The natural history of caliceal diverticula in children is unknown. We review our series of child... more The natural history of caliceal diverticula in children is unknown. We review our series of children with caliceal diverticula to examine the presentation, natural history and management. We retrospectively reviewed the records of 22 children presenting with caliceal diverticula between 1983 and 2006. All pertinent clinical data were recorded, including demographics, imaging studies, treatment and clinical outcome. A total of 22 children had 23 caliceal diverticula. There were 14 girls and 8 boys who presented at a mean age of 5.4 +/- 3.1 years (range 0.2 to 12). At clinical presentation 10 patients had febrile urinary tract infection, 2 had hematuria, 2 had abdominal pain, 1 had flank pain and 8 were asymptomatic. Mean diameter of all caliceal diverticula was 2.2 +/- 1.7 cm. All patients underwent voiding cystourethrogram. Two patients (9%) had concomitant ipsilateral caliceal diverticula and vesicoureteral reflux. A total of 10 patients with caliceal diverticula (43%) were treated at a mean of 3.0 +/- 2.3 years after initial presentation due to symptomatic enlargement in 5, symptomatic calculus in 3, complicated abscess in 1 and urosepsis in 1. Treatment modalities included percutaneous ablation, open marsupialization/ablation, partial nephrectomy and laparoscopic marsupialization/ablation. There were no recurrences during a mean followup of 3.1 +/- 2.9 years (range 0.1 to 10.1) in these 10 patients. In the 13 caliceal diverticula (57%) that were observed mean followup was 6 +/- 3.7 years (range 1.2 to 10.5). These caliceal diverticula were stable in size and remained asymptomatic. Caliceal diverticula in children are rare. Most caliceal diverticula remain stable and asymptomatic but approximately 20% may have symptomatic enlargement that may warrant surgical management. Given the morbidity associated with caliceal diverticula and concomitant vesicoureteral reflux, evaluation for ipsilateral reflux is mandatory. Overall approximately 43% of children with caliceal diverticula require surgical intervention, and various treatment options are available.
This article is a standardization document of the International Children's Continence Society... more This article is a standardization document of the International Children's Continence Society (ICCS); it represent a consensus of ICCS on the management of pediatric daytime urine incontinence (DUI). This document was designed and written by a multi-disciplinary core group of authors appointed by the ICCS' board. Based on evidence of studies and the experience of experts, the treatment guideline of DUI is assembled in this standardization document. Guidelines and the algorithm of management include non-pharmacological treatment (urotherapy), as well as the pharmacological therapy and other modalities that are presented for DUI in general, as along with recommendations for individual conditions. The final document is not a systematic literature review. It includes relevant research when available as well as experts' opinion on the current understanding of daytime incontinence in children. This document illustrates that specific treatment of DUI based on an exact diagnosis...
Congenital anomalies of the kidneys and urinary tract (CAKUT) are the most common cause of chroni... more Congenital anomalies of the kidneys and urinary tract (CAKUT) are the most common cause of chronic kidney disease in the first three decades of life. Identification of single-gene mutations that cause CAKUT permits the first insights into related disease mechanisms. However, for most cases the underlying defect remains elusive. We identified a kindred with an autosomal-dominant form of CAKUT with predominant ureteropelvic junction obstruction. By whole exome sequencing, we identified a heterozygous truncating mutation (c.1010delG) of T-Box transcription factor 18 (TBX18) in seven affected members of the large kindred. A screen of additional families with CAKUT identified three families harboring two heterozygous TBX18 mutations (c.1570C>T and c.487A>G). TBX18 is essential for developmental specification of the ureteric mesenchyme and ureteric smooth muscle cells. We found that all three TBX18 altered proteins still dimerized with the wild-type protein but had prolonged protein...
