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1.
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
2.
Curr Urol Rep ; 20(9): 48, 2019 Jul 08.
Artigo em Inglês | MEDLINE | ID: mdl-31286274

RESUMO

PURPOSE OF REVIEW: The exstrophy-epispadias complex (EEC) represents a group of congenitally acquired malformations involving the musculoskeletal, gastrointestinal, and genitourinary systems. Classic bladder exstrophy (CBE) is the most common and best studied entity within the EEC. In this review, imaging features of CBE anatomy will be presented with surgical correlation. RECENT FINDINGS: Magnetic resonance imaging (MRI) has emerged as a useful modality for pre- and postnatal assessment of the abdominal wall, pelvic floor, and gastrointestinal and genitourinary systems of children with CBE. The authors' experience supports use of preoperative MRI, in conjunction with navigational software, as a method for identifying complex CBE anatomy. Imaging facilitates surgical approach and improves visualization of complex anatomy, potentially helping to avoid complications. Continued investigation of imaging guidance in CBE repair is needed as surgical techniques improve.


Assuntos
Extrofia Vesical/diagnóstico por imagem , Extrofia Vesical/cirurgia , Epispadia/diagnóstico por imagem , Epispadia/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Imagem por Ressonância Magnética , Masculino , Procedimentos Cirúrgicos Urológicos
3.
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 , Imagem 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
4.
J Pediatr Orthop B ; 28(3): 207-213, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30830014

RESUMO

Bladder exstrophy is a congenital and rare malformation of the lower abdominal wall with exposure of the bladder mucosa to the external environment, and it is related to pelvis abnormalities. Eighteen patients with bladder exstrophy were treated with bilateral oblique pelvic osteotomy in conjunction with urologic reconstruction after they were stabilized by cast. No failure of midline closure was observed (wound dehiscence or recurrence of bladder exstrophy). Follow-up showed no leg length discrepancy or problems in walking. Bilateral oblique pelvic osteotomy is a safe procedure to treat bladder exstrophy, and it results in good orthopedic and urological function.


Assuntos
Extrofia Vesical/diagnóstico por imagem , Extrofia Vesical/cirurgia , Osteotomia/métodos , Ossos Pélvicos/diagnóstico por imagem , Ossos Pélvicos/cirurgia , Cuidados Pós-Operatórios/métodos , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Osteotomia/tendências , Cuidados Pós-Operatórios/tendências , Resultado do Tratamento
5.
BMJ Case Rep ; 12(1)2019 Jan 14.
Artigo em Inglês | MEDLINE | ID: mdl-30642850

RESUMO

The exstrophy-epispadias complex represents a spectrum of genitourinary malformations ranging from simple glanular epispadias to an overwhelming multisystem defect, cloacal exstrophy. Neonatal total reconstruction of bladder exstrophy-epispadias complex is the treatment of choice. An adult patient presenting with untreated exstrophy is very rare. Malignant transformation, commonly adenocarcinoma, in such cases is a known complication due to mucosal metaplasia of urothelium. Management in such cases necessitates a radical surgical procedure that often results in a massive defect in the anterior abdominal wall. Providing a cover for such defects is a challenging task for the reconstructive surgeon. Local skin flaps and wide mobilisation of the rectus muscle are the usually employed techniques for closure of such defects. However, these may be inadequate in extremely large defects such as those encountered in our patients. We, hereby, describe our technique of closure of the abdominal wall defect using a pedicled anterolateral thigh flap.


Assuntos
Parede Abdominal/patologia , Extrofia Vesical/cirurgia , Cistectomia/efeitos adversos , Bexiga Urinária/cirurgia , Parede Abdominal/anormalidades , Parede Abdominal/cirurgia , Adenocarcinoma/tratamento farmacológico , Adenocarcinoma/cirurgia , Adolescente , Extrofia Vesical/complicações , Extrofia Vesical/diagnóstico por imagem , Extrofia Vesical/patologia , Diástase Óssea , Epispadia/diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Osso Púbico/anormalidades , Doenças Raras , Procedimentos Cirúrgicos Reconstrutivos/métodos , Retalhos Cirúrgicos/transplante , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Bexiga Urinária/anormalidades , Neoplasias da Bexiga Urinária/tratamento farmacológico , Neoplasias da Bexiga Urinária/patologia , Neoplasias da Bexiga Urinária/cirurgia
6.
J Pediatr Surg ; 54(3): 491-494, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30029844

