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2.
J Pediatr Urol ; 2020 Mar 29.
Artigo em Inglês | MEDLINE | ID: mdl-32340883

RESUMO

INTRODUCTION: After unsuccessful repair of bladder exstrophy, when to repeat surgical intervention is unclear. One must balance time required for tissue healing with the damaging effects of an exposed urothelium to the environment. OBJECTIVE: The authors aim to study whether a relationship exists between bladder growth/capacity and time till eventual successful closure. STUDY DESIGN: An institutional database of exstrophy-epispadias complex patients was queried for failed exstrophy closure with successful repeat reconstruction, at least three consecutive bladder capacity measurements, and measurements obtained at least three months following successful closure. Patients closed successfully in the neonatal period were used as a comparative group. Linear mixed effects models were used to study the effect of time and age on bladder capacity. RESULTS: Forty-seven patients requiring reclosure and 117 who had successful neonatal closures were included. Two models were created. The first linear mixed effects model found that for a given age, the bladder capacity declined approximately 9.6 mL per year (p = 0.016). The second model found that when time to successful closure was grouped by quartiles, compared to neonates, those in the fourth quartile had significantly decreased bladder capacity of 28.8 cc (p = 0.042). An interaction model comparing neonates and those requiring reclosure did not demonstrate a significant change in bladder growth rate (p = 0.098). A model stratified by quartiles similarly did not find any significant impact to bladder growth rate. DISCUSSION: From the general linear mixed effects models, the authors conclude when compared to neonates, (1) there was an approximate 9.6 cc loss of total bladder capacity per year taken until successful closure, and that (2) those who were delayed the longest had the most significant difference in bladder capacity. This study required stricter inclusion criteria compared to previous publications, and therefore the conclusions that can be drawn regarding bladder growth rates may be more reliable. Future studies will examine the effects of delayed closure on the bladder at the cellular level. CONCLUSIONS: There is a demonstrable significant impact on overall bladder capacity with increasing delay to successful reclosure. One should be cautious when prolonging reconstruction of the bladder as these data demonstrate a time dependent decline in overall capacity.

3.
Urology ; 136: e1-e2, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31758982

RESUMO

Posterior urethral valves (PUVs) present clinically across a varied spectrum, ranging from severe obstruction with massive distension of the bladder and upper urinary tracts in the fetus, to a much more indolent course with minor secondary changes in the young or older child. Type III (diaphragm) PUVs are relatively uncommon, and are associated with difficulty in passing a transurethral catheter into the bladder as well as smooth dilation of the bladder. The following case examines the unusual experiences of both diagnosing PUVs in a teenager, and capturing visual evidence of type III valves during cystourethroscopy.


Assuntos
Obstrução Uretral/diagnóstico , Adolescente , Cistoscopia , Humanos , Masculino
4.
J Urol ; 203(1): 200-205, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31437120

RESUMO

PURPOSE: We investigated surgical approaches to urinary incontinence and long-term continence outcomes after successful bladder reconstruction in a heterogeneous patient population with classic bladder exstrophy. We hypothesized that while most patients will achieve urinary continence after surgery, only a select group will void volitionally per urethra. MATERIALS AND METHODS: An institutional database of 1,323 patients with exstrophy-epispadias complex was reviewed for patients with classic bladder exstrophy who underwent successful bladder closure and a subsequent continence procedure between 1975 and 2017. Procedures included bladder neck reconstruction, bladder neck reconstruction with augmentation cystoplasty or continent catheterizable stoma, and bladder neck closure with continent catheterizable stoma. Cloacal exstrophy, epispadias and variant exstrophy cases were excluded from analysis. Continence at last followup was defined as a dry interval of 3 or more hours without nighttime leakage. Those patients with more than 3 months of followup were assessed. RESULTS: Overall 432 patients underwent successful bladder closure (primary 71.5%, repeat 28.5%) and a urinary continence procedure. At last followup 162 (37%) underwent bladder neck reconstruction, 76 (18%) underwent bladder neck reconstruction with augmentation cystoplasty or continent catheterizable stoma, 173 (40%) underwent bladder neck closure with continent catheterizable stoma and 18 underwent other procedures. Median followup from the first continence procedure was 7.2 years (IQR 2.3-13.7). Continence was assessed in 350 patients. After isolated bladder neck reconstruction 91 of 142 patients were continent (64%, 95% CI 56-72). After bladder neck closure with continent catheterizable stoma 124 of 133 patients evaluated were continent (93%, 95% CI 87-97). CONCLUSIONS: Most patients with classic bladder exstrophy require multiple reconstructive procedures to achieve continence. Only about 25% of patients are expected to void normally per urethra without reliance on catheterization or urinary diversion.