This pilot study evaluated the safety, feasibility, and usefulness of the Self-Cathing Experience... more This pilot study evaluated the safety, feasibility, and usefulness of the Self-Cathing Experience Journal (SC-EJ), an online resource for patients and families to address issues and stigma surrounding clean intermittent catheterization (CIC). Modeled after previous assessments of the Cardiac and Depression Experience Journals (EJs), this project uniquely included patients and caregivers. We explored whether patients and caregivers would find the SC-EJ helpful in increasing their understanding of CIC, accepting the medical benefits of self-catheterization, improving hopefulness, and diminishing social isolation. Patients seen in a tertiary urology clinic were asked to view the SC-EJ for 30 min and rate its safety and efficacy. The cross-sectional sample included 25 families: 17 surveys were completed by the patient and their caregiver, five by the patient only, and three by the caregiver only. Mean patient age was 15.7 ± 5.8 years (range 7-29 years). The patients were 64% female, and...
Existing survey instruments for bowel dysfunction in the pediatric population are either parent-r... more Existing survey instruments for bowel dysfunction in the pediatric population are either parent-reported or focus on non-neurogenic bowel dysfunction. The purpose of this study was to develop and validate an adolescent-reported survey to assess the severity of bowel dysfunction in spina bifida patients and examine its impact on quality of life (QOL). We performed a cross-sectional study of patients in our Myelodysplasia Program, aged 11-17 years, with a history of constipation and/or fecal incontinence (FI) from November 2010 to June 2013. Control patients, aged 11-17 years, were recruited from the stone clinic. Exclusion criteria were lack of English fluency, insufficient reading skills, or an incontinent fecal diversion. A 29-item version of the Adolescent Fecal Incontinence and Constipation Symptom Index (A-FICSI) was developed with five domains (Figure). Test re-test reliability and correlation with the total global health-related QOL score from the Parkin survey were measured using the Pearson correlation coefficient. A factor analysis model with four-fold correlations was tested. Of the 65 study-eligible individuals approached, 25 (11 boys) completed the A-FICSI (median age 12.6 years, IQR 11.8-14.7 years) and 17 completed another survey on urinary incontinence (64.6% response rate). Twenty-one control patients with nephrolithiasis completed the A-FICSI. Nine of the 25 completed a second administration of the survey. The mean correlation between repeated administrations of the survey was r = 0.43. There was a significant negative correlation between severity of constipation (r = -0.299, p < 0.05) and severity of FI (r = -0.316, p < 0.05) with the total Parkin QOL score. The Comparative Fit Index (CFI) was 0.864. Most items loaded significantly on their respective factors. Between-factor correlations were all significant (>0.30) in the predicted direction. Unstandardized residuals were 8.7% (95% CI 6.4-10.9%). Item reduction was performed on the 29-item instrument based on results of the factor analysis. The finalized instrument contained 21 items. This is the first adolescent-reported bowel dysfunction instrument to undergo formal psychometric assessment in the spina bifida population. The instrument demonstrated adequate reliability and the five-factor structure fit the data well. This study highlights the negative impact of bowel dysfunction on the QOL of spina bifida patients. It is limited by the moderate sample size that is a common issue in relatively rare diseases. The A-FICSI possesses desirable psychometric properties for the measurement of bowel dysfunction in the spina bifida population.
The purpose of this study was to perform a psychometric assessment of the Incontinence Symptom In... more The purpose of this study was to perform a psychometric assessment of the Incontinence Symptom Index-Pediatric (ISI-P) in a cohort of adolescents with spina bifida (SB) and neuropathic urinary incontinence (UI) to test its validity and reliability. The ISI-P, an 11-item instrument with domains for symptom severity and impairment, was self-administered by subjects 11-17 years old with SB and UI. Controls were 11-17 years old, with nephrolithiasis and no history of UI. Formal psychometric assessment included an evaluation of internal consistency, test re-test reliability and factor analysis. Of 78 study-eligible subjects we attempted to contact, 33 (66.7% female) with a median age of 13.1 years completed the ISI-P (42.3% response rate). 21 control patients also completed the ISI-P. Cronbach's alpha was 0.936 and 0.792 for the severity and bother factors respectively. The delta Chi-square test for the two-factor (vs. one-factor) model was significantly [χ(2)(89)=107.823, p<0.05]...
