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Re: Evaluation of Need for Salvage Continence Procedures After Failed Modern Staged Repair
1906
PEDIATRIC UROLOGY
Contemporary Epidemiology and Characterization of Newborn Males With Prune Belly Syndrome J. C. Routh, L. Huang, A. B. Retik and C. P. Nelson Department of Urology, Children’s Hospital Boston, Harvard Medical School, Boston, Massachusetts Urology 2010; 76: 44 – 48.
Objectives: Prune belly syndrome (PBS) is a rare condition characterized by the congenital absence or deficiency of the abdominal wall musculature, with associated abnormalities of the genitourinary tract, including hydronephrosis and cryptorchidism. Few population-based epidemiology or mortality data are available. Methods: We retrospectively reviewed the Kids’ Inpatient Database to evaluate PBS among newborn infants during their initial hospitalization in 2000, 2003, and 2006. The International Classification of Diseases, Ninth Revision, Clinical Modification codes were used to identify patients and to determine the comorbidity status. The PBS incidence, demographics, comorbid conditions, and disposition were assessed. Results: A total of 133 newborn male infants diagnosed with PBS were identified of 1,420,991 live male births, for a weighted incidence estimate of 3.8 cases/100,000 live births. Of the newborns with PBS, 50% were white, 31% black, and 10% were Hispanic. In-hospital mortality was high (39 of 133, 29%). Of the 133 patients with PBS, 55 (41%) were discharged home and 39 (29%) required inpatient transfer or home nursing care. Fifty-seven patients (43%) were born premature; 56% of the PBS deaths occurred in premature infants. Mechanical ventilation was required in 64 newborns (48%), and 33 (24%) had coexisting congenital cardiovascular anomalies. Renal failure was uncommon, occurring in only 5 newborns (4%); none required dialysis. Only 13 patients (10%) underwent urinary diversion (vesicostomy or ureterostomy). Conclusions: The incidence of PBS was 3.8 cases/100,000 live births. Despite advances in care for children with PBS, this condition continues to be associated with high perinatal mortality, likely related to the associated prematurity and pulmonary complications. Renal failure was rare, as was immediate urinary diversion. Editorial Comment: Following multivariate analysis, only the presence of pulmonary disorders was associated with hospital mortality during the initial inpatient hospitalization in this recent survey of American boys born with prune belly syndrome. The number of children who likely would have gone on to renal failure (reported here as 4%) may have been much higher, probably closer to the 20% to 30% range that has previously been reported, if the time horizon of the survey had been longer. In this series the proportion of African American newborns with prune belly syndrome was significantly greater than in the general population (31% vs 15%). Conversely the proportion of Hispanic boys with prune belly syndrome was lower than in the general population (10% vs 23%). This finding suggests that prune belly syndrome is somewhat more common in African American newborns and somewhat less common in Hispanics. Douglas A. Canning, M.D.
Re: Evaluation of Need for Salvage Continence Procedures After Failed Modern Staged Repair Y. Kibar, C. C. Roth, R. A. Ashley, D. Frimberger and B. P. Kropp Department of Pediatric Urology, University of Oklahoma Health Science Center, Oklahoma City, Oklahoma Urology 2010; 76: 39 – 42.
Objectives: To assess our experience with salvage continence procedures in patients with bladder exstrophy. Methods: We retrospectively reviewed our surgical records of patients with bladder exstrophy who had undergone failed modern staged repair elsewhere and underwent salvage continence operations at our institute from 1996 to 2008. Results: A total of 16 patients had undergone salvage continence operations. Bladder augmentation was performed in 11 patients (68.8%); 14 children (87.5%) received a continent, catheterizable stoma. In 6 children (37.5%) bladder neck
PEDIATRIC UROLOGY
1907
injections were done. Three patients (18.8%) underwent bladder neck closure combined with other reconstructive procedures. A total of 49 continence procedures were performed, with an average of 3.06 per patient. We included all 16 children ⬎5 years. After these operations, 13 of 16 (81.3%) became continent. Two children were able to void spontaneously and were dry day and night (12.5%). Of the remaining 14 patients, all of whom used clean intermittent catheterization, 11 (68.7%) were continent during the day and night with augmentation and/or a catheterizable stoma. Three patients were incontinent (18.7%). Conclusions: Although the need to perform salvage continence procedures after failed bladder modern staged repair is high, when patients are appropriately selected, continence can be achieved eventually.
Failed Exstrophy Closure: Management and Outcome T. E. Novak, J. P. Costello, R. Orosco, P. D. Sponseller, E. Mack and J. P. Gearhart Division of Pediatric Urology, James Buchanan Brady Urological Institutions, Johns Hopkins Hospital, Johns Hopkins Medical Institutions, Baltimore, Maryland J Pediatr Urol 2010; 6: 381–384.