Assuntos
Extrofia Vesical/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Incontinência Urinária/cirurgia , Procedimentos Cirúrgicos Urológicos/métodos , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Masculino
5.
Urology ; 137: 146-151, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31887351

RESUMO

OBJECTIVE: To explore a series of classic bladder exstrophy (CBE) cases referred to the authors' institution where primary closure with penile disassembly epispadias repair was complicated by penile injury. The penile disassembly technique is frequently combined with bladder closure in patients with CBE undergoing the complete primary repair of exstrophy (CPRE). Penile disassembly has been posited as a risk for penile injury by ischemic mechanisms. METHODS: A prospectively-maintained institutional database of 1337 exstrophy-epispadias complex patients was reviewed for CPRE cases referred to the authors' institution, and those with injury to the penis were identified. The location, extent of injury, and subsequent management is reported. RESULTS: One hundred and thirteen male CBE patients were referred after prior CPRE. Twenty-six (20%) were identified with penile loss and reviewed. Eighty-one percent were closed in the neonatal period, and 54% had a pelvic osteotomy. Median follow-up time was 9.9 years (range 0.6-21.3). Of 26 patients with penile loss, 77% had unilateral loss and in 23% had bilateral loss involving the glans and/or one or both corpora cavernosa. Three patients were successfully managed with myocutaneous neophalloplasty. CONCLUSION: Complete penile disassembly during bladder exstrophy closure may lead to penile injury. This major complication questions the continued application of complete penile disassembly in the reconstruction of bladder exstrophy.


Assuntos
Extrofia Vesical/cirurgia , Epispadia/cirurgia , Complicações Intraoperatórias , Doenças do Pênis , Pênis , Procedimentos Cirúrgicos Urológicos , Atrofia , Criança , Humanos , Recém-Nascido , Complicações Intraoperatórias/diagnóstico , Complicações Intraoperatórias/cirurgia , Masculino , Avaliação de Processos e Resultados em Cuidados de Saúde , Doenças do Pênis/diagnóstico , Doenças do Pênis/etiologia , Doenças do Pênis/cirurgia , Pênis/irrigação sanguínea , Pênis/lesões , Pênis/patologia , Pênis/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Reoperação/métodos , Fatores de Risco , Procedimentos Cirúrgicos Urológicos/efeitos adversos , Procedimentos Cirúrgicos Urológicos/métodos , Adulto Jovem
6.
Urology ; 131: 211-216, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31176739

RESUMO

OBJECTIVE: To compare the surgical subspecialties performing bladder exstrophy closures and characterize their practice patterns using both a national and institutional database. METHODS: The National Surgical Quality Improvement Program Pediatric (NSQIPP) database was reviewed for all bladder exstrophy closures performed from 2012 to 2017. A single institutional exstrophy-epispadias complex database of 1337 patients was reviewed for patients with a bladder closure at a referring institution from 1975 to 2018. Patients with cloacal exstrophy were excluded. The subspecialties of the surgeons performing the closures were identified. Practice patterns such as the use of a pelvic osteotomy and postoperative immobilization, and perioperative outcomes were compared for each subspecialty group. RESULTS: A total of 84 bladder exstrophy patients from NSQIPP and 263 from the author's institutional database met the inclusion criteria. From NSQIPP, 88% of closures were performed by pediatric urologists while 12% were done by other subspecialists. From the institutional database, 75% of referred bladder exstrophy closures were done by a pediatric urologist, and 25% by other services. Gender, race, operation time, length of stay, and postoperative complications were not significantly different between the groups. In one database, pediatric surgeons performed closures earlier, and in another database, pediatric urologists had greater utilization of osteotomy with different immobilization techniques. Pediatric urologists had a higher success rate. CONCLUSION: Pediatric urologists performed the most bladder exstrophy closures in both databases; they operated on more delayed closures with a greater use of adjunctive procedures and a higher success rate. Differences in surgical training may contribute to the differences in practice patterns.


