Cumulative diagnostic radiation exposure in children with ventriculoperitoneal shunts: a review.

Smyth MD, Narayan P, Tubbs RS, Leonard JR, Park TS, Loukas M, Grabb PA. Cumulative diagnostic radiation exposure in children with ventriculoperitoneal shunts: a review. Child’s Nervous System. 2008 Apr;24(4):493-7. Review.

INTRODUCTION: Children may be more vulnerable to diagnostic radiation exposure because of the increased dose-volume ratio and the increased lifetime risk per unit dose of radiation from early exposure. Moreover, recent radiological literature suggests that exposure to ionizing radiation from imaging studies may play a role in the later development of malignancies.

MATERIALS AND METHODS: We review the literature and present two illustrative clinical examples of children (each child developed head and neck malignancies during their late teen years) with hydrocephalus requiring multiple cerebrospinal fluid (CSF) shunt revisions and diagnostic computerized tomography (CT) scans throughout their life.

DISCUSSION: The literature reviewed suggests that children are more prone to diagnostic radiation exposure. Although it is not possible to prove that the multiple diagnostic studies result in malignancies, our review of the literature and illustrative cases describing malignancy risk and radiation exposure should give clinicians pause when considering requesting multiple diagnostic CT studies in children during the evaluation of possible CSF shunt dysfunction. Alternative tests such as "shunt MRI" protocols should be considered for patients and used whenever possible to minimize exposure to ionizing radiation.

PMID: 18180935

Memory functions in children with early hydrocephalus.

Scott MA, Fletcher JM, Brookshire BL, Davidson KC, Landry SH, Bohan TC, Kramer LA, Brandt ME, Francis DJ. Memory functions in children with early hydrocephalus.
Neuropsychology. 1998 Oct;12(4):578-89.

Department of Pediatrics, University of Arkansas for Medical Sciences, Springdale 72765-0768, USA. mscott3@aol.com

Children with arrested, shunted, and no hydrocephalus were compared on verbal and nonverbal memory tasks assessing multiple components of memory. A gradient of severity was hypothesized, with the shunted hydrocephalus group expected to exhibit the most significant memory impairments and the arrested group expected to perform more poorly than children with no hydrocephalus. Etiologies of prematurity, spina bifida, and aqueductal stenosis were represented by 157 participants. Results supported the hypothesis; the shunted hydrocephalus group performed poorer on all memory measures. Differences for the arrested group were less frequently statistically significant relative to children with no hydrocephalus. Irrespective of etiology, the shunted hydrocephalus group exhibited a pattern of performance suggestive of encoding and retrieval deficits on both verbal and nonverbal tasks, showing a pervasive disturbance of memory processes.

PMID: 9805328

Sustained attention in children with two etiologies of early hydrocephalus.

Swartwout, Maegan D.; Cirino, Paul T.; Hampson, Amy W.; Fletcher, Jack M.; Brandt, Michael E.; Dennis, Maureen; Sustained attention in children with two etiologies of early hydrocephalus. Neuropsychology, Vol 22(6), Nov, 2008. pp. 765-775.

Department of Psychology, University of Houston, Houston, TX 77204-5355, USA. mdswartwout@uh.edu

Several studies have shown that children with spina bifida meningomyelocele (SBM) and hydrocephalus have attention problems on parent ratings and difficulties in stimulus orienting associated with a posterior brain attention system. Less is known about response control and inhibition associated with an anterior brain attention system. Using the Gordon Vigilance Task (Gordon, 1983), we studied error rate, reaction time, and performance over time for sustained attention, a key anterior attention function, in 101 children with SBM, 17 with aqueductal stenosis (AS; another condition involving congenital hydrocephalus), and 40 typically developing controls (NC). In SBM, we investigated the relation between cognitive attention and parent ratings of inattention and hyperactivity and explored the impact of medical variables. Children with SBM did not differ from AS or NC groups on measures of sustained attention, but they committed more errors and responded more slowly. Approximately one-third of the SBM group had attention symptoms, although parent attention ratings were not associated with task performance. Hydrocephalus does not account for the attention profile of children with SBM, which also reflects the distinctive brain dysmorphologies associated with this condition.

PMID: 18999350

Transcallosal connectivity and cortical rhythms: findings in children with spina bifida.