RESUMO

PURPOSE: To investigate the factors affecting primary bladder closure in cloacal exstrophy (CE). A successful primary closure is important for optimizing reconstructive outcomes, and it is a critical first-step in the reconstruction of CE. The authors' hypothesize that a smaller diastasis and use of an osteotomy are independent predictors of a successful closure. METHODS: A prospectively maintained database of 1332 exstrophy-epispadias complex (EEC) patients was reviewed for CE patients closed between 1975 and 2015. Univariate and multivariable analyses were performed to identify significant factors associated with CE primary bladder closure. RESULTS: Of 143 CE patients identified, 99 patients met inclusion criteria. Median follow-up time was 8.82 [IQR 5.43-14.26] years. In the multivariable model, the odds of having a successful closure are about 4 times greater for the staged cloacal approach compared to the 1-stage approach (OR, 3.7; 95% CI 1.2-11.5; p-value = 0.023). Also, having an osteotomy increases the chance of a successful closure by almost six-fold (OR, 5.8; 95% CI 1.7-19.6; p-value = 0.004). CONCLUSIONS: Using the staged approach with a pelvic osteotomy is paramount to a successful primary closure in CE. The authors strongly recommend using the staged approach and osteotomy as these factors independently increase the chance for a successful primary bladder closure. STUDY TYPE: Therapeutic study. LEVEL OF EVIDENCE: Level III, Retrospective comparative study.


Assuntos
Extrofia Vesical/cirurgia , Cloaca/anormalidades , Osteotomia/métodos , Bexiga Urinária/cirurgia , Procedimentos Cirúrgicos Urológicos/métodos , Cloaca/cirurgia , Epispadia/complicações , Feminino , Humanos , Masculino , Análise Multivariada , Estudos Retrospectivos , Resultado do Tratamento
7.
Ann Chir Plast Esthet ; 64(1): 120-123, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29980317

RESUMO

Classical bladder exstrophy (CBE), affecting 1 birth out of 30,000, is characterized by an evaginated bladder plate through a defect in the lower abdominal wall, multiple abdominal wall anomalies including a pubic bone arch dehiscence. Numerous approaches from childhood to adulthood are thus required, depending on the severity of the deformity, including the associated genital anomalies. We report the case of a 19-year-old woman with CBE with a history of three-failed primary closure. We performed a secondary neck closure with a concomitant suspension of the bladder neck and reconstruction of the lower abdominal wall using a bilateral gracilis muscle flap transposition. The early postoperative course was uneventful. The patient was discharged at day ten postoperatively. The upper part of the genital sutures (labia minora) secondary healed in three weeks. Assessment at 2, 6 and 16 months postoperatively, respectively noticed a complete healing with successful sexual intercourses, perceived gracilis contraction by the patient, and finally, recent attempts to get pregnant. Neither urinary infection nor urinary leaks occurred. Bilateral crossed gracilis muscles transfer linking both rectus abdominis muscle in front of the reconstructed bladder neck might benefit to bladder exstrophy patients.


Assuntos
Extrofia Vesical/cirurgia , Músculo Grácil/transplante , Retalhos Cirúrgicos , Parede Abdominal/cirurgia , Feminino , Humanos , Adulto Jovem
8.
Ann R Coll Surg Engl ; 101(1): e32-e34, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30286654

RESUMO

Ureteric herniation into the inguinal canal is a rare condition in adults and even more so in the paediatric population. These hernia types can be categorised as paraperitoneal or extraperitoneal. The former are the only varieties encountered in the paediatric literature, where patients are generally infants, while the latter phenomenon is seen in 20% of ureteric herniations in adults. We present a case of an extraperitoneal ureteric herniation into the inguinal canal in a 12-year-old boy with previous surgery for cloacal exstrophy, analyse the existing literature on the condition and make the speculation that the extraperitoneal variety is an adult variant seen in this child because of weakness in the surrounding structures as a result of previous surgery.