Objective: In a series of failed exstrophy closures, to identify determinants of successful repeat closure and the impact of failed closure on the fate of the lower urinary tract and continence status. Patients and Methods: We performed a retrospective review of operative notes and medical records of patients with a history of one or more failed exstrophy closures in 1978-2007. The primary surgical endpoints were failure rate of repeat closure attempts, mode of continence surgery and continence outcome. Continence was defined as achieving a dry interval of ⬎3h and voiding through the urethra. Results: We identified 122 patients (85 male/37 female) who had undergone repeat closure following failure. The success rate of repeat closure attempts at our institution was 98%. Of the 94 patients who had undergone successful repeat closure, definitive continence management and had their dryness evaluated, 38 were candidates for bladder neck reconstruction and 17(18%) were continent. Of the remaining patients, 90% were able to attain dryness, but at the expense of clean intermittent catheterization and continent urinary diversion. Conclusion: A failed exstrophy closure has significant implications for long-term surgical outcome. Reclosure can be accomplished in the majority of cases. In comparison to patients with successful primary closure, the rates of urethral continence following successful repeat closure were lower. Editorial Comment: Kibar et al review their experience with children with bladder exstrophy who were referred to their academic center with incontinence following modern staged repair. A total of 49 continence procedures were performed with an average of just more than 3 procedures per patient. The majority required bladder augmentation. Ureteral reimplant was required in nearly all. About half of the patients required a second bladder neck reconstruction. Followup was long (average 9.2 years). Large amounts of resources were devoted to the 16 patients, which is common for an exstrophy series. Ultimately only 2 children (12.5%) voided spontaneously with urinary continence. Novak et al report a large series of children with failed initial bladder closure and describe their journey toward continence or dryness. The authors appropriately distinguish dryness (with intermittent catheterization) from continence (spontaneous voiding) and found that their success achieving true continence following bladder neck reconstruction in those children with failed initial repair was much less likely than following a successful initial closure. Both series illustrate the effort required in boys and girls born with exstrophy with failed first attempts at reconstruction. Few will achieve volitional voiding with continence. Both studies provide evidence to suggest the importance of a good closure despite the initial approach. A failed initial bladder exstrophy closure results in a much more difficult and likely less successful bladder neck reconstruction. Douglas A. Canning, M.D.
PEDIATRIC UROLOGY
Contemporary Epidemiology and Characterization of Newborn Males With Prune Belly Syndrome J. C. Routh, L. Huang, A. B. Retik and C. P. Nelson Department of Urology, Children’s Hospital Boston, Harvard Medical School, Boston, Massachusetts Urology 2010; 76: 44 – 48.
Objectives: Prune belly syndrome (PBS) is a rare condition characterized by the congenital absence or deficiency of the abdominal wall musculature, with associated abnormalities of the genitourinary tract, including hydronephrosis and cryptorchidism. Few population-based epidemiology or mortality data are available. Methods: We retrospectively reviewed the Kids’ Inpatient Database to evaluate PBS among newborn infants during their initial hospitalization in 2000, 2003, and 2006. The International Classification of Diseases, Ninth Revision, Clinical Modification codes were used to identify patients and to determine the comorbidity status. The PBS incidence, demographics, comorbid conditions, and disposition were assessed. Results: A total of 133 newborn male infants diagnosed with PBS were identified of 1,420,991 live male births, for a weighted incidence estimate of 3.8 cases/100,000 live births. Of the newborns with PBS, 50% were white, 31% black, and 10% were Hispanic. In-hospital mortality was high (39 of 133, 29%). Of the 133 patients with PBS, 55 (41%) were discharged home and 39 (29%) required inpatient transfer or home nursing care. Fifty-seven patients (43%) were born premature; 56% of the PBS deaths occurred in premature infants. Mechanical ventilation was required in 64 newborns (48%), and 33 (24%) had coexisting congenital cardiovascular anomalies. Renal failure was uncommon, occurring in only 5 newborns (4%); none required dialysis. Only 13 patients (10%) underwent urinary diversion (vesicostomy or ureterostomy). Conclusions: The incidence of PBS was 3.8 cases/100,000 live births. Despite advances in care for children with PBS, this condition continues to be associated with high perinatal mortality, likely related to the associated prematurity and pulmonary complications. Renal failure was rare, as was immediate urinary diversion. Editorial Comment: Following multivariate analysis, only the presence of pulmonary disorders was associated with hospital mortality during the initial inpatient hospitalization in this recent survey of American boys born with prune belly syndrome. The number of children who likely would have gone on to renal failure (reported here as 4%) may have been much higher, probably closer to the 20% to 30% range that has previously been reported, if the time horizon of the survey had been longer. In this series the proportion of African American newborns with prune belly syndrome was significantly greater than in the general population (31% vs 15%). Conversely the proportion of Hispanic boys with prune belly syndrome was lower than in the general population (10% vs 23%). This finding suggests that prune belly syndrome is somewhat more common in African American newborns and somewhat less common in Hispanics. Douglas A. Canning, M.D.