Castillo EM, Fletcher JM, Li Z, Hoskison MM, Hasan KM, Passaro A, Papanicolaou AC. Transcallosal connectivity and cortical rhythms: findings in children with spina bifida. NeuroReport. 2009 Aug 26;20(13):1188-92.

Center for Clinical Neurosciences, Department of Pediatrics, Medical School, University of Texas Health Science Center at Houston, Houston, Texas 77030, USA. Eduardo.m.castillo@uth.tmc.edu

We studied the relation between cortical oscillatory rhythms and the structural integrity of the corpus callosum in 21 children with spina bifida and hydrocephalus. Participants underwent resting state neuromagnetic recordings and diffusion tensor imaging. Areas of three segments of the corpus callosum (genu, body, splenium) were derived through diffusion tensor imaging-based morphometrics. Children with spina bifida showed reduced values of spectral power in the theta, alpha and beta bands when compared with age-matched controls, but only in the posterior and temporal regions. Reduced spectral power in posterior regions correlated with decreased area of the posterior segments of the corpus callosum. Atypical cortical oscillatory activity is associated with reduced transcallosal connectivity in children with spina bifida.

Fetal surgery for myelomeningocele.

Hirose S, Farmer DL. (2009) Fetal surgery for myelomeningocele. Clinics in Perinatology. 2009 Jun;36(2):431-8, xi.

Division of Pediatric Surgery, Department of Surgery, Fetal Treatment Center, University of California, San Francisco, CA 94143-0570, USA. hiroses@surgery.ucsf.edu

Fetal intervention for myelomeningocele (MMC) may improve hydrocephalus and hindbrain herniation associated with the Arnold-Chiari II malformation and may reduce the need for ventriculoperitoneal shunting. As of now, there is little evidence that prenatal repair of MMC improves neurologic function. MMC is the first nonlethal disease under consideration and study for fetal surgery. As a result, potential improvements in outcome must be balanced with maternal safety and well-being, in addition to that of the unborn patient.

PMID: 19559329

After the honeymoon comes divorce: long-term use of the antegrade continence enema procedure.

Yardley IE, Pauniaho SL, Baillie CT, Turnock RR, Coldicutt P, Lamont GL, Kenny SE. (2009) After the honeymoon comes divorce: long-term use of the antegrade continence enema procedure. Journal of Pediatric Surgery. 2009 Jun;44(6):1274-6; discussion 1276-7.

Department of Paediatric Surgery, Alder Hey Children's Hospital, Liverpool, UK.

BACKGROUND: Having reported that 18% of children discontinue use of the antegrade continence enema (ACE) after 5 years, we aimed to determine long-term use after an ACE procedure.

METHODS: A postal/telephone questionnaire was conducted. Subjects were consecutive children undergoing an ACE between 1993 and 1999. Outcome measures were use of ACE, reasons for nonuse, complications, and overall satisfaction.

RESULTS: Of 84 eligible subjects, data were available on 61 (73%) aged 22.4 years (15.5-35.1 years). Underlying diagnoses included spina bifida (n = 27), anorectal malformations (n = 18), constipation (n = 11), Hirschsprung's disease (n = 1), sacral agenesis (n = 2), and trauma/tumor (n = 2). Follow-up was 11.02 years (8.34-14.39 years). Thirty-six (59%) of 61 patients were still using their ACE. Reasons for nonuse were lack of effectiveness (n = 14), complications (n = 5), psychologic issues (n = 2), and poor compliance (n = 2). There was no association between diagnosis and nonuse (chi(2), P = .63). In those still using ACE, the overall satisfaction score was 4.1 (1-5). Several individuals reported feeling abandoned on becoming adults and losing the support they had in childhood.

CONCLUSION: There is a late "failure" rate for the ACE procedure. However, satisfaction was high among those still using the ACE. This study further emphasizes the need for robust transitional care arrangements.

PMID: 19524753

Treatment of fecal incontinence with a comprehensive bowel management program.

Bischoff A, Levitt MA, Bauer C, Jackson L, Holder M, Peña A. (2009) Treatment of fecal incontinence with a comprehensive bowel management program. Journal of Pediatric Surgery. 2009 Jun;44(6):1278-83; discussion 1283-4.

Department of Pediatric Surgery, Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.

PURPOSE: Many articles describe the antegrade continence enemas (ACEs), but few refer to a bowel management program. A successful ACE may not help a patient without such management. Valuable lessons were learned by implementation of bowel management in 495 fecally incontinent patients.