Assuntos
Hérnia Inguinal/diagnóstico , Doenças Ureterais/diagnóstico , Extrofia Vesical/cirurgia , Criança , Hérnia Inguinal/etiologia , Hérnia Inguinal/cirurgia , Humanos , Masculino , Doenças Ureterais/etiologia , Doenças Ureterais/cirurgia
9.
J Pediatr Surg ; 54(2): 223-228, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30527758

RESUMO

This is based on the 2018 Storz Urology Lecture at the BAPS Conference and is a personal review of three reconstructive paediatric urological conditions: hypospadias, congenital adrenal hyperplasia, and bladder exstrophy from the perspective of changing expectations and outcomes. LEVEL OF EVIDENCE: V (Expert Opinion).


Assuntos
Hiperplasia Suprarrenal Congênita/cirurgia , Extrofia Vesical/cirurgia , Hipospadia/cirurgia , Procedimentos Cirúrgicos Reconstrutivos , Procedimentos Cirúrgicos Urológicos , Criança , Feminino , Humanos , Masculino
10.
Urology ; 125: 256-259, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30580006

RESUMO

OBJECTIVE: "To describe management options for pelvic fluid collections in adult patients with classic bladder exstrophy." METHODS: A single institution retrospective chart review was performed of patients who presented between 1998 and 2016 with a history of bladder exstrophy and pelvic fluid collections and 3 patients were identified. Patients had been followed for a mean of 9.0 years (1-23). RESULTS: All 3 patients required urinary diversions at various intervals following their exstrophy repair as newborns. All initially presented with symptomatic fluid collections located inferior to the bladder visualized by cross-sectional imaging. Mean age at presentation was 32.3 years (26-38 years). Two patients underwent drainage and sclerosing of cystic fluid collections with durable symptomatic relief for 1 patient. The other had recurrence of the fluid collections so he underwent marsupialization of the fluid collection which failed to sufficiently alleviate his symptoms. Ultimately, he along with the last patient, underwent open excision of the presumed hypoplastic prostate leading to resolution of pain symptoms, though the last patient did have some persistence of the fluid collection. All patients maintained their erectile function subsequent to these interventions. CONCLUSION: Adult patients with bladder exstrophy can present with painful cystic fluid collections potentially due to secretions from presumed hypoplastic prostate tissue. Sclerosing of the cyst can be successful in a subset of these patients, though some may require removal of the presumed prostatic tissue, which is curative and can be achieved with preservation of erectile function.


Assuntos
Extrofia Vesical/complicações , Extrofia Vesical/cirurgia , Líquidos Corporais , Cistos/cirurgia , Disfunção Erétil/prevenção & controle , Complicações Pós-Operatórias/prevenção & controle , Complicações Pós-Operatórias/cirurgia , Adulto , Humanos , Masculino , Pelve , Recidiva , Estudos Retrospectivos
11.
Paediatr Anaesth ; 29(2): 161-168, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30447167

RESUMO

BACKGROUND: The local anesthetic, levobupivacaine, is the safer enantiomer of racemic bupivacaine. Present protocols for levobupivacaine are based on studies and pharmacokinetic modeling with racemic bupivacaine. AIMS: The aim is to investigate total serum levobupivacaine concentrations after a caudalepidural loading dose followed by a maintenance infusion over 48 hours in infants aged 3-6 months. METHODS: The clinical trial was conducted in eight infants aged 3-6 months, undergoing bladder exstrophy repair. Pharmacokinetic modeling allowed optimization of clinical sampling to measure total levobupivacaine and α1 -acid glycoprotein and prediction of the effect of α1 -acid glycoprotein on levobupivacaine plasma protein binding. RESULTS: The observed median total levobupivacaine serum concentration was 0.30 mg/L (range: 0.20-0.70 mg/L) at 1 hour after the loading dose of 2 mg/kg. The median total levobupivacaine concentration after 47 hours of infusion, at 0.2 mg/kg/h, was 1.21 mg/L (0.07-1.85 mg/L). Concentrations of α1 -acid glycoprotein were found to rise throughout the study period. Pharmacokinetic modeling suggested that unbound levobupivacaine quickly reached steady state at a concentration of approximately 0.03 mg/L. CONCLUSION: The study allows the development of a pharmacokinetic model, combining levobupivacaine and α1 -acid glycoprotein data. Modeling indicates that unbound levobupivacaine quickly reaches steady state once the infusion is started. Simulations suggest that it may be possible to continue the infusion beyond 48 hours.