We evaluated the relationship between testicular volume differential, total testis volume and tot... more We evaluated the relationship between testicular volume differential, total testis volume and total motile sperm count in adolescents with varicocele. Prior reports have been inconclusive regarding the association of testis volume with semen analysis parameters. We retrospectively reviewed a single institution database of Tanner 5 adolescents with nonoperated left varicocele with at least 1 semen analysis available. Patients were included in analysis if they had clinical left varicocele, ultrasound measurement of testis volume and no prior inguinal or scrotal surgery. We analyzed the records of 100 patients. Mean ± SD age at presentation was 15.2 ± 3.5 years. The mean testicular volume differential was 8% ± 23% and 31 patients (31%) had a greater than 20% testicular volume differential. Mean total testis volume was 30.4 ± 13.3 cc. Varicocele was grade 3 in 39 patients, grade 2 in 50, grade 1 in 8 and ungraded in 3. Median total motile sperm count in the cohort was 42.0 (IQR 9.2, 105...
We present our experience with diuretic renography in the evaluation of dilatation of the urinary... more We present our experience with diuretic renography in the evaluation of dilatation of the urinary tract in 27 children. The usefulness of this method along with its pitfalls are discussed.
Twenty-two boys with myelodysplasia and incontinence were evaluated urodynamically. Three types o... more Twenty-two boys with myelodysplasia and incontinence were evaluated urodynamically. Three types of bladder function were noted, but each could not be correlated with any particular neuroligic level. The integrity of the external sphincter innervation was determined by electomyographic monitoring of periurethral striated muscle. Bladder sphincter dyssynergia was found in one of the boys with voluntary control, five with involuntary bladder contractions, and five with adynamic bladders who voided by Credé&#39;s method. A radiologically narrow external sphincter on voiding cystography could only be correlated with the bioelectric activity in children with dyssynergia. A narrow sphincter was also noted in three children with synergy and four with complete lower motor neuron lesions. Marked fibrosis was found to be the cause of narrowing of external sphincter area in these boys. Thus, urodynamic evaluation helped define the etiology of outlet obstruction when it was present in the boy with myelodysplasia. A classification of bladder sphincter function is proposed.
Patients with end-stage bladder disease can be treated with cystoplasty using gastrointestinal se... more Patients with end-stage bladder disease can be treated with cystoplasty using gastrointestinal segments. The presence of such segments in the urinary tract has been associated with many complications. We explored an alternative approach using autologous engineered bladder tissues for reconstruction. Seven patients with myelomeningocele, aged 4-19 years, with high-pressure or poorly compliant bladders, were identified as candidates for cystoplasty. A bladder biopsy was obtained from each patient. Urothelial and muscle cells were grown in culture, and seeded on a biodegradable bladder-shaped scaffold made of collagen, or a composite of collagen and polyglycolic acid. About 7 weeks after the biopsy, the autologous engineered bladder constructs were used for reconstruction and implanted either with or without an omental wrap. Serial urodynamics, cystograms, ultrasounds, bladder biopsies, and serum analyses were done. Follow-up range was 22-61 months (mean 46 months). Post-operatively, the mean bladder leak point pressure decrease at capacity, and the volume and compliance increase was greatest in the composite engineered bladders with an omental wrap (56%, 1.58-fold, and 2.79-fold, respectively). Bowel function returned promptly after surgery. No metabolic consequences were noted, urinary calculi did not form, mucus production was normal, and renal function was preserved. The engineered bladder biopsies showed an adequate structural architecture and phenotype. Engineered bladder tissues, created with autologous cells seeded on collagen-polyglycolic acid scaffolds, and wrapped in omentum after implantation, can be used in patients who need cystoplasty.