METHODS: We previously reported 201 patients. Thereafter, another 294 patients participated in our program. On the basis of a contrast enema and symptoms, they were divided as follows: (a) 220 constipated patients and (b) 74 patients with tendency toward diarrhea. Colonic stool was monitored with abdominal radiographs, modifying the management according to the patient's response and radiologic findings. For constipated patients, the emphasis was on using large enemas. For patients with tendency toward diarrhea, we used small enemas, a constipating diet, loperamide, and pectin. Diagnoses included anorectal malformation (223), Hirschsprung's (36), spina bifida (12), and miscellaneous (23).

RESULTS: The management was successful in 279 patients (95%)-higher in constipated patients (98%) and less successful in patients with tendency toward diarrhea (84%).

CONCLUSIONS: The key to a successful bowel management program rests in tailoring the type of enema, medication, and diet to the specific type of colon. The best way to determine the effect of an enema is with an abdominal film. The ACE procedures should be recommended only after successful bowel management.


PMID: 19524754

Motor profile and cognitive functioning in children with spina bifida.

Vinck A, Nijhuis-van der Sanden MW, Roeleveld NJ, Mullaart RA, Rotteveel JJ, Maassen BA. Motor profile and cognitive functioning in children with spina bifida.
European Journal of Pediatric Neurology. 2009 Feb 21. [Epub ahead of print]

Department of Medical Psychology, Radboud University Nijmegen Medical Centre, The Netherlands; Department of Paediatric Neurology, Radboud University Nijmegen Medical Centre, The Netherlands.

BACKGROUND: Spina bifida is a complex neuroembryological disorder resulting from incomplete closure of the posterior neural tube. Morbidity in the different fields of motor and cognitive neurodevelopment is variable in nature and severity, and often hard to predict.

AIMS: The current study investigates the relationship between cognitive functioning, fine motor performance and motor quality in children with spina bifida myelomeningocele (SBM) and SB-only, taking into consideration the cerebral malformations.

MATERIAL AND METHODS: Forty-one children were included (22 girls and 19 boys aged between 6 and 14 years, mean age 10;0 years) in the study. A comprehensive assessment was conducted of cognitive functioning and motor profile, including fine motor and visual-motor functioning, and motor quality. The performance outcomes were analyzed for the total group of children and separately for the nonretarded children (FSIQ>/=70, N=30) to eliminate the influence of global intellectual impairment.

RESULTS: Although the children with spina bifida showed increased incidence of cognitive and fine motor impairment, and impaired motor quality, after exclusion of the overall retarded children no associations were found between cognitive functioning and motor profile. In the comparison of SBM to SB-only specific differences were found for performance IQ, visual-motor functioning and motor quality, but not fine motor functioning.

CONCLUSION: Our findings underscore the role of cerebral malformation in spina bifida and its consequences for neuropsychological functioning. The complicated developmental interactions found strengthen the need for an individualized management of children with SB.

PMID: 19237302

The scientific basis for eliminating folic acid-preventable spina bifida: a modern miracle from epidemiology.

Oakley GP Jr. The scientific basis for eliminating folic acid-preventable spina bifida: a modern miracle from epidemiology. Annals of Epidemiology. 2009 Apr;19(4):226-30.

Department of Epidemiology, Rollins School of Public Health of Emory University, Atlanta, GA, USA. gpoakley@mindspring.com

One of the most remarkable successes of epidemiology was the demonstration in the late twentieth century that spina bifida and anencephaly-two of the most common and severe birth defects-are caused primarily by folate deficiency. This article reviews the descriptive epidemiological studies that began when we did not have a clue about etiology. The paper tells the success story of the trials that proved that folic acid would prevent folic-acid-preventable spina bifida. Finally, it will tell how difficult it is to get prevention policy implemented, even when the scientific evidence is compelling. It concludes by noting that the inaction or inappropriate actions of food regulatory bodies in so many countries means that only 10% of folic-acid-preventable spina bifida is actually being prevented--a serious failure of public health policy.

PMID: 19344858

Mouse models for neural tube closure defects.

Juriloff DM, Harris MJ. Mouse models for neural tube closure defects. Human Molecular Genetics. 2000 Apr 12;9(6):993-1000. Review.

