Neuropathic Bladder Management of the Urinary Tract in Spina Bifida Cases Varies With Lesion Level and Shunt Presence Peter Metcalfe,* Darren Gray and Darcie Kiddoo From the Division of Pediatric Surgery, Department of Surgery and Faculty of Medicine (DG), University of Alberta, Edmonton, Alberta, Canada Purpose: The urinary and gastrointestinal tracts remain an enormous burden to the patient with spinal dysraphism. We examined our cases to determine the relationship between the level and type of spinal dysraphism, presence of a ventricular shunt, and urinary and gastrointestinal tract management. Materials and Methods: After receiving ethics approval we reviewed the charts of 155 patients younger than 17 years with spinal dysraphism. We compiled all pertinent neurological, genitourinary and gastrointestinal outcomes from our pediatric and adolescent spina bifida clinic. Results: Of our cohort 43% performed clean intermittent catheterization, including significantly more patients with myelomeningocele vs those with lipomeningocele or tethered cord (73% vs 19%, p 0.01). The intestinocystoplasty rate varied with lesion level and was significantly higher in cases of thoracic and thoracolumbar lesions than in cases of tethered cord, lipomeningocele and sacral myelomeningocele (p 0.005). Of the patients 52% older than 5 years were continent of urine but this varied significantly with lesion level and ventricular shunt presence. The urinary continence rate was highest in patients with a tethered spinal cord (57%), lipomyelomeningocele (65%) or sacral myelomeningocele (60%) (vs thoracic and lumbar myelomeningocele p 0.005). However, there was minimal difference between lumbar and thoracic lesions (25% and 26%, respectively). Of our patients 73% were also continent of feces, which did not vary with lesion level. Conclusions: Our data confirm the association of lesion level, a ventricular shunt and continence. Abbreviations and Acronyms CIC clean intermittent catheterization Study received ethics approval. * Correspondence: University of Alberta, Stollery Children s Hospital, 2C3.79 WMC, 8440-112 St., Edmonton, Alberta, T6G 2B7, Canada (e-mail: pmetcalf@ualberta.ca). Key Words: urinary incontinence, catheterization, spinal dysraphism, ventricular dysfunction, fecal incontinence SPINA bifida is considered 1 of the most devastating congenital anomalies compatible with life. However, enormous progress has been made with respect to survival and life expectancy to adulthood is now the norm. 1 Thus, contemporary treatment has become increasingly focused on management issues. Personnel at the Northern Alberta spina bifida clinic provide comprehensive care to 155 pediatric and adolescent patients younger than 17 years. Thus, we reviewed the records of our patients, focusing on the management and continence rates of the gastrointestinal and genitourinary tracts, and their association with neurosurgical variables, such as spinal dysraphism type, lesion level and presence of a ventricular shunt. MATERIALS AND METHODS After receiving institutional ethics approval we retrospectively reviewed the 0022-5347/11/1856-2547/0 Vol. 185, 2547-2551, June 2011 THE JOURNAL OF UROLOGY Printed in U.S.A. 2011 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION AND RESEARCH, INC. DOI:10.1016/j.juro.2011.01.025 www.jurology.com 2547
2548 MANAGEMENT OF URINARY TRACT IN SPINA BIFIDA CASES VARIES charts of all 155 patients seen at the Northern Alberta spina bifida clinic at the Glenrose Rehabilitation Hospital. Data on patients with all spina bifida variations were collected, including myelomeningocele, lipomeningocele and primary tethered cord. Most of our patients are seen at least annually with annual renal imaging (primarily ultrasound) and urodynamics. Although most patients undergo annual urodynamics, we did not include this in our assessment since we focused on more directly clinically relevant data. Statistical analysis was done using the chi-square test and ANOVA. Patient demographics were collected as well as the level and type of lesion. Lesion level in open spinal dysraphism cases was defined as thoracic, thoracolumbar, lumbar, lumbosacral or sacral. These lesions were closed in the immediate neonatal period. Patients with a tethered cord or lipomeningocele underwent surgical intervention at neurosurgeon discretion. All patients with tethered cord or lipomeningocele in our study underwent surgical intervention. These patients had discrete, obvious clinical (sacral dimple, lipoma or pigmentation) and radiographic findings consistent with tethered cord, excluding occult tethered cords (no definite radiographic lesion) 2 and a secondary tethered cord. Continence outcomes were strictly defined based on patient reports of complete freedom from any urinary leakage or fecal accidents in the 117 patients older than 5 years. The 14 patients who underwent incontinent urinary diversion, such as vesicostomy, ileovesicostomy or ileal conduit creation, were also excluded from continence analysis. The determination