Sacral agenesis is a rare congenital anomaly of the lower vertebral column which usually produces... more Sacral agenesis is a rare congenital anomaly of the lower vertebral column which usually produces lower urinary tract dysfunction. Sixteen children with varying degrees of sacral agenesis and urinary symptoms were extensively evaluated. The neurologic lesion varied from no denervation to a complete loss of sacral motor and sensory function. The treatment instituted was individualized and based on specific urodynamic and radiologic findings. Overall, 12 of the 16 children (75%) achieved continence. Clues to the diagnosis including symptoms, physical findings, and the relationship to maternal diabetes are discussed.
The results of surgery for megaureter are reviewed in 40 children. Special attention is focused o... more The results of surgery for megaureter are reviewed in 40 children. Special attention is focused on the etiologic causes of these dilated ureters in relation to the surgical results. Thirty of 31 children with obstructive or refluxing megaureters and normal bladders had satisfactory results. Less satisfactory results occurred in children with abnormal bladder dynamics. No child in this series required upper ureteral tapering. A discussion of the causes and further management of the surgical failures is presented.
This workshop was conducted in an attempt to analyze critically the role of reconstruction of the... more This workshop was conducted in an attempt to analyze critically the role of reconstruction of the myelodysplastic patient who had undergone urinary diversion and to develop guidelines for selecting those patients in whom urinary undiversion might be undertaken safely. The collective experience initially seems to be acceptable; however, the authors emphasize the gravity of the decision and the complexity of the evaluation which must be undertaken prior to embarking on such reconstructive surgery. Contrary to some reports, we believe that the defunctionalized bladder frequently can be evaluated. Further, many of the contraindications to urinary undiversion have been identified and several of the hazards involved therein can be avoided. We believe that the neurogenic bladder is no longer an absolute contraindication to undiversion. Our experience suggests that undiversion is a reasonable surgical treatment in select patient with neurogenic bladder dysfunction. But, the decision to remove a satisfactorily functioning conduit must not be undertaken lightly. Patients should be selected only after a thorough, detailed, and properly conducted evaluation. A protocol has been developed which will hopefully assist in this evaluation. Perhaps additional shared experience will further refine and delineate the circumstances appropriate for reconstruction of these patients.
The natural history of caliceal diverticula in children is unknown. We review our series of child... more The natural history of caliceal diverticula in children is unknown. We review our series of children with caliceal diverticula to examine the presentation, natural history and management. We retrospectively reviewed the records of 22 children presenting with caliceal diverticula between 1983 and 2006. All pertinent clinical data were recorded, including demographics, imaging studies, treatment and clinical outcome. A total of 22 children had 23 caliceal diverticula. There were 14 girls and 8 boys who presented at a mean age of 5.4 +/- 3.1 years (range 0.2 to 12). At clinical presentation 10 patients had febrile urinary tract infection, 2 had hematuria, 2 had abdominal pain, 1 had flank pain and 8 were asymptomatic. Mean diameter of all caliceal diverticula was 2.2 +/- 1.7 cm. All patients underwent voiding cystourethrogram. Two patients (9%) had concomitant ipsilateral caliceal diverticula and vesicoureteral reflux. A total of 10 patients with caliceal diverticula (43%) were treated at a mean of 3.0 +/- 2.3 years after initial presentation due to symptomatic enlargement in 5, symptomatic calculus in 3, complicated abscess in 1 and urosepsis in 1. Treatment modalities included percutaneous ablation, open marsupialization/ablation, partial nephrectomy and laparoscopic marsupialization/ablation. There were no recurrences during a mean followup of 3.1 +/- 2.9 years (range 0.1 to 10.1) in these 10 patients. In the 13 caliceal diverticula (57%) that were observed mean followup was 6 +/- 3.7 years (range 1.2 to 10.5). These caliceal diverticula were stable in size and remained asymptomatic. Caliceal diverticula in children are rare. Most caliceal diverticula remain stable and asymptomatic but approximately 20% may have symptomatic enlargement that may warrant surgical management. Given the morbidity associated with caliceal diverticula and concomitant vesicoureteral reflux, evaluation for ipsilateral reflux is mandatory. Overall approximately 43% of children with caliceal diverticula require surgical intervention, and various treatment options are available.
