to maintain a healthy upper urinary tract , but also to improve the quality of life .
although uncommon , this ideally occurs in the setting of a multi - disciplinary spina bifida clinic , of which urologists play an integral role . as patients transition from pediatric to adult clinics ,
many practitioners are faced with the challenge of treating this medically complex group of patients .
the transition from a well - known and trusted pediatric clinic to an unfamiliar adult clinic can be difficult , and the ideal protocol for transition or establishment of care in an adult spina bifida clinic , and subsequent follow - up , is not clearly defined or standardized . currently , there is a scarcity of published literature regarding urologic features and management of adults with spina bifida .
one of the steps necessary to optimize our care for this growing population is to better define their patient characteristics as a group .
we aim to describe the population of the adult spina bifida patients in our own experience in the setting of a multi - disciplinary clinical practice , including demographics , urologic management and co - morbidities throughout life , in order to better understand how we can more effectively care for , reduce mortality and improve quality of life .
we performed an institutional review board approved retrospective review of patients seen in our multidisciplinary adult spina bifida clinic by the same physician ( sjk ) between january 1 , 2004 and november 1 , 2011 .
patients with the diagnosis of neural tube defects ( e.g. myelomeningocele , spina bifida occulta , tethered cord syndrome ) , as identified through the northwestern enterprise data warehouse by cpt code and age over 18 years were included in the analysis .
chart review was performed using our electronic medical record for progress notes , operative reports , lab values , pathology and imaging studies .
demographic information , urologic medications , prior urologic surgery , bladder management techniques , and co - morbidities were reviewed based on inpatient and outpatient history , physical findings , progress notes and patient reported surgical history .
history of urologic procedures and surgeries over the patient lifetime were reviewed for indication , patient age and outcomes .
when multiple stone surgeries were performed on the same patient , this was documented as a separate procedure , but notation to indicate the number of procedures on unique patients was also recorded .
mann - whitney u and fischer 's exact tests were used to evaluate statistically significant differences between procedures performed in pediatric versus adult patients . for all statistical analyses , p < 0.05 was considered statistically significant .
a total of 225 patients were identified and included in the analysis . demographic information and neurologic lesions
median current age of patients was 30 years ( range 16 - 72 , iqr 27 , 36 ) .
median age at time of presentation to the adult spina bifida clinic was 25 years ( iqr 22 , 30 ) . mean time of follow up was 37.9 ( sd 29.2 ) months , with an average frequency of 2.4 clinic appointments per year .
the majority of patients in our cohort had a diagnosis of myelomeningocele ( 86.7% ) .
the associated co - morbidities of our patient population can be found in table 2 .
21.5% of patients were identified as having one or more of the comorbidities referenced in table 2 .
the most common co - morbidities were hypertension , gastro - esophageal reflux and seizure disorders .
eight patients , or 3.6% , had documented chronic kidney disease , while one patient ( 0.9% ) had end - stage renal disease . regarding bladder management , 23 patients ( 10.2% ) voided spontaneously , 7 ( 3.1% ) voided by valsalva , 11 ( 4.9% ) into diapers , 159 ( 70.7% ) utilized clean intermittent catheterization ( cic ) ( with 10 ( 4.4% ) catheterizing through continent stomas ) , 4 ( 1.8% ) used indwelling urethral catheters , 3 ( 1.3% ) suprapubic catheter , and 8 ( 3.6% ) had an ileal conduit .
anticholinergics were prescribed for 111 patients ( 49.3% ) , including oxybutynin ( 24.0% ) , solifenacin ( 18.7% ) , darifenacin ( 4.4% ) , and tolterodine ( 2.2% )
. additionally , 65.8% underwent urodynamics at least once during their care in the spina bifida clinic .
serum creatinine was available in 180 patients ( 80% ) , with a median creatinine of 0.67 , and 93.3% having a cr < 1.3 . 
 
documented in 176 of 213 ( 82.6% ) patients with diagnoses of myelomeningocele , lipomeningocele and spina bifida occulta .
one hundred and one patients ( 44.9% ) had at least one urologic procedure during their lifetime , with a total of 191 procedures performed .
continence procedures , including pubovaginal sling , ileal conduit , urethral bulking agent , artificial urinary sphincter , male stamey sling , and suprapubic catheter placement , were 18.3% of procedures performed .
procedures for vesicoureteral reflux ( vur ) were documented in 24 patients ( 12.6% ) .
twelve patients ( 6.3% ) underwent procedures involving the urethra and bladder outlet , including urethral dilation or incision , transurethral resection bladder neck , urethral diverticulectomy and urethrovaginal repair .
most procedures were performed after 18 years of age ( 62.6% ) , while 20.7% were performed before age 18 , and 16.7% were performed at an unknown age from the available documentation . 