Assuntos
Extrofia Vesical/cirurgia , Padrões de Prática Médica/normas , Especialidades Cirúrgicas , Procedimentos Cirúrgicos Urológicos/normas , Urologia/normas , Bases de Dados Factuais , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Melhoria de Qualidade , Estudos Retrospectivos , Estados Unidos
7.
J Pediatr Surg ; 54(11): 2408-2412, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31079865

RESUMO

PURPOSE: Due to the large abdominal defect from the omphalocele and extreme pubic diastasis in cloacal exstrophy (CE), bioprosthetic material may be used to bridge this gap during abdominal closure in CE. This study examined presurgical factors associated with the use of bioprosthetic materials in CE closure and complications in these patients. METHODS: An institutional database of exstrophy-epispadias complex patients was reviewed for CE. Inclusion criteria included CE and primary closure performed at the host institution from 1998 to 2018. Data collection included demographics, presurgical factors, use of bioprosthetic material, complications, and outcomes. RESULTS: All 32 patients had a staged closure and pelvic osteotomy prior to bladder closure. Ten of the 32 patients incorporated a bioprosthetic material during abdominal wall closure. There is at least 3 months follow up for all patients, all had successful bladder closure without any postoperative hernias. Those who underwent closure without bioprosthetic material were younger at the time of closure (565 vs 693 days, p = 0.043). The differences in complication rates and mean pubic diastasis was not statistically significant, p = 0.079 and p = 0.457 respectively. CONCLUSIONS: The use of bioprosthetic material is associated with older age at abdominal wall and bladder closure. The use of bioprosthetic material is a useful adjunct for secure abdominal wall closure in the reconstruction of CE. TYPE OF STUDY: Prognostic. LEVEL OF EVIDENCE: III.


Assuntos
Abdominoplastia/métodos , Anus Imperfurado/cirurgia , Bioprótese , Extrofia Vesical/cirurgia , Hérnia Umbilical/cirurgia , Escoliose/cirurgia , Anormalidades Urogenitais/cirurgia , Parede Abdominal/cirurgia , Pré-Escolar , Epispadia/cirurgia , Feminino , Humanos , Lactente , Masculino , Osteotomia , Estudos Retrospectivos
8.
J Urol ; 202(2): 406-412, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30840542

RESUMO

PURPOSE: We determined the safety and efficacy of intraoperative magnetic resonance imaging guided surgical reconstruction of bladder exstrophy for the identification of the urogenital diaphragm fibers and the thickened muscular attachments between the posterior urethra, bladder plate and pubic rami. MATERIALS AND METHODS: Institutional review board and U.S. Food and Drug Administration approval was obtained for the use of Brainlab (Munich, Germany) intraoperative magnetic resonance imaging guided navigation of the pelvic floor anatomy during closure of classic bladder exstrophy and cloacal exstrophy at our institution. Preoperative pelvic 3-dimensional magnetic resonance imaging was obtained 1 day before closure in patients undergoing pelvic osteotomies. Intraoperative registration was performed after preoperative planning with a pediatric radiologist using 5 anatomical landmarks immediately before initiation of surgery. Accuracy of pelvic anatomy identification was assessed by 2 pediatric urological surgeons and 1 pediatric radiologist. RESULTS: In 43 patients with classic bladder exstrophy and 4 patients with cloacal exstrophy closed at our institution, Brainlab technology was used successfully to navigate and guide the dissection of the pelvic floor intraoperatively. In all patients there was 100% accuracy in the correlation of gross anatomical landmarks with 3-dimensional magnetic resonance imaging identified landmarks intraoperatively, and all patients had successful closure without any major complications. CONCLUSIONS: Brainlab intraoperative 3-dimensional magnetic resonance imaging guided pelvic floor navigation and dissection is an effective way to accurately identify pelvic anatomy during classic bladder exstrophy and cloacal exstrophy closure. This technology offers a unique opportunity for surgical skill education in this complex reconstructive operation.