of continence was based on patient reporting during the physician visit without qualitative or prospective instruments. We did not control for patient or family compliance with the prescribed regimens. Five years was chosen as a cutoff to determine continence since in our experience families are often much more motivated to catheterize and increase anticholinergic dosing in preparation for school. However, we included all patients with spinal dysraphism, including those who were not motivated to become continent and those who were not compliant with prescribed regimens. This intentionally provided a real world analysis free from any selection bias and considering many other factors that may contribute to continence, ie social support, medical comorbidity and physical impairment. Patients were also divided by urinary tract management strategies. Spontaneous emptiers were defined as those who did not use any CIC to empty the bladder. We could not determine how many patients used the Credé or Valsalva maneuver to void, or the precise post-void residual urine. CIC via the urethra pertained to patients who were prescribed CIC into an otherwise naïve urinary tract. CIC with reconstruction included all patients who performed CIC and had undergone any surgical reconstruction, ie a Mitrofanoff or Monti catheterizable channel, bladder augmentation of any sort and bladder neck surgery. Prescribed management of the urinary tract is individualized in each patient. CIC is prescribed at birth for all patients and maintained in all with a poorly emptying bladder (post-void residual urine greater than 50%) and increased bladder pressure (greater than 20 cm H 2 O) or recurrent urinary tract infections, or to attain continence. However, when patients become 5 years old, have a low pressure bladder and clinically irrelevant post-void residual urine volume with no urinary tract infection, no hydronephrosis, no vesicoureteral reflux and bladder pressure less than 20 cm H 2 O, the family may choose not to perform CIC. This would result in the patient being considered a spontaneous emptier. Anticholinergic medication was prescribed for instability on urodynamics or increased bladder pressure, or to attain continence. Surgical intervention was done in bladders with pressure above 40 cm H 2 O, hydronephrosis or incontinence refractory to maximal medical treatment. In the young child vesicostomy was performed and when the family was prepared to invest the requisite time and effort, continent urinary diversion was done. In the older child (and family) who was not able or willing to care for continent reconstruction ileovesicostomy was recommended. Additional surgical procedures, such as bladder neck surgery or a continent catheterizable channel, were performed based on individual need. Fecal continence was defined as complete continence, as reported by the patient in clinic notes. Surgical management included cases with a Malone antegrade continence enema procedure or cecostomy button. Conservative treatment was defined as any management routine, ie no additional therapy, a prescribed stool softener, laxative or rectal enema. RESULTS Demographics A total of 155 patients who regularly attended our clinic had sufficient data for collection, including 71 males and 84 females. Mean age was 10 years (median 11, range 0.5 to 17 years). Of the patients 81 (52%) were born with myelomeningocele, 36 (23%) were born with lipomeningocele and 3 (2%) were born with meningocele. A total of 35 patients (21%) were diagnosed with tethered cord and underwent surgical intervention (fig. 1, A). Of the 120 cases of open spinal dysraphism 4 (5%) were thoracic, 11 (9%) were thoracolumbar, 33 (27%) were lumbar, 50 (42%) were lumbosacral and 22 (18%) were sacral (fig. 2). Of the 36 patients with lipomyelomeningocele 30 (83%) and 17 of the 35 (49%) with tethered cord underwent surgery before age 1 year. Only 1 patient (3%) with lipomyelomeningocele and 7 (20%) with tethered cord underwent surgery between ages 3 and 5 years, and none underwent surgery after age 5 years. None of the patients had a primary indication for surgery that involved bowel or bladder symptoms (see table). Management Urinary tract. Of our 155 patients 78 (50%) emptied the bladder spontaneously and 66 (43%) were prescribed CIC at least 3 times daily. Of those who