 
sting = subureteric transurethral injection ; turbt = transurethral resection of bladder tumor ; tur = transurethral resection ; mace = malone antegrade continence enema
mann - whitney u and fischer 's exact tests were used to evaluate significant difference between procedures performed in pediatric ( < 18 years ) versus adult ( 18 years ) patients over the course of follow - up in our cohort of 225 patients , 181 patients ( 80.4% ) completed upper urinary tract imaging ( renal ultrasound , computed tomography abdomen / pelvis or magnetic resonance imaging abdomen / pelvis ) at least once .
one hundred twenty - six ( 56.0% ) obtained imaging at least every other year and 64 ( 28.4% ) did yearly .
surgery for stone disease was more commonly performed in the adult sb patient ( 80.4% of stone surgeries ) than those < 18 years of age ( 19.6% ) ( p < 0.001 ) . of the surgical interventions ,
31.1% were performed for bladder stones , 11.5% for ureteral stones , and 57.4% for renal stones ( table 4 ) .
two patients ( 0.9% ) were diagnosed with bladder cancer during the course of follow - up , the details of which are described in a recently published case series .
briefly , both patients were young women , aged 34 and 46 years , who managed their native bladders with clean intermittent catheterization for over 20 years and were otherwise healthy prior to their diagnosis .
both patients were found to have high grade , muscle - invasive malignancy ( t2 ) , with one case of urothelial carcinoma and the other with squamous cell carcinoma .
sb is a chronic disease process that requires care from a number of medical sub - specialties and social services .
one of the obstacles to cohesive care is the effective transition from pediatric to adult care .
our results demonstrate a median age of presentation of 25 years ( iqr 27 , 36 ) , similar to reported presentation in adult spina bifida clinics of 26.8 years in the literature .
these findings suggest the existence of a  coverage gap in the appropriate care of sb patients as they transition to adult clinics .
factors impeding the timely transition of care were outside the realm of our study , although proposed factors include inability of pediatric care providers to  let go  of their patients , lack of planning , delay in readiness of transfer , little incentive for patients to transition and inability of parents to relinquish control .
neurogenic bladder in spina bifida patients can be associated with compromised renal function with elevated bladder pressures , urinary tract infections and stone formation .
patients utilizing clean intermittent catheterization have a significantly lower incidence of urinary tract dilation and vesicoureteral reflux when compared to patients voiding with valsalva , reflex voiding , or incontinence to pads [ 15 , 16 ] .
the majority ( 70.7% ) of patients in our cohort managed their bladders with clean intermittent catheterization , comparable to reported 61 - 85% clean intermittent catheterization rate reported in the literature for adults with myelomeningocele [ 1 , 17 ] . though the majority of our patients have a serum cr within the normal range , the decrease in body muscle mass in this population may lead to an over - estimation of renal function based on serum cr , which may limit our detection of renal insufficiency .
historically , many young patients with spina bifida and dysfunctional voiding were treated early with urinary diversion in hopes of preventing renal pathology .
however , studies have shown that more conservative management ( clean intermittent catheterization and anticholinergic medications ) may be as effective and pose significantly fewer risks [ 18 , 19 ] .
clean intermittent catheterization was the primary bladder management technique in 70.7% , with anticholinergic use reported in 49.3% .
nine patients ( 4.0% ) in our study had an ileal conduit and 29 patients ( 12.8% ) had undergone augmentation cystoplasty .
recent research assessing the quality of life in spina bifida adults suggests that the bladder management technique and history of urologic reconstruction did not translate into a significant change in health - related nor incontinence - related quality of life .
this highlights the importance of balancing both patient goals and quality of life related to urologic care as well as renal preservation . while the minority of patients had undergone reconstructive surgery to reduce bladder storage pressures , a large proportion of patients with spina bifida required at least one urologic surgical procedure during their lifetime ( 44.9% ) , with the majority of these procedures performed after the age of 18 ( 127/191 procedures , 66.5% ) .
we report on the lifetime incidence of urologic procedures in spina bifida patients , demonstrating both a significant frequency as well as a proportion of urologic surgeries performed over the age of 18 .
the most common urologic procedures performed were urinary tract stone treatment ( 26.7% ) , reconstructive surgery ( 25.1% ) , and continence procedures ( 18.3% ) .
the incidence of stone disease in individuals with neural tube defects ( 6.1% ) is significantly higher than the general population ( 1.0% ) .
the high incidence of stone procedures ( 51/191 procedures , 26.7% ) in our population of adult spina bifida patients may be higher than that reported for neural tube defects due to inclusion of only spina bifida patients , a group with risk factors including an indwelling catheter or clean intermittent catheterization and selection bias for routine imaging while being followed by a urologist . a higher incidence of stone procedures occurred in the adult spina bifida population ( 80.4% ) when compared to pediatric ( 19.6% ) patients .