Department of Medical Genetics, University of British Columbia, 6174 University Boulevard, Vancouver, British Columbia, Canada. juriloff@interchange.ubc.ca

Neural tube closure defects (NTDs), in particular anencephaly and spina bifida, are common human birth defects (1 in 1000), their genetics is complex and their risk is reduced by periconceptional maternal folic acid supplementation. There are > 60 mouse mutants and strains with NTDs, many reported within the past 2 years. Not only are NTD mutations at loci widely heterogeneous in function, but also most of the mutants demonstrate variable low penetrance and some show complex inheritance patterns (e.g. SELH/Bc, Abl / Arg, Mena / Profilin1 ). In most of these mouse models, the NTDs are exencephaly (equivalent to anencephaly) or spina bifida or both, reflecting failure of neural fold elevation in well defined, mechanistically distinct elevation zones. NTD risk is reduced in various models by different maternal nutrient supplements, including folic acid ( Pax3, Cart1, Cd mutants), inositol ( ct ) and methionine ( Axd ). Lack of de novo methylation in embryos ( Dnmt3b -null) leads to NTD risk, and we suggest a potential link between methylation and the observed female excess among cranial NTDs in several models. Some surprising NTD mutants ( Gadd45a, Terc, Trp53 ) suggest that genes with a basic mitotic function also have a function specific to neural fold elevation. The genes mutated in several mouse NTD models involve actin regulation ( Abl/Arg, Macs, Mena/Profilin1, Mlp, Shrm, Vcl ), support the postulated key role of actin in neural fold elevation, and may be a good candidate pathway to search for human NTD genes.

PMID: 10767323

Adverse reproductive outcomes among pregnancies of aunts and (spouses of) uncles in Irish families with neural tube defects

Byrne J, Carolan S. Adverse reproductive outcomes among pregnancies of aunts and (spouses of) uncles in Irish families with neural tube defects. American journal of medical genetics. Part A. 2006 Jan 1;140(1):52-61.

Boyne Research Institute, 5 Potato Market Square East, Drogheda, Ireland. boyne.research@verizon.net

Adverse pregnancy outcomes may be more frequent among sibs of individuals with neural tube defects (NTDs), and transmission of risk in families with an NTD may be more frequent among maternal relatives. In a study designed to evaluate matrilineal risk for NTDs, we compared adverse pregnancy outcomes among maternal and paternal first cousin pregnancies. Pregnancy histories were obtained by interview with 288 uncles and aunts (parents of the first cousin pregnancies) in 48 Irish NTD families. We analyzed pregnancy outcomes (preterm deliveries, stillbirths, and miscarriages) among 1,033 singleton first cousin pregnancies and compared risk among maternal versus paternal relatives. Maternal first cousin pregnancies were more likely to end adversely when compared to paternal first cousin pregnancies (17.4% vs. 11.7%, P = 0.01). In a logistic regression analysis of pregnancies unaffected by birth defects, maternal line remained independently associated with adverse outcomes (odds ratio (OR) = 1.55, 95% confidence interval (CI) 1.06, 2.27) after controlling for NTD type, maternal age, maternal smoking during pregnancy, first cousin pregnancy's year of birth. The excess risk with maternal line related mainly to spina bifida occulta families (OR = 42.4; CI 2.64, 681; P = 0.008); risk in open spina bifida families was 1.24 (CI 0.82, 1.87; P = 0.3). These results support the hypothesis of excess risk for adverse pregnancy outcomes among maternal relatives in NTD families. Further work is needed, epidemiological as well as clinical and molecular, not only to confirm these findings, but also to define the underlying biological mechanisms linking adverse reproductive outcomes, excess maternal risk and occurrence of NTDs.

PMID: 16333827

Further evidence for a maternal genetic effect and a sex-influenced effect contributing to risk for human neural tube defects.

Deak KL, Siegel DG, George TM, Gregory S, Ashley-Koch A, Speer MC; NTD Collaborative Group. (2008) Further evidence for a maternal genetic effect and a sex-influenced effect contributing to risk for human neural tube defects. Birth defects research. Part A, Clinical and molecular teratology. 2008 Oct;82(10):662-9.

Center for Human Genetics, Duke University Medical Center, Durham, North Carolina, USA.

BACKGROUND: Neural tube defects (NTDs), including spina bifida and anencephaly, are the second most common birth defect with an incidence of 1/1000. Genetic factors are believed to contribute to NTD risk and family-based studies can be useful for identifying such risk factors.