Assuntos
Extrofia Vesical/diagnóstico por imagem , Extrofia Vesical/cirurgia , Imageamento Tridimensional , Imagem por Ressonância Magnética , Diafragma da Pelve/diagnóstico por imagem , Diafragma da Pelve/cirurgia , Cirurgia Assistida por Computador , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Imagem por Ressonância Magnética/métodos , Masculino , Resultado do Tratamento , Procedimentos Cirúrgicos Urológicos/efeitos adversos , Procedimentos Cirúrgicos Urológicos/métodos
9.
J Pediatr Surg ; 54(11): 2416-2420, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30879754

RESUMO

PURPOSE: Cloacal exstrophy (CE) is the most severe presentation of the Exstrophy-Epispadias Complex (EEC) and is associated with an omphalocele, making the bladder and abdominal wall closure difficult. If the bladder closure fails, a secondary closure is necessary. The objective of this study is to identify patient or surgical factors associated with a successful secondary closure. METHODS: The institution's EEC database was reviewed for CE patients between 1975 and 2015. Inclusion criteria included a failed primary bladder closure with a secondary closure. Patient demographics, surgical factors and outcomes of the secondary bladder closure were reviewed. RESULTS: Twenty-four patients met inclusion criteria. 8/8 patients had a successful two-staged closure at the author's institution (100%); 2/16 patients had a successful closure at an outside institution (12.5%). Older median age at secondary closure was associated with outcome, p = 0.045. Pelvic osteotomy was associated with successful secondary closure, p = 0.013. Using Buck's immobilization with external fixation was associated with a higher proportion of successful secondary closures compared to Spica cast, p = 0.012. CONCLUSION: Successful reclosure in CE patients is associated with the use of osteotomy as well as Buck's immobilization with external fixation. While successful reclosure can be achieved, it is often at the cost of multiple procedures and, therefore, all efforts should be expended to achieve a successful primary closure. TYPE OF STUDY: Prognostic. LEVEL OF EVIDENCE: III.


Assuntos
Anus Imperfurado/cirurgia , Extrofia Vesical/cirurgia , Hérnia Umbilical/cirurgia , Escoliose/cirurgia , Anormalidades Urogenitais/cirurgia , Procedimentos Cirúrgicos Urológicos , Epispadia/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Osteotomia , Prognóstico , Estudos Retrospectivos , Falha de Tratamento
10.
Urol Case Rep ; 23: 101-102, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30740310

RESUMO

Circumcision is often the earliest surgery performed in a young male's life. Though complications in this procedure are rare, prolong postoperative bleeding may be the first sign of undiagnosed hemophilia. Hemophilia is a rare X-linked bleeding disorder and if not treated prophylactically or promptly during surgical intervention can be fatal. In this case presentation we describe the diagnosis of hemophilia in a child presenting with postoperative bleeding from circumcision. We review the literature regarding the history of this disease with early surgery and highlight the current treatments.

11.
Urology ; 125: 190, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30798969
12.
Urology ; 125: 184-190, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30576745

RESUMO

OBJECTIVE: To investigate the diagnosis, surgical management, and outcomes in patients with variant EEC. Variant presentations of the exstrophy-epispadias complex (EEC) span a wide range of abnormalities. The rarity and diversity of EEC variants can lead to challenges in the diagnosis and subsequent management of this population. METHODS: The authors reviewed an institutional database of 1336 EEC patients from 1975 to 2018 for variant presentations of EEC. Variant presentations included those with skin covered bladder exstrophy (BE), duplicate bladders, superior vesical fistula, and epispadias with major bladder prolapse. Surgical management and outcomes were assessed. RESULTS: In total, 44 EEC variants were identified. Nineteen (43%) presented with a skin-covered BE variant. Five patients presented with duplicate BE, while 6 presented with superior vesical fistula. Fourteen patients (32%) presented with epispadias with major bladder prolapse. Overall, 36 (82%) EEC variants underwent primary bladder closure, at a median of 135 days after birth (range 1-2010), with 21 (58%) undergoing pelvic osteotomy. Primary closures were successful in 89% of cases. Continence procedures were performed in 17 patients. This includes 5 patients who underwent bladder augmentation. However even without a continence procedure, continence with volitional voiding was found in 8 patients. CONCLUSION: The most common EEC variant is the skin-covered form of BE. In order to expedite appropriate management, accurate diagnosis upon initial presentation is crucial. Still, successful surgical reconstruction often results in continence that is similar to, or better than, nonvariant EEC presentations.