MANAGEMENT OF URINARY TRACT IN SPINA BIFIDA CASES VARIES 2549 Figure 1. A, lesion types at Northern Alberta Spina Bifida clinic. Dark blue area indicates myelomeningocele. Red area indicates meningocele. Green area indicates lipomyelomeningocele. Purple area indicates spinal cord injury. Light blue area indicates tethered cord. Orange area indicates other. B, bladder emptying methods. Most patients emptied spontaneously (blue area) but many required CIC. Red area indicates urethral CIC. Green area indicates catheterizable channel. Purple area indicates incontinent urinary diversion. performed CIC 61 (78%) used the urethra and 17 (22%) used a continent catheterizable channel. Ten patients (6%) underwent incontinent surgical diversion, including 8 with vesicostomy, 1 with ileovesicostomy and 1 with an indwelling catheter. Of our patients 42% were prescribed anticholinergics. In our cohort 117 children were older than 5 years. Excluding the 14 children with incontinent diversion the continence rate was 52% (54 of 103). A total of 68 patients older than 5 years spontaneously emptied, of whom 47 (69%) were continent. Nine of the 17 patients (53%) who underwent surgical reconstruction were continent of urine. The urinary continence rate in all patients who performed CIC via the urethra without surgical reconstruction was the lowest at 31% (17 of 55). These differences were statistically significant (p 0.005, fig. 3, A). The continence rate was highest in patients with sacral myelomeningocele (6 of 10 or 60%) lipomeningocele (17 or 26 or 65%) and tethered cord (16 of 28 or 57%). The continence rate was significantly lower in patients with thoracic (1 of 4 or 25%), thoracolumbar (3 of 11 or 27%), lumbar (5 of 19 or 26%) or lumbosacral (8 of 23 or 34%) lesions (p 0.005, fig. 3, B). Information on the presence of a ventricular shunt was available in 114 cases, of which 46 (40%) were shunted. The continence rate varied significantly with continence achieved by 65% of patients without a shunt and urinary continence achieved by 35% of those with a shunt by age 5 years (p 0.005). The incidence of bladder augmentation in the entire cohort was 10%. This depended on lesion level with the highest rate in patients with a thoracic or thoracolumbar lesion (3 of 15 or 20%). Eight of the 53 patients (15%) with a lumbar or lumbosacral lesion underwent augmentation. One of the 36 patients (3%) with lipomeningocele underwent augmentation compared with none of the 35 with a tethered cord (p 0.005, fig. 4, A). All augmentations were done with ileum and in all cohort patients a continent catheterizable channel was created. Bladder neck surgery was performed when leak point pressure was low (approximately less than 15 cm H 2 O), which occurred in 12 of 17 patients (70%). Ten of the 12 patients (83%) underwent bladder neck closure and the remainder received a suburethral sling. Gastrointestinal tract. The overall fecal continence rate in patients older than 5 years was 77% Table Age No. Lipomyelomeningocele No. Tethered Cord Figure 2. Myelomeningocele levels. As expected, lumbosacral lesions account for most cases. Birth-less than 1 wk 2 0 Greater than 1 wk-less than 6 mos 23 12 Greater than 6 mos-less than 1 yr 5 5 1 2 Yrs 5 9 2 3 Yrs 0 2 3 5 Yrs 1 7 Totals 36 35
2550 MANAGEMENT OF URINARY TRACT IN SPINA BIFIDA CASES VARIES maximizing quality of life. 5,6 Ideally patients are treated at comprehensive clinics to address neurosurgical, musculoskeletal, developmental and urological issues. 7 This cooperative care has made great strides in allowing many of our patients to attend school, graduate and become productive members of society. However, management of the urinary and gastrointestinal tracts remains a significant challenge with respect to the burden to patient and difficulty in attaining continence. We assessed cases at our spina bifida clinic to determine the rates of CIC, surgical intervention and continence, and their relationship to lesion type and level as well as the presence of a ventricular shunt. Our overall urinary and fecal continence rates are less than ideal and represent a major shortcoming of the data. We defined urinary and fecal continence based only on physician and nursing history during the most recent clinic visit and inclusion in the com- Figure 3. Urinary continence. A, bladder emptying. Patients with spontaneous voiding were more likely to be continent than those with urethral catheterization. Antichol, anticholinergics. B, continence varied by lesion level and type with better continence in patients with sacral (S) myelomeningocele, lipomeningocele (LM, Lipo) and tethered cord (TC) than in those with lumbar (L) and thoracic (T) myelomeningocele. (90 of 117). No patient underwent colostomy for fecal control. Patients requiring only conservative treatment achieved a 79% continence rate (76 of 96). Of our 117 patients 21 (18%) underwent surgical intervention for fecal control with a resultant continence rate of 67% (14 of 21). Fecal continence did not significantly differ with respect to the lesion. The highest rates were seen in patients with lipomeningocele or tethered cords (85% and 82%, respectively). Cases of thoracic, lumbar and sacral lesions had a 67% (10 of 15), 66% (27 of 41) and 64% (7 of 11) rate of fecal continence, respectively (fig. 4, B). DISCUSSION Since the introduction of the ventriculoperitoneal shunt and CIC, 3 life expectancy in patients with spina bifida has improved so that 85% attain adulthood. 4 Thus, increased emphasis has been placed on Figure 4. A, bladder augmentation rate varied by lesion level and type. B, fecal continence rate was relatively good without significant difference among lesion types. MACE, Malone antegrade continence enema.