METHODS: We ascertained 1066 NTD families (1467 affected patients), including 307 multiplex NTD families. We performed pedigree analysis to describe the inheritance patterns, pregnancy outcomes, and recurrence risks to relatives of various types.

RESULTS: Myelomeningocele or spina bifida (66.9%) and cranial defects (17.7%) were the most common NTD subtypes observed. The overall male:female ratio for affected individuals was 0.82, and there were even fewer males among individuals with an upper level NTD (0.62). Among twins, 2 of the 5 monozygotic twins and only 3 of 35 dizygotic twins were concordant, while 27% of the same sex twins were concordant, but none of the different sex twins. The estimated 6.3% recurrence risk to siblings (CI 0.04-0.08) is consistent with previous reports. Families with two or more affected individuals show a higher proportion of female transmitters (p = 0.0002). Additionally, the number of affected relatives in maternal compared to paternal lineages was more than double (p = 0.006). There were significantly more miscarriages, infant deaths, and stillborn pregnancies of the maternal aunts and uncles (p < 0.0001) and of first cousins (p = 0.04).

CONCLUSIONS: Our data provide several lines of evidence consistent with a maternal effect, as well as a sex-influenced effect, in the etiology of NTDs.

PMID: 18937341

The Chiari malformations: A constellation of anomalies

Robert M. Shuman RM. (1995) The Chiari malformations: A constellation of anomalies. Seminars in Pediatric Neurology. 1995 Sep;2(3):220-6. Review.


The Chiari malformations form a group of abnormalities that are pathogenetically interrelated. The most important member of the group is the Chiari type II malformation, known as the Arnold-Chiari malformation. Its cardinal features are the myelomeningocele in the thoraco-lumbar spine, the venting of the intracranial cerebrospinal fluid through the central canal, the hypoplasia of the posterior fossa, the herniation of hindbrain into the cervical spinal canal, and the compressive damage to cranial nerves. Some of the abnormalities are progressive, and thus treatable. Limitation of progression may improve outcomes. The challenges to our treatment programs involve early diagnosis, delivery by Caesarean section, emergent closure of the neural plaque and prophylaxis of hydrocephalus, anticipatory prevention of the neurological compression syndromes, multidisciplinary teams, and age-appropriate interventions.

The Natural History of Spina Bifida Cystica: Detailed Analysis of 407 Cases

K. M. Laurence The Natural History of Spina Bifida Cystica: Detailed Analysis of 407 Cases Archives of Disease in Childhood. 1964 February; 39(203): 41–57.

Partial agenesis of the corpus callosum in spina bifida meningomyelocele and potential compensatory mechanisms.

Hannay HJ, Dennis M, Kramer L, Blaser S, Fletcher JM. (2009) Partial agenesis of the corpus callosum in spina bifida meningomyelocele and potential compensatory mechanisms.
Journal of Clinical and Experimental Neuropsychology. 2009 Feb;31(2):180-94.

After a review of Arthur Benton's conceptual and methodological contributions to the understanding of normal and pathological development, we discuss agenesis of the corpus callosum (CC), criteria for potential neuroanatomical compensatory mechanisms in CC agenesis, and the results of an examination of magnetic resonance imaging (MRI) data of the CC in 193 children with spina bifida meningomyelocele (SBM). There were 26 CC regional patterns. Although complete agenesis did not occur, partial agenesis was observed in 102 children and within 15 CC regional patterns. Only 4.1% had a normal CC. Quantitative assessment of the area of the CC in 26 NC children and 68 children with SBM revealed that all subgroups with CC anomalies had smaller areas than did a subgroup with a normal CC. Areas were especially small in rostral/splenial agenesis and splenial agenesis but larger with rostral agenesis. Subgroups with normal/hypoplastic regions or complete hypoplasia also had CC areas that were smaller than normal but larger than the areas for the splenial agenesis groups. The relative rarity of anterior commissure enlargement (3.1%) and longitudinal bundles of Probst (0.1%) suggest that these particular fiber tract anomalies are unlikely candidates for structural compensatory mechanisms. The hippocampal commissure, enlarged in 13%, may be a more promising candidate. Overall, however, the functionality of anomalous fiber tracts and commissures in SBM is yet to be determined.