Assuntos
Extrofia Vesical/diagnóstico , Extrofia Vesical/cirurgia , Epispadia/diagnóstico , Epispadia/cirurgia , Extrofia Vesical/classificação , Pré-Escolar , Epispadia/classificação , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Fatores de Tempo , Resultado do Tratamento
13.
J Pediatr Urol ; 14(5): 427.e1-427.e7, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29909193

RESUMO

INTRODUCTION: Newborns with classic bladder exstrophy (CBE) may present with a bladder template that is inadequate for closure in the neonatal period (figure). In these cases, a delayed primary closure (DPC) is conducted to permit growth of the bladder template. This study reports the surgical and long-term urinary continence outcomes of poor template CBE patients undergoing DPC and compares them to patients who underwent DPC for reasons unrelated to bladder quality (i.e., prematurity, comorbidities, or a late referral). METHODS: An institutionally approved, prospectively maintained database of 1330 exstrophy-epispadias complex patients was reviewed for CBE patients who underwent DPC at the authors' institution. A bladder template was considered inadequate for neonatal closure if found to be inelastic, <3 cm in diameter, and/or covered in hamartomatous polyps. RESULTS: In total, 63 patients (53 male and 10 female) undergoing DPC were identified. Of these, 36 had poor bladder templates (group 1). The remaining 27 patients (group 2) had adequate templates and their bladder closure was delayed for reasons unrelated to bladder quality. At the time of DPC, those in group 1 were relatively than those in group 2 (median of 229 vs. 128 days, p = 0.094). All 36 group 1 patients and 26 (96%) group 2 patients underwent pelvic osteotomy during DPC (p = 0.429). All patients in this study had a successful primary closure. There was little difference in longitudinal bladder capacities between group 1 and group 2 (p = 0.518). Also, there was minimal difference in the median number of continence procedures between groups, with both groups having 1 (IQR 1-1) continence procedure (p = 0.880). Eight patients in group 1, and three patients in group 2 underwent a bladder neck transection with urinary diversion. Of the 13 and 16 patients who have undergone a continence procedure in group 1 and 2, respectively, 11 (84.6%) and 13 (81.3%) are continent of urine. The age of first continence procedure was different between groups 1 and 2 at 8.0 years (5.8-9.9 years) and 4.8 (3.5-6.0 years), respectively p = 0.009. The majority of patients in group 1 established continence at a relatively later age when compared to those in group 2, at 11.4 (8.0-14.8) years and 7.9 (2.6-13.2) years of age respectively p = 0.087. DISCUSSION: In the authors' view, neonatal bladder closure is ideal for CBE patients as it minimizes potential damage to exposed bladder mucosa. However, prior studies indicate that the rate of bladder growth for patients undergoing a delayed primary closure does not differ from patients with a neonatal closure. Results from this study show continued evidence that patients with poor templates who undergo delayed closure have excellent primary closure outcomes, which is critical for further management. Furthermore, this study shows that an inadequate bladder does not affect DPC outcomes or the continence outcomes in DPC patients. However, the inadequate template does affect the type of continence procedure available to a DPC patient, the age of first continence procedure, and the age of continence. CONCLUSIONS: DPC of the exstrophic bladder has a high rate of success when pelvic osteotomy is utilized as an adjunct. Patients having a DPC for reasons of an inadequate bladder template have comparable rates of bladder growth when compared to DPC of an adequate bladder template. The inadequate bladder template affects the type of continence procedure, with the majority of patients requiring urinary diversion for continence. Patients with an inadequate bladder template have a later age of first continence procedure and a relatively later age of continence, because of an inherently smaller bladder template at birth. The inadequate bladder template patients require a longer period of surveillance to access bladder growth and capacity in preparation of a continence procedure. Furthermore, as the majority of inadequate bladder template patients require a catheterizable channel for continence, the age of continence is also likely influenced by the patient's preparation as they transition from volitional voiding to catheterization.