MANAGEMENT OF URINARY TRACT IN SPINA BIFIDA CASES VARIES 2551 prehensive medical record. Although this was susceptible to a reporting bias due to the physicianpatient relationship, we think that our strict definition helped counterbalance this. If the patient or parent reported any incontinence regardless of voiding or catheterizing interval, we considered them incontinent. The lack of a prospective or standardized questionnaire also resulted in our inability to account for patient or caregiver compliance with a prescribed medical or catheterizing regimen. The lack of quality of life data is another significant shortcoming since we could not differentiate patients who did not choose to aggressively pursue continence from those who were not bothered by minor incontinence. Our bladder augmentation rate is low compared to that in other published reports, which may reflect local bias and not be generalizable to other centers. Despite this there was a definite relationship to the dysraphism level and type. Furthermore, as success with contemporary bladder reconstruction increases and more of our patients elect continent reconstruction, we hope that the continence rates will improve. Fecal continence rates were generally good and relatively equal across all patients. Of those in whom conservative management failed two-thirds achieved continence via an antegrade enema. We believed that it was important to divide patients by lesion level since the neural pathophysiology can be quite different. We noted significant differences between the spinal defect site with thoracic and thoracolumbar lesions associated with higher rates of bladder augmentation, CIC requirements and urinary incontinence. We were encouraged that patients with sacral myelomeningocele behaved more like those with the closed lesions of lipomeningocele and tethered cord with respect to continence and CIC. It has been confirmed that the incidence of spina bifida is decreasing 8 10 and a greater proportion of new patients have more distal lesions, ie sacral. 9 Thus, the overall continence rate and the spontaneous voiding rate will likely increase. To our knowledge the association between a ventricular shunt and continence has not been described previously. Although this is not likely directly related to the central innervation of the bladder, it may be associated with lesion level and an associated Chiari malformation. We believe that this merits further investigation since the rate of ventriculoperitoneal shunts may be decreasing with neonatal closure of myelomeningocele. 11 13 CONCLUSIONS These data provide valuable insight into urinary and fecal tract management and continence in spinal dysraphism cases. We noted a clear relationship with lesion level and type. Despite data flaws this provides a comprehensive overview of contemporary outcomes. The incidence of prescribed management regimens is as important as the continence rate to accurately educate and compare our patients. REFERENCES 1. Dicianno BE, Kurowski BG, Yang JM et al: Rehabilitation and medical management of the adult with spina bifida. Am J Phys Med Rehabil 2008; 87: 1027. 2. Metcalfe PD, Luerssen TG, King SJ et al: Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale. J Urol 2006; 176: 1826. 3. Lapides J, Diokno AC, Silber SJ et al: Clean, intermittent self-catheterization in the treatment of urinary tract disease. J Urol 1972; 107: 458. 4. Mukherjee S: Transition to adulthood in spina bifida: changing roles and expectations. Sci World J 2007; 7: 1890. 5. MacNeily AE, Jafari S, Scott H et al: Health related quality of life in patients with spina bifida: a prospective assessment before and after lower urinary tract reconstruction. J Urol 2009; 182: 1984. 6. Yerkes EB, Cain MP, King S et al: The Malone antegrade continence enema procedure: quality of life and family perspective. J Urol 2003; 169: 320. 7. Kinsman SL, Levey E, Ruffing V et al: Beyond multidisciplinary care: a new conceptual model for spina bifida services. Eur J Pediatr Surg, suppl., 2000; 10: 35. 8. Kondo A, Kamihira O and Ozawa H: Neural tube defects: prevalence, etiology and prevention. Int J Urol 2009; 16: 49. 9. Aguilera S, Soothill P, Denbow M et al: Prognosis of spina bifida in the era of prenatal diagnosis and termination of pregnancy. Fetal Diagn Ther 2009; 26: 68. 10. Cameron M and Moran P: Prenatal screening and diagnosis of neural tube defects. Prenat Diagn 2009; 29: 402. 11. Husler MR, Danzer E, Johnson MP et al: Prenatal diagnosis and postnatal outcome of fetal spinal defects without Arnold-Chiari II malformation. Prenat Diagn 2009; 29: 1050. 12. Adzick NS: Fetal myelomeningocele: natural history, pathophysiology, and in-utero intervention. Semin Fetal Neonatal Med 2010; 15: 9. 13. Hirose S and Farmer DL: Fetal surgery for myelomeningocele. Clin Perinatol 2009; 36: 431.