This article is a standardization document of the International Children's Continence Society... more This article is a standardization document of the International Children's Continence Society (ICCS); it represent a consensus of ICCS on the management of pediatric daytime urine incontinence (DUI). This document was designed and written by a multi-disciplinary core group of authors appointed by the ICCS' board. Based on evidence of studies and the experience of experts, the treatment guideline of DUI is assembled in this standardization document. Guidelines and the algorithm of management include non-pharmacological treatment (urotherapy), as well as the pharmacological therapy and other modalities that are presented for DUI in general, as along with recommendations for individual conditions. The final document is not a systematic literature review. It includes relevant research when available as well as experts' opinion on the current understanding of daytime incontinence in children. This document illustrates that specific treatment of DUI based on an exact diagnosis...
Congenital anomalies of the kidneys and urinary tract (CAKUT) are the most common cause of chroni... more Congenital anomalies of the kidneys and urinary tract (CAKUT) are the most common cause of chronic kidney disease in the first three decades of life. Identification of single-gene mutations that cause CAKUT permits the first insights into related disease mechanisms. However, for most cases the underlying defect remains elusive. We identified a kindred with an autosomal-dominant form of CAKUT with predominant ureteropelvic junction obstruction. By whole exome sequencing, we identified a heterozygous truncating mutation (c.1010delG) of T-Box transcription factor 18 (TBX18) in seven affected members of the large kindred. A screen of additional families with CAKUT identified three families harboring two heterozygous TBX18 mutations (c.1570C>T and c.487A>G). TBX18 is essential for developmental specification of the ureteric mesenchyme and ureteric smooth muscle cells. We found that all three TBX18 altered proteins still dimerized with the wild-type protein but had prolonged protein...
This pilot study evaluated the safety, feasibility, and usefulness of the Self-Cathing Experience... more This pilot study evaluated the safety, feasibility, and usefulness of the Self-Cathing Experience Journal (SC-EJ), an online resource for patients and families to address issues and stigma surrounding clean intermittent catheterization (CIC). Modeled after previous assessments of the Cardiac and Depression Experience Journals (EJs), this project uniquely included patients and caregivers. We explored whether patients and caregivers would find the SC-EJ helpful in increasing their understanding of CIC, accepting the medical benefits of self-catheterization, improving hopefulness, and diminishing social isolation. Patients seen in a tertiary urology clinic were asked to view the SC-EJ for 30 min and rate its safety and efficacy. The cross-sectional sample included 25 families: 17 surveys were completed by the patient and their caregiver, five by the patient only, and three by the caregiver only. Mean patient age was 15.7 ± 5.8 years (range 7-29 years). The patients were 64% female, and...
Existing survey instruments for bowel dysfunction in the pediatric population are either parent-r... more Existing survey instruments for bowel dysfunction in the pediatric population are either parent-reported or focus on non-neurogenic bowel dysfunction. The purpose of this study was to develop and validate an adolescent-reported survey to assess the severity of bowel dysfunction in spina bifida patients and examine its impact on quality of life (QOL). We performed a cross-sectional study of patients in our Myelodysplasia Program, aged 11-17 years, with a history of constipation and/or fecal incontinence (FI) from November 2010 to June 2013. Control patients, aged 11-17 years, were recruited from the stone clinic. Exclusion criteria were lack of English fluency, insufficient reading skills, or an incontinent fecal diversion. A 29-item version of the Adolescent Fecal Incontinence and Constipation Symptom Index (A-FICSI) was developed with five domains (Figure). Test re-test reliability and correlation with the total global health-related QOL score from the Parkin survey were measured using the Pearson correlation coefficient. A factor analysis model with four-fold correlations was tested. Of the 65 study-eligible individuals approached, 25 (11 boys) completed the A-FICSI (median age 12.6 years, IQR 11.8-14.7 years) and 17 completed another survey on urinary incontinence (64.6% response rate). Twenty-one control patients with nephrolithiasis completed the A-FICSI. Nine of the 25 completed a second administration of the survey. The mean correlation between repeated administrations of the survey was r = 0.43. There was a significant negative correlation between severity of constipation (r = -0.299, p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.05) and severity of FI (r = -0.316, p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.05) with the total Parkin QOL score. The Comparative Fit Index (CFI) was 0.864. Most items loaded significantly on their respective factors. Between-factor correlations were all significant (&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;0.30) in the predicted direction. Unstandardized residuals were 8.7% (95% CI 6.4-10.9%). Item reduction was performed on the 29-item instrument based on results of the factor analysis. The finalized instrument contained 21 items. This is the first adolescent-reported bowel dysfunction instrument to undergo formal psychometric assessment in the spina bifida population. The instrument demonstrated adequate reliability and the five-factor structure fit the data well. This study highlights the negative impact of bowel dysfunction on the QOL of spina bifida patients. It is limited by the moderate sample size that is a common issue in relatively rare diseases. The A-FICSI possesses desirable psychometric properties for the measurement of bowel dysfunction in the spina bifida population.