Assuntos
Extrofia Vesical/cirurgia , Criança , Pré-Escolar , Feminino , Humanos , Recém-Nascido , Masculino , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Bexiga Urinária/fisiologia , Bexiga Urinária/cirurgia , Micção , Procedimentos Cirúrgicos Urológicos/métodos
14.
J Urol ; 200(6): 1354-1361, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-29906437

RESUMO

PURPOSE: We used magnetic resonance imaging to define the innate pelvic neurovascular course and prostatic anatomy in infants with classic bladder exstrophy before the pelvis was altered by surgery. MATERIALS AND METHODS: Pelvic magnetic resonance imaging was performed in male infants with classic bladder exstrophy and compared to a group of age matched controls. Data collected included prostatic dimensions as well as course of the prostatic artery, periprostatic vessels and pudendal neurovasculature. RESULTS: The prostate was larger in the transverse (p <0.001) and anteroposterior (p <0.001) dimensions in patients with classic bladder exstrophy compared to those with normal prostates but was smaller in the craniocaudal dimension (p <0.001). This finding resulted in a larger calculated prostate volume in patients with classic bladder exstrophy compared to controls (p = 0.015). The pelvic vasculature and prostatic artery followed a similar course in patients with classic bladder exstrophy and controls. Relative to each other, the lateral to medial course of the prostatic arteries in males with classic bladder exstrophy was less pronounced than in normal males. A similar externally rotated pattern was seen when both sides of the pudendal vasculature were compared in males with classic bladder exstrophy. CONCLUSIONS: The prostate in infants with classic bladder exstrophy has a consistent configuration and dimensions that differ from those in normal infants. When both sides are compared, the periprostatic vasculature and penile sensory neurovascular bundles are externally rotated in infants with classic bladder exstrophy. However, these components course along the same landmarks as in normal patients.


Assuntos
Extrofia Vesical/diagnóstico por imagem , Imagem por Ressonância Magnética/métodos , Pênis/diagnóstico por imagem , Próstata/diagnóstico por imagem , Pontos de Referência Anatômicos , Extrofia Vesical/cirurgia , Estudos de Casos e Controles , Pré-Escolar , Humanos , Lactente , Recém-Nascido , Masculino , Pelve/irrigação sanguínea , Pelve/diagnóstico por imagem , Pênis/irrigação sanguínea , Período Pré-Operatório , Estudos Prospectivos , Próstata/irrigação sanguínea
15.
J Urol ; 200(4): 882-889, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29723567

RESUMO

PURPOSE: Understanding the distinct female anatomy in classic bladder exstrophy is crucial for optimal reconstructive and functional outcomes. We present novel quantitative anatomical data in females with classic bladder exstrophy before primary closure. MATERIALS AND METHODS: 3-Dimensional reconstruction was performed in patients undergoing pelvic magnetic resonance imaging, and pelvic anatomy was characterized, including measurements of the vagina, cervix and erectile bodies. RESULTS: We examined magnetic resonance imaging of 5 females (mean age 5.5 months) with classic bladder exstrophy and 4 age matched controls (mean age 5.8 months). Mean distance between the anal verge and vaginal introitus was greater in patients with classic bladder exstrophy (2.43 cm) than in controls (1.62 cm). Mean total vaginal length in patients with classic bladder exstrophy was half that of controls (1.64 cm vs 3.39 cm). All 4 controls had posterior facing cervical ora, while 4 of 5 females with exstrophy had anterior facing cervical ora located in the anterior vaginal wall. Lateral deviation of the cervical ora was also seen in all 5 patients with classic bladder exstrophy but in only 1 control. Clitoral body length was comparable in both groups (26.2 mm and 28.0 mm). However, the anterior cavernosa-to-posterior (pelvic rami associated) cavernosa ratio was much greater in patients with classic bladder exstrophy (6.4) compared to controls (2.5). CONCLUSIONS: This study uncovers the uniquely novel finding that contrary to their male counterparts, females with classic bladder exstrophy have the majority of the clitoral body anterior to the pelvic attachment. This discovery has surgical and embryological implications.


Assuntos
Extrofia Vesical/diagnóstico por imagem , Extrofia Vesical/cirurgia , Genitália Feminina/diagnóstico por imagem , Imageamento Tridimensional , Imagem por Ressonância Magnética/métodos , Procedimentos Cirúrgicos Reconstrutivos/métodos , Estudos de Casos e Controles , Feminino , Genitália Feminina/anatomia & histologia , Humanos , Lactente , Amostragem , Sensibilidade e Especificidade
16.
Urology ; 119: 133-136, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29807047