Assuntos
Anestesia Epidural/métodos , Anestésicos Locais/administração & dosagem , Levobupivacaína/administração & dosagem , Orosomucoide/metabolismo , Analgesia Epidural/métodos , Anestésicos Locais/sangue , Anestésicos Locais/farmacocinética , Extrofia Vesical/cirurgia , Humanos , Lactente , Levobupivacaína/sangue , Levobupivacaína/farmacocinética , Medição da Dor , Dor Pós-Operatória/tratamento farmacológico , Dor Pós-Operatória/metabolismo , Estudos Prospectivos
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.
Einstein (Sao Paulo) ; 16(4): eRC3887, 2018 Nov 08.
Artigo em Inglês, Português | MEDLINE | ID: mdl-30427488

RESUMO

We report a case of secondary urinary reconstruction of previously separated conjoined twins with exstrophic bladder and urinary incontinence. Patients were male and aged 13-year-old. Twin one had a history of failed enterocystoplasty that extruded and was visible like an exstrophic neobladder. He underwent a procedure to close bladder neck and reconfigure abdominal wall. After the procedure the patient developed a fistula that was treated, but it persisted and, for this reason, a catheterizable pouch was constructed and native bladder was discarded. Twin two required the immediately construction of catheterizable pouch using the Macedo's technique. Currently, both patients are continent at 4 hour intervals. The mean follow-up was 8 months. Modern continent urinary diversion techniques offer new perspectives and hope for such complex population.


Assuntos
Extrofia Vesical/cirurgia , Epispadia/cirurgia , Gêmeos Unidos/cirurgia , Incontinência Urinária/cirurgia , Adolescente , Humanos , Masculino , Ilustração Médica , Procedimentos Cirúrgicos Reconstrutivos/métodos , Reoperação/métodos , Falha de Tratamento
14.
J Wound Care ; 27(10): 686-691, 2018 10 02.
Artigo em Inglês | MEDLINE | ID: mdl-30332360

RESUMO

OBJECTIVE: To introduce a practical technique for the early detection and prompt management of a probable bladder dehiscence (BD) and glanular dehiscence (GD) in patients with bladder exstrophy epispadias complex (BEEC) and hypospadias. METHOD: In this prospective study, paediatric patients with BEEC (group 1) and with proximal hypospadias (group 2) underwent body temperature measurement using a non-contact infrared radiant digital temperature measurement device in four body regions, including the surgical wound, forehead, right hand, and right foot at eight hour intervals, postoperatively. This technique was performed to detect wound temperature rises before whole body temperature rise or visible local wound skin redness, cellulitis or any sign of inflammation or wound dehiscence (WD). RESULTS: A total of 24 paediatric patients were recruited. Temperature rise in the surgical wound area was discovered in two patients with BEEC. The temperature reached 39.2°C in the first case (12 days postoperative) and 39.4°C in the second case (16 days postoperative). Urinalysis, urine culture, and clean surgical wound sampling was performed and the presence of Gram-positive microorganisms was detected. Both patients were managed with intravenous imipenem and vancomycin. After changing the antibiotic regimen, wound temperature was gradually decreased to 37.2°C in the first patient by day 16, and to 36.9°C in the second patient by day 21, without rise in body temperature. Other patients in group 1 and all patients in group 2 had normal wound temperature fluctuations within the follow-up period. CONCLUSION: Postoperative periodical temperature measurement by a non-contact infrared radiant digital temperature measurement device is a safe and feasible technique that has the ability to detect deep wound infection, and may prevent the occurrence of WD before any visible sign of inflammation.


Assuntos
Extrofia Vesical/cirurgia , Temperatura Corporal , Hipospadia/cirurgia , Infecção da Ferida Cirúrgica/diagnóstico , Pré-Escolar , Diagnóstico Precoce , Humanos , Lactente , Masculino , Complicações Pós-Operatórias/diagnóstico , Valor Preditivo dos Testes , Estudos Prospectivos , Termômetros
15.
J Pediatr Urol ; 14(4): 322.e1-322.e6, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-30078549