PMID: 19052950

Development and validation of the fecal incontinence and constipation quality of life measure in children with spina bifida

Nanigian DK, Nguyen T, Tanaka ST, Cambio A, DiGrande A, Kurzrock EA. Development and validation of the fecal incontinence and constipation quality of life measure in children with spina bifida. Journal of Urology. 2008 Oct;180(4 Suppl):1770-3; discussion 1773.

Department of Urology, University of California-Davis, Davis Children's Hospital, California, USA.

PURPOSE: Fecal incontinence and constipation in children with spina bifida are recognized to impact quality of life. Most disease specific quality of life instruments on fecal incontinence target adults and/or children without neuropathic bowel. We developed an instrument to evaluate bowel function and its impact on quality of life in children with spina bifida and their caregivers. MATERIALS AND METHODS: A 51-item questionnaire termed the FIC QOL (Fecal Incontinence and Constipation Quality of Life) survey was developed from expert opinion, patient interviews, and modification of previously published adult and pediatric studies for nonneuropathic bowel dysfunction. The items are divided into 7 quality of life factor groupings, including bowel program, dietary management, symptoms, travel and socialization, family relationships, caregiver emotional impact and financial impact. The questionnaire was given to caregivers of children with and without spina bifida. Discriminant validity was evaluated by comparing the spina bifida and control groups. Test-retest reliability was evaluated by having 41 patients complete 2 surveys within 4 to 6 weeks. RESULTS: Comparing questionnaires from 92 index patients and 52 controls showed a statistically significant difference for all 7 quality of life factor groupings. The FIC QOL instrument objectively demonstrated the negative impact of fecal incontinence and constipation on quality of life in these families. Comparing 82 questionnaires at 2 time points demonstrated the reliability of all FIC QOL questions. CONCLUSIONS: The FIC QOL instrument provides a valid and reliable measure of the effect of fecal incontinence and constipation on the quality of life of caregivers and their children with spina bifida.

PMID: 18721959

Evaluating Outcomes of Enterocystoplasty in Patients With Spina Bifida: A Review of the Literature

Scales CD Jr, Wiener JS. Evaluating Outcomes of Enterocystoplasty in Patients With Spina Bifida: A Review of the Literature. Journal of Urology. 2008 Oct 16.

Duke University Medical Center, Durham, North Carolina.

PURPOSE: The urological complications of spina bifida impose a significant burden of disability and disease. Therapy is focused on the bladder to achieve the primary goals of maintaining normal renal function and attaining urinary continence. When medical management fails, surgical intervention, including enterocystoplasty, is frequently performed. However, practice patterns for enterocystoplasty show significant variation. Given this context, we examined outcome measures for enterocystoplasty in patients with spina bifida.

MATERIALS AND METHODS: A MEDLINE(R) search was performed for articles on enterocystoplasty in patients with spina bifida. A total of 226 articles were identified and manually reviewed for relevant studies. Additional articles were selected based on a cited reference search. RESULTS: Almost all studies are retrospective, single institution case series of a relatively small number of patients. Few uniform or validated outcome measures for enterocystoplasty exist but reported measures typically include urodynamic, continence and satisfaction parameters. Interinstitutional variability in urodynamic measurements and in definitions of continence makes a comparison of outcomes difficult. The complication rate following enterocystoplasty is significant, well described and primarily related to the use of gastrointestinal segments for urine storage.

CONCLUSIONS: Medical management is the mainstay of neurogenic bladder therapy in the spina bifida population. Enterocystoplasty remains an important option to prevent or reverse upper tract deterioration, and/or improve or cure socially unacceptable incontinence despite poorly defined outcome measures. The development of appropriate and validated outcomes measures may enable more uniform, effective and safe urological care of patients with spina bifida.

PMID: 18930285

Endourethral injection of bulking agents for urinary incontinence in children

Godbole P, Bryant R, MacKinnon AE, Roberts JP. Endourethral injection of bulking agents for urinary incontinence in children. BJU International (Journal of the British Association of Urological Surgeons). 2003 Apr;91(6):536-9.

Department of Paediatric Surgery, Sheffield Children's Hospital, Sheffield, UK. prasadgodbole@btinternet.com

OBJECTIVE: To assess the early and late outcome of endourethral injection with bulking agents in children with urinary incontinence (a neuropathic bladder or exstrophy-epispadias complex), by reviewing our experience over a 5-year period.