RESUMO

OBJECTIVE: To explore the use of concomitant bladder neck reconstruction (BNR) and creation of a continent stoma (CS) in patients who are not quite eligible for BNR but still strongly desire volitional voiding. METHODS: The authors retrospectively reviewed an institutional database of patients with exstrophy-epispadias complex who underwent BNR-CS between 2000 and 2015. Indications for a BNR-CS, perioperative outcomes, and continence status were evaluated. Method of voiding and continence status were analyzed for patients with greater than 6 months of follow-up after the BNR-CS. RESULTS: A total of 24 patients with exstrophy-epispadias complex (15 male and 9 female) underwent BNR-CS at a median age of 8.9 years (range 5.4-17.4). This included 18 patients with classic bladder exstrophy, 5 with epispadias, and 1 with a cloacal exstrophy variant. There were 5 surgical complications (20.1%) following the BNR-CS, including 3 febrile urinary tract infections, 1 superficial wound infection, and 1 urethrocutaneous fistula. The median follow-up time from the time of BNR-CS was 1.1 years (range 0.1-14.1). Seventeen of 24 patients (71%) had a follow-up greater than 6 months and were evaluated for continence. Twelve patients (71%) were completely dry for intervals greater than 3 hours following BNR-CS. Five (29%) did not achieve continence with BNR-CS. Of those 5 patients, 3 (60%) underwent subsequent bladder neck transection. CONCLUSION: Combined BNR and CS is a suitable alternative to achieve urinary continence in patients who are not ideal candidates for BNR alone. This approach can offer a select group of patients the opportunity for volitional voiding.


Assuntos
Extrofia Vesical/cirurgia , Epispadia/cirurgia , Bexiga Urinária/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos , Estomas Cirúrgicos , Procedimentos Cirúrgicos Urológicos/métodos
17.
J Pediatr Urol ; 14(5): 426.e1-426.e6, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29627154

RESUMO

INTRODUCTION: Successful primary closure is one of the main factors for achieving continence in a classic bladder exstrophy (CBE) patient. Even with contemporary management, patients still have failed primary closures. We sought to understand the role of training, surgical technique, and their impacts on outcomes of CBE closure. MATERIALS AND METHODS: A retrospective cohort study from the largest single-institution database of primary and re-closure CBE patients in the world was performed. Failed closure was defined as developing bladder outlet obstruction, wound dehiscence, bladder prolapse, or any need for a re-closure operation. Patient demographics and surgical factors were abstracted and analyzed. Multivariable analysis was performed to test for associations with successful exstrophy closure. RESULTS: Data from 722 patients were analyzed. On bivariate analysis, successful closure was associated with gestational age at presentation, time of closure, location of closure, credential of surgeon performing the closure, closure type, concomitant osteotomy, and type of immobilization. Multivariable analysis, adjusting for patient comorbidity and location of closure, demonstrated increased odds of failure for closure by pediatric surgeon compared with pediatric urologist (OR 4.32, 95% CI 1.98-9.43; p = 0.0002), closure by unknown credentialed surgeon (OR 1.86, 95% CI 1.15-2.99; p = 0.011), Complete Primary Repair of Exstrophy (CPRE) closure compared with Modern Staged Repair of Exstrophy (OR 2.05, 95% CI 1.29-2.99; p = 0.0024), and unknown closure type (OR 4.81, 95% CI 2.94-7.86; p < 0.0001) (Table). DISCUSSION: Many factors associated with failure on bivariate analysis can be explained by these patients presenting to a center of excellence or the selection bias of this cohort stemming from a single center database that have been previously published. However, the finding on adjusted multivariable logistic regression analysis that closure by a pediatric surgeon is associated with higher odds of failure is novel. The additional finding that CPRE closure is associated with failure is most likely secondary to these patients being referred to our institution after having been closed with CPRE which falsely increases its impact on closure failure. Nevertheless, as a center with a large exstrophy volume, this study draws from a cohort that is larger than any other. CONCLUSION: Classic bladder exstrophy closure should be performed at a center with pediatric urologists to ensure the best chance of a successful primary closure.