RESUMO

INTRODUCTION: There have been few prior studies on patient-centered outcomes of adults with bladder exstrophy; those performed have been single institution reviews. In collaboration with the Association for the Bladder Exstrophy Communities (ABeC) - an international support network for patients and families living with bladder exstrophy - an anonymous social media survey was performed of patient-reported genital appearance and erectile function. OBJECTIVE: The study evaluated the Penile Perception Score (PPS) and the International Index of Erectile Function (IIEF-15) in adult men with bladder exstrophy. It was hypothesized that scores would be significantly lower than prior reported scores for men without the condition. STUDY DESIGN: After review by an anonymous patient advocate volunteer, the ABeC performed an anonymous online survey using social media. RESULTS: Adult men with bladder exstrophy reported PPS, and satisfaction with penile length and axis that were lower than prior published controls (n = 54, P < 0.05). Orgasmic function, sexual desire, and overall satisfaction were also lower (n = 46, P < 0.05). There was no difference in erectile function or intercourse satisfaction compared to controls. There was an association between PPS and erectile function, intercourse satisfaction, and overall satisfaction (P < 0.05) (Summary Fig.). Respondents identified urinary issues, genital appearance, and sexual function as the three main domains that warranted further research. CONCLUSION: This study found that PPS and IIEF-15 were low in adult men with bladder exstrophy, and worse perception of genital appearance was associated with worse sexual satisfaction. Social media is a novel tool for patient-centered outcomes research, and continued collaboration with patients and patient advocate groups is vital.


Assuntos
Extrofia Vesical/cirurgia , Medidas de Resultados Relatados pelo Paciente , Satisfação do Paciente , Pênis/anatomia & histologia , Mídias Sociais , Adolescente , Adulto , Idoso , Humanos , Masculino , Pessoa de Meia-Idade , Orgasmo , Ereção Peniana , Adulto Jovem
16.
Urologe A ; 57(10): 1208-1213, 2018 Oct.
Artigo em Alemão | MEDLINE | ID: mdl-30116832

RESUMO

BACKGROUND: Because sick and injured children from war and crisis areas can often only be helped to a limited extent in their countries of origin, those children with the most complex diseases often receive medical treatment in Europe. MATERIALS AND METHODS: To evaluate the postoperative outcome of reconstructive interventions in children from war and crisis areas, an evaluation of the clinic's internal database between 1997 and 2017 was carried out. The operative indication, the surgical procedure, any revisions or conversions and the overall mortality were analyzed. RESULTS: During the above-mentioned period a total of 44 (male: 32, female: 12) children aged 3-14 years from Afghanistan, Angola, Central Asia and the Caucasus were treated. Indications for the reconstructive procedures were bladder exstrophy (n = 23), neurogenic bladder dysfunction (n = 9), penile partial amputation (n = 1) and traumatic bladder dysfunction (n = 11). In 26 children, a conduit was created, 11 children received a sigma rectum pouch and 1 child an ileocecal pouch, while 5 other children received bladder augmentation and 1 child an oral mucosal urethroplasty. In total, 7 revisions or conversions due to ureteral implantation stenoses and recurrent acidosis had to be performed. Five children died in their home country due to acidosis, sepsis, end stage renal failure, accident and social exclusion. CONCLUSION: In the long term 39 of the 44 (88.6%) children live socially integrated in their home country and are regularly monitored by local aid organizations. When choosing the reconstructive intervention, the local conditions must be considered.


Assuntos
Extrofia Vesical/cirurgia , Epispadia/cirurgia , Bexiga Urinaria Neurogênica/cirurgia , Bexiga Urinária/lesões , Bexiga Urinária/cirurgia , Derivação Urinária/métodos , Procedimentos Cirúrgicos Urológicos/métodos , Adolescente , Criança , Pré-Escolar , Europa (Continente) , Feminino , Humanos , Masculino , Complicações Pós-Operatórias , Estudos Retrospectivos , Resultado do Tratamento , Uretra , Derivação Urinária/efeitos adversos , Incontinência Urinária/etiologia
17.
J Pediatr Urol ; 14(6): 558-564, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30126745