PATIENTS AND METHODS: The records of 15 children (10 boys) were reviewed retrospectively; 10 had spina bifida and a neurogenic bladder, four had a neurogenic bladder from other causes and one had epispadias. All children had a stable low-pressure detrusor and a compliant bladder with sphincteric weakness on preoperative urodynamic testing. Four children had undergone previous enterocystoplasty with a Mitrofanoff stoma, with concomitant urethral lengthening in two and a Goretex trade mark bladder neck sling in two. Three children voided spontaneously while 12 depended on intermittent catheterization. The agent was injected under general anaesthesia in all patients but one, with an endourethral submucosal injection of the bulking agent into four or more points at the junction of the bladder neck and proximal urethra, aiming to obtain visual occlusion of the urethra. The median (range) number of injections was 2 (1-3); five children had one injection, seven had two and three had three. There were no procedure-related complications and most were day-case procedures. Initially PTFE paste was used as the bulking agent, being replaced by bovine collagen or polydimethylsiloxane in the latter half of the series.

RESULTS: At a median (range) follow-up of 28 (11-65) months three children were completely dry after a single injection; there was no change in four and a short-term improvement (median 25 months, range 4 days to 37 months) in eight. After this period all children deteriorated to their original incontinence grade; hence the overall cure rate was three of 15.

CONCLUSION: This experience with a long-term follow-up differs from previously reported high success rates for the endourethral injection of bulking agents for urinary incontinence in children. Despite a short-term benefit, in the long-term this technique was unreliable and often ineffective. Patients and their carers should be given a realistic and guarded prognosis.

PMID: 12656911

The management of the failed bladder neck procedure in patients with spina bifida

Walker RD. The management of the failed bladder neck procedure in patients with spina bifida. BJU International (Journal of the British Association of Urological Surgeons).. 2003 Oct;92 Suppl 1:35-7. Review.

Urinary incontinence secondary to decreased outlet resistance is common in patients with spina bifida. Most often these patients require both augmentation cystoplasty and a procedure to increase resistance at the bladder neck. The choices for operations to increase bladder neck resistance include the artificial urinary sphincter (AUS), the Kropp and Leadbetter procedures (and variations) and bladder neck wraps or slings. Bladder neck injection with bulking agents has also been used but the long-term results are unknown and most authors do not address what to do when the procedure fails. While the short-term success with each of the open procedures has been good, the longer-term success has been less satisfactory. The purpose of this study is twofold; to review the author's experience with those patients in whom a rectus fascial wrap has failed and in the discussion, to review how others have
managed the failures in procedures that they have advocated.

PMID: 12969007

Epanded PTFE bladder neck slings for incontinence in children: the long-term outcome

Godbole P, Mackinnon AE. Epanded PTFE bladder neck slings for incontinence in children: the long-term outcome. BJU International (Journal of the British Association of Urological Surgeons). 2004 Jan;93(1):139-41.

Department of Paediatric Urology, Sheffield Children's NHS Trust, Sheffield, UK. prasadgodbole@btinternet.com

OBJECTIVE: To assess the long-term outcome of circumferential expanded PTFE (Gore-tex, WL Gore Associates, Scotland) bladder neck slings for achieving urethral continence in children with a neuropathic bladder.

PATIENTS AND METHODS: The records were reviewed of 19 children undergoing bladder reconstruction (most with a neuropathic bladder) who had a Gore-tex sling placed circumferentially at the bladder neck, over a 5-year period. Of these, seven had spina bifida; two each spinal dysraphism, surgery for anorectal anomalies and an idiopathic neuropathic bladder; five who developed a neuropathic bladder from other causes, and one born with bladder exstrophy. All children had an uncompliant bladder with a low urethral leak-point pressure on preoperative urodynamics. In all children conventional clean intermittent catheterization and pharmacotherapy had failed. Four had had previous augmentation surgery while 15 had concomitant bladder augmentation and formation of a Mitrofanoff stoma. The main outcome measure was achieving dryness. The original intention of the procedure was also to maintain urethral catheterization.

RESULTS: Full details of the follow-up were available in 17 patients. Despite initial good short-term results, at a median follow up of 7 years, in 14 patients the sling had to be removed because of erosion, often with transient urethral leakage before the bladder neck subsequently closed. A bladder calculus was associated with each case of erosion except one.

CONCLUSION: Although in the short term this technique had favourable results, it was not a useful technique in the long term.

PMID: 14678386