The purpose of this study was to perform a psychometric assessment of the Incontinence Symptom In... more The purpose of this study was to perform a psychometric assessment of the Incontinence Symptom Index-Pediatric (ISI-P) in a cohort of adolescents with spina bifida (SB) and neuropathic urinary incontinence (UI) to test its validity and reliability. The ISI-P, an 11-item instrument with domains for symptom severity and impairment, was self-administered by subjects 11-17 years old with SB and UI. Controls were 11-17 years old, with nephrolithiasis and no history of UI. Formal psychometric assessment included an evaluation of internal consistency, test re-test reliability and factor analysis. Of 78 study-eligible subjects we attempted to contact, 33 (66.7% female) with a median age of 13.1 years completed the ISI-P (42.3% response rate). 21 control patients also completed the ISI-P. Cronbach's alpha was 0.936 and 0.792 for the severity and bother factors respectively. The delta Chi-square test for the two-factor (vs. one-factor) model was significantly [χ(2)(89)=107.823, p<0.05]...
We evaluated the relationship between testicular volume differential, total testis volume and tot... more We evaluated the relationship between testicular volume differential, total testis volume and total motile sperm count in adolescents with varicocele. Prior reports have been inconclusive regarding the association of testis volume with semen analysis parameters. We retrospectively reviewed a single institution database of Tanner 5 adolescents with nonoperated left varicocele with at least 1 semen analysis available. Patients were included in analysis if they had clinical left varicocele, ultrasound measurement of testis volume and no prior inguinal or scrotal surgery. We analyzed the records of 100 patients. Mean ± SD age at presentation was 15.2 ± 3.5 years. The mean testicular volume differential was 8% ± 23% and 31 patients (31%) had a greater than 20% testicular volume differential. Mean total testis volume was 30.4 ± 13.3 cc. Varicocele was grade 3 in 39 patients, grade 2 in 50, grade 1 in 8 and ungraded in 3. Median total motile sperm count in the cohort was 42.0 (IQR 9.2, 105...
We present our experience with diuretic renography in the evaluation of dilatation of the urinary... more We present our experience with diuretic renography in the evaluation of dilatation of the urinary tract in 27 children. The usefulness of this method along with its pitfalls are discussed.
Twenty-two boys with myelodysplasia and incontinence were evaluated urodynamically. Three types o... more Twenty-two boys with myelodysplasia and incontinence were evaluated urodynamically. Three types of bladder function were noted, but each could not be correlated with any particular neuroligic level. The integrity of the external sphincter innervation was determined by electomyographic monitoring of periurethral striated muscle. Bladder sphincter dyssynergia was found in one of the boys with voluntary control, five with involuntary bladder contractions, and five with adynamic bladders who voided by Credé&#39;s method. A radiologically narrow external sphincter on voiding cystography could only be correlated with the bioelectric activity in children with dyssynergia. A narrow sphincter was also noted in three children with synergy and four with complete lower motor neuron lesions. Marked fibrosis was found to be the cause of narrowing of external sphincter area in these boys. Thus, urodynamic evaluation helped define the etiology of outlet obstruction when it was present in the boy with myelodysplasia. A classification of bladder sphincter function is proposed.