Re: Evaluation of Need for Salvage Continence Procedures After Failed Modern Staged Repair Y. Kibar, C. C. Roth, R. A. Ashley, D. Frimberger and B. P. Kropp Department of Pediatric Urology, University of Oklahoma Health Science Center, Oklahoma City, Oklahoma Urology 2010; 76: 39 – 42.
Objectives: To assess our experience with salvage continence procedures in patients with bladder exstrophy. Methods: We retrospectively reviewed our surgical records of patients with bladder exstrophy who had undergone failed modern staged repair elsewhere and underwent salvage continence operations at our institute from 1996 to 2008. Results: A total of 16 patients had undergone salvage continence operations. Bladder augmentation was performed in 11 patients (68.8%); 14 children (87.5%) received a continent, catheterizable stoma. In 6 children (37.5%) bladder neck
PEDIATRIC UROLOGY
1907
injections were done. Three patients (18.8%) underwent bladder neck closure combined with other reconstructive procedures. A total of 49 continence procedures were performed, with an average of 3.06 per patient. We included all 16 children ⬎5 years. After these operations, 13 of 16 (81.3%) became continent. Two children were able to void spontaneously and were dry day and night (12.5%). Of the remaining 14 patients, all of whom used clean intermittent catheterization, 11 (68.7%) were continent during the day and night with augmentation and/or a catheterizable stoma. Three patients were incontinent (18.7%). Conclusions: Although the need to perform salvage continence procedures after failed bladder modern staged repair is high, when patients are appropriately selected, continence can be achieved eventually.
Failed Exstrophy Closure: Management and Outcome T. E. Novak, J. P. Costello, R. Orosco, P. D. Sponseller, E. Mack and J. P. Gearhart Division of Pediatric Urology, James Buchanan Brady Urological Institutions, Johns Hopkins Hospital, Johns Hopkins Medical Institutions, Baltimore, Maryland J Pediatr Urol 2010; 6: 381–384.
Objective: In a series of failed exstrophy closures, to identify determinants of successful repeat closure and the impact of failed closure on the fate of the lower urinary tract and continence status. Patients and Methods: We performed a retrospective review of operative notes and medical records of patients with a history of one or more failed exstrophy closures in 1978-2007. The primary surgical endpoints were failure rate of repeat closure attempts, mode of continence surgery and continence outcome. Continence was defined as achieving a dry interval of ⬎3h and voiding through the urethra. Results: We identified 122 patients (85 male/37 female) who had undergone repeat closure following failure. The success rate of repeat closure attempts at our institution was 98%. Of the 94 patients who had undergone successful repeat closure, definitive continence management and had their dryness evaluated, 38 were candidates for bladder neck reconstruction and 17(18%) were continent. Of the remaining patients, 90% were able to attain dryness, but at the expense of clean intermittent catheterization and continent urinary diversion. Conclusion: A failed exstrophy closure has significant implications for long-term surgical outcome. Reclosure can be accomplished in the majority of cases. In comparison to patients with successful primary closure, the rates of urethral continence following successful repeat closure were lower. Editorial Comment: Kibar et al review their experience with children with bladder exstrophy who were referred to their academic center with incontinence following modern staged repair. A total of 49 continence procedures were performed with an average of just more than 3 procedures per patient. The majority required bladder augmentation. Ureteral reimplant was required in nearly all. About half of the patients required a second bladder neck reconstruction. Followup was long (average 9.2 years). Large amounts of resources were devoted to the 16 patients, which is common for an exstrophy series. Ultimately only 2 children (12.5%) voided spontaneously with urinary continence. Novak et al report a large series of children with failed initial bladder closure and describe their journey toward continence or dryness. The authors appropriately distinguish dryness (with intermittent catheterization) from continence (spontaneous voiding) and found that their success achieving true continence following bladder neck reconstruction in those children with failed initial repair was much less likely than following a successful initial closure. Both series illustrate the effort required in boys and girls born with exstrophy with failed first attempts at reconstruction. Few will achieve volitional voiding with continence. Both studies provide evidence to suggest the importance of a good closure despite the initial approach. A failed initial bladder exstrophy closure results in a much more difficult and likely less successful bladder neck reconstruction. Douglas A. Canning, M.D.