Assuntos
Extrofia Vesical/cirurgia , Procedimentos Cirúrgicos Urológicos/educação , Procedimentos Cirúrgicos Urológicos/métodos , Competência Clínica , Estudos de Coortes , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Resultado do Tratamento
18.
Urology ; 117: 137-141, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29704585

RESUMO

OBJECTIVE: To evaluate human acellular dermis (HAD) as an adjunct during bladder neck transection (BNT) by comparing surgical outcomes with other types of tissue interposition. METHODS: A prospectively maintained institutional database of exstrophy-epispadias complex (EEC) patients was reviewed for those who underwent a BNT with at least 6 months follow-up. The primary outcome was the occurrence of BNT-related fistulas. RESULTS: In total, 147 EEC patients underwent a BNT with a mean follow-up time of 6.9 years (range 0.52-23.35 years). There were 124 (84.4%) classic exstrophy patients, 22 (15.0%) cloacal exstrophy patients, and 1 (0.7%) penopubic epispadias patient. A total of 12 (8.2%) BNTs resulted in fistulization, including 4 vesicoperineal fistulas, 7 vesicourethral fistulas, and 1 vesicovaginal fistula. There were 5 (22.7%) fistulas in the cloacal exstrophy cohort and 7 (5.6%) fistulas in the classic bladder exstrophy cohort (P = .019). Using either HAD or native tissue flaps resulted in a lower fistulization rate than using no interposed layers (5.8% vs 20.8%; P = .039). Of those with HAD, the use of a fibrin sealant did not decrease fistulization rates when compared to HAD alone (6.5% vs 8.8%, P = .695). There was no statistical difference in surgical complications between the use of HAD and native flaps (8.6% vs 5%, P = .716). CONCLUSION: Use of soft tissue flaps and HAD is associated with decreased fistulization rates after BNT. HAD is a simple option and an effective adjunct that does not require harvesting of tissues in patients where a native flap is not feasible.


Assuntos
Derme Acelular , Extrofia Vesical/cirurgia , Epispadia/cirurgia , Períneo , Doenças Uretrais/prevenção & controle , Fístula da Bexiga Urinária/prevenção & controle , Fístula Vesicovaginal/prevenção & controle , Adolescente , Adulto , Extrofia Vesical/complicações , Criança , Pré-Escolar , Epispadia/complicações , Feminino , Adesivo Tecidual de Fibrina/uso terapêutico , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/prevenção & controle , Estudos Retrospectivos , Retalhos Cirúrgicos , Adesivos Teciduais/uso terapêutico , Bexiga Urinária/cirurgia , Fístula da Bexiga Urinária/etiologia , Adulto Jovem
20.
J Pediatr Surg ; 53(10): 1937-1941, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29555156

RESUMO

INTRODUCTION: Cloacal exstrophy (CE) is a severe midline congenital abnormality that requires numerous surgical corrections to achieve an acceptable quality of life. Candidates for urinary continence undergo multiple procedures, most often continent bladder diversions, to become socially dry. Here, the authors investigate the number of genitourinary interventions that patients with CE undergo to attain urinary continence. MATERIALS AND METHODS: A retrospective review of a prospectively maintained database of 1311 exstrophy epispadias complex patients was performed. Patients with CE who have had at least one continence procedure were included. A continence procedure was defined as bladder neck reconstruction with or without augmentation, bladder neck transection with continent urinary diversion, augmentation cystoplasty, or use of injectable bulking agents. Continence was defined as a dry interval greater than 3 hours without leakage at night. RESULTS: In total, 140 CE and CE variant patients have been managed at the authors' institution. Of the 116 CE patients, 59 received at least one continence procedure, 14 were excluded for incontinent diversion or cystectomy, and the remaining 43 patients are awaiting a continence procedure. At the time of analysis, 42 (71%) patients who underwent a continence procedure were dry. The median number of total urologic procedures to reach urinary continence was 4 (range 2-10). This included 1 bladder closure (range 1-3), 2 urinary continence procedures (range 1-4), and 1 (range 0-4) "other" genitourinary procedures. The median time to urinary continence was 11.0 years (95% CI [9.2-14.2]). CONCLUSIONS: A majority of CE patients who undergo a diversion procedure can achieve urinary continence. However multiple continence procedures are likely necessary. Of patients who are candidates for a continence procedure, half will be continent by the age of 11. LEVEL OF EVIDENCE: Level IV, Case series with no comparison group.


Assuntos
Extrofia Vesical/cirurgia , Epispadia/cirurgia , Incontinência Urinária/cirurgia , Procedimentos Cirúrgicos Urológicos/estatística & dados numéricos , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Análise de Sobrevida
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