Patients with end-stage bladder disease can be treated with cystoplasty using gastrointestinal se... more Patients with end-stage bladder disease can be treated with cystoplasty using gastrointestinal segments. The presence of such segments in the urinary tract has been associated with many complications. We explored an alternative approach using autologous engineered bladder tissues for reconstruction. Seven patients with myelomeningocele, aged 4-19 years, with high-pressure or poorly compliant bladders, were identified as candidates for cystoplasty. A bladder biopsy was obtained from each patient. Urothelial and muscle cells were grown in culture, and seeded on a biodegradable bladder-shaped scaffold made of collagen, or a composite of collagen and polyglycolic acid. About 7 weeks after the biopsy, the autologous engineered bladder constructs were used for reconstruction and implanted either with or without an omental wrap. Serial urodynamics, cystograms, ultrasounds, bladder biopsies, and serum analyses were done. Follow-up range was 22-61 months (mean 46 months). Post-operatively, the mean bladder leak point pressure decrease at capacity, and the volume and compliance increase was greatest in the composite engineered bladders with an omental wrap (56%, 1.58-fold, and 2.79-fold, respectively). Bowel function returned promptly after surgery. No metabolic consequences were noted, urinary calculi did not form, mucus production was normal, and renal function was preserved. The engineered bladder biopsies showed an adequate structural architecture and phenotype. Engineered bladder tissues, created with autologous cells seeded on collagen-polyglycolic acid scaffolds, and wrapped in omentum after implantation, can be used in patients who need cystoplasty.
Sacral agenesis is a rare congenital anomaly of the lower vertebral column which usually produces... more Sacral agenesis is a rare congenital anomaly of the lower vertebral column which usually produces lower urinary tract dysfunction. Sixteen children with varying degrees of sacral agenesis and urinary symptoms were extensively evaluated. The neurologic lesion varied from no denervation to a complete loss of sacral motor and sensory function. The treatment instituted was individualized and based on specific urodynamic and radiologic findings. Overall, 12 of the 16 children (75%) achieved continence. Clues to the diagnosis including symptoms, physical findings, and the relationship to maternal diabetes are discussed.
The results of surgery for megaureter are reviewed in 40 children. Special attention is focused o... more The results of surgery for megaureter are reviewed in 40 children. Special attention is focused on the etiologic causes of these dilated ureters in relation to the surgical results. Thirty of 31 children with obstructive or refluxing megaureters and normal bladders had satisfactory results. Less satisfactory results occurred in children with abnormal bladder dynamics. No child in this series required upper ureteral tapering. A discussion of the causes and further management of the surgical failures is presented.
This workshop was conducted in an attempt to analyze critically the role of reconstruction of the... more This workshop was conducted in an attempt to analyze critically the role of reconstruction of the myelodysplastic patient who had undergone urinary diversion and to develop guidelines for selecting those patients in whom urinary undiversion might be undertaken safely. The collective experience initially seems to be acceptable; however, the authors emphasize the gravity of the decision and the complexity of the evaluation which must be undertaken prior to embarking on such reconstructive surgery. Contrary to some reports, we believe that the defunctionalized bladder frequently can be evaluated. Further, many of the contraindications to urinary undiversion have been identified and several of the hazards involved therein can be avoided. We believe that the neurogenic bladder is no longer an absolute contraindication to undiversion. Our experience suggests that undiversion is a reasonable surgical treatment in select patient with neurogenic bladder dysfunction. But, the decision to remove a satisfactorily functioning conduit must not be undertaken lightly. Patients should be selected only after a thorough, detailed, and properly conducted evaluation. A protocol has been developed which will hopefully assist in this evaluation. Perhaps additional shared experience will further refine and delineate the circumstances appropriate for reconstruction of these patients.
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