this is the largest series of its kind in the adult spina bifida population , reporting a significant number of stone procedures in adult spina bifida patients that has not been previously well characterized .
we follow our adult spina bifida patients regularly and counsel them regarding urinary tract stone risk , presentation , management options and serial imaging .
patients with neurogenic bladder have an increased incidence of bladder cancer ( 2.310% ) that typically presents at a younger age with more aggressive and advanced disease , compared to a 2% incidence in the general population [ 22 , 23 , 24 ] .
this is consistent with our two female patients , with high grade , muscle - invasive bladder cancer ( squamous cell and urothelial ) .
hematuria should be promptly assessed with close follow - up with cystoscopy and upper tract imaging .
in addition to the need for longitudinal urologic care , appropriate medical management of comorbid conditions is essential .
analysis of our patient population indicated that 11.1% were diagnosed with hypertension and 5.3% suffered from gastro - esophageal reflux disease , compared to the ultrasound prevalence of 30.4% and 1020% , respectively [ 2528 ] . in our cohort ,
3.6% of patients were found to have chronic kidney disease with 0.9% of our patients requiring dialysis .
the relative high incidence of chronic , comorbid conditions in this cohort draws attention to the need for early and regular evaluation by primary care physicians for the optimization of general medical management , in addition to the need for close urologic follow - up .
it should be noted that these medical comorbidities were culled from reviewing multiple provider 's clinical notes and medication history and may therefore , under - represent incidence of comorbid conditions in this population .
there are a few limitations of this study that deserve mention . as a retrospective review
our findings represent only a snapshot in time of our patient population and is limited in its design for analyzing individual patient trends over time .
incomplete patient memory and the unavailability of pediatric records hindered our ability to collect complete information regarding pediatric procedures performed .
furthermore , analyses of pediatric versus adult surgeries and history of interventions should be considered within the context of the number of years within each category .
this is the first report showing the breadth of bladder management methods in an adult multidisciplinary clinic population for spina bifida .
future work is planned to investigate long - term outcomes for bladder and renal health , as well as general health outcomes in adults with spina bifida .
there is a need for longitudinal , prospective studies to better characterize the medical and urologic needs of the adult spina bifida population , their ideal management and the long - term outcomes from medical and surgical intervention .
the results presented here are the first report showing the span of modern medical and surgical management strategies utilized in a large single center sample of adult spina bifida patients managed in an adult multidisciplinary clinic .
there is a need for organized and efficient transfer of care to an adult clinic .
the multi - disciplinary clinic may be a promising start for the appropriate care of patients , given the systemic pathology seen in spina bifida .
stone procedures were the most common urologic procedure performed , with a large proportion occurring in adulthood .
future directions of research and areas for concern include optimization of quality of life , long - term outcomes , stone management and prevention and malignancy risk .
none of the contributing authors have any conflict of interest , including specific financial interests or relationships and affiliations relevant to the subject matter or materials discussed in the manuscript .
 introductionto describe the urologic outcomes of contemporary adult spina bifida patients managed in a multidisciplinary clinic.material and methodsa retrospective chart review of patients seen in our adult spina bifida clinic from january 2004 to november 2011 was performed to identify urologic management , urologic surgeries , and co-morbidities.results225 patients were identified ( 57.8% female , 42.2% male ) . 
 current median age was 30 years ( iqr 27 , 36 ) with a median age at first visit of 25 years ( iqr 22 , 30 ) . 
 the majority ( 70.7% ) utilized clean intermittent catheterization , and 111 patients ( 49.3% ) were prescribed anticholinergic medications . 
 65.8% had urodynamics performed at least once , and 56% obtained appropriate upper tract imaging at least every other year while under our care . 
 101 patients ( 44.9% ) underwent at least one urologic surgical procedure during their lifetime , with a total of 191 procedures being performed , of which stone procedures ( n = 51 , 26.7% ) were the most common . 
 other common procedures included continence procedures ( n = 35 , 18.3% ) and augmentation cystoplasty ( n = 29 , 15.2% ) . 
 only 3.6% had a documented diagnosis of chronic kidney disease and 0.9% with end - stage renal disease.conclusionsmost adult spina bifida patient continue on anticholinergic medications and clean intermittent catheterization . 
 a large percentage of patients required urologic procedures in adulthood . 
 patients should be encouraged to utilize conservative and effective bladder management strategies to reduce their risk of renal compromise . 
INTRODUCTION
MATERIAL AND METHODS
RESULTS
DISCUSSION
CONCLUSIONS
CONFLICTS OF INTEREST