RESUMO

BACKGROUND: The radical soft-tissue mobilization (RSTM, or Kelly repair) is an anatomical reconstruction of bladder exstrophy generally performed as a second part of a two-step strategy, following successful neonatal bladder closure. OBJECTIVE: The objective of this study is to determine the feasibility of a combined procedure of delayed bladder closure and RSTM in one stage without pelvic osteotomy, in both primary and failed initial closure. DESIGN, SETTING, AND PARTICIPANTS: From 11/2015 to 01/2018, 27 bladder exstrophy patients underwent combined bladder closure with RSTM by the same surgical team at four cooperating tertiary referral centers for bladder exstrophy, including 20 primary repairs (delayed bladder closure, median age 3.0m [0.5-37m]) and seven secondary repairs after failed attempt at neonatal closure, median age 10m [8-33m]. INTERVENTION: RSTM included full mobilization of the bladder plate, urogenital diaphragm, and corpora cavernosa from the medial pelvic walls, followed by anatomical reconstruction with antireflux procedure, bladder closure, urethrocervicoplasty, muscle sphincter approximation, and penile/clitoral reconstruction. OUTCOME MEASUREMENTS: The main criteria were bladder dehiscence or prolapse. Secondary outcomes included bladder neck fistula or urethral fistula, urethral stenosis, and parietal hernia. Continence and voiding have not been addressed at this stage. RESULTS AND LIMITATIONS: All bladder exstrophy cases were successfully closed without osteotomy, with no case of bladder dehiscence after 12 m [3-30] follow-up. COMPLICATIONS: Urethral fistula or stenosis occurred in eight patients: 4/5 fistulae closed spontaneously in less than 3 months; four urethral stenoses were successfully treated with 1-3 sessions of endoscopic high-pressure balloon dilatation or meatoplasty; one patient with persistent bladder neck fistula is currently awaiting repair. Although the follow-up is short, it does allow examination of the main outcome criterion, namely bladder dehiscence, which is usually expected to happen very early after surgery. CONCLUSION: The Kelly RSTM can be safely combined with delayed bladder closure without osteotomy in both primary and redo cases in classic bladder exstrophy.


Assuntos
Extrofia Vesical/cirurgia , Bexiga Urinária/cirurgia , Pré-Escolar , Estudos de Viabilidade , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Procedimentos Cirúrgicos Urológicos/métodos
18.
Pan Afr Med J ; 29: 197, 2018.
Artigo em Francês | MEDLINE | ID: mdl-30061975

RESUMO

Bladder exstrophy is rare malformation pathology with an incidence of about 1 in 50,000 newborns. Not treated in time exposes to two main complications: kidney failure and bladder plaque cancer with a risk up to 200 times normal, which usually occurs around the fourth and fifth decade. In 95% it is adenocarcinoma and 5% squamous cell carcinoma. We present a rare case of adenocarcinoma developed on a bladder exstrophy in a 61-year-old patient who underwent an excision of the bladder plate carrying the whole tumor mass with a non-continent urinary diversion type bricker.


Assuntos
Adenocarcinoma/patologia , Extrofia Vesical/cirurgia , Neoplasias da Bexiga Urinária/patologia , Adenocarcinoma/diagnóstico , Adenocarcinoma/cirurgia , Extrofia Vesical/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias da Bexiga Urinária/diagnóstico , Neoplasias da Bexiga Urinária/cirurgia
19.
J Pediatr Urol ; 14(5): 431.e1-431.e6, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30031742

RESUMO

INTRODUCTION: In children who remain incontinent after reconstruction of bladder exstrophy-epispadias complex (BEEC), continent anal urinary diversion (CAD) is one option to achieve continence. Known problems after CAD are an increased stool frequency and ureterointestinal stenosis. We devised a new surgical technique of CAD that we named the "Cologne pouch procedure" (CPP) that renders the possibility of separate evacuation of urine and feces. Furthermore, we connect the bladder plate to the rectosigmoid pouch instead of performing a ureterosigmoidostomy to reduce the rate of ureterointestinal stenosis. In this study, we want to introduce the CCP and critically evaluate our results. STUDY DESIGN: In CPP a detubularized sigmoid-bladder pouch is created, which is naturally connected to the rectum. A retrospective study was performed including all patients with BEEC and CPP treated in our hospital between January 1, 2007, and December 31, 2016. Epidemiological and surgical key data, complications, and the need for alkaline supplementation were assessed. At follow-up examinations, we evaluated continence, ability of independent urine and feces evacuation, need for bicarbonate supplementation, status of the upper urinary tract, and complications such as urinary tract infections or urolithiasis. RESULTS: In total, 29 patients with BEEC and CPP were included. The mean age at surgery was 4.2 ± 3.3 years (range 0.1-12.7 years). Overall, 14 short-term complications occurred in nine patients. Postoperatively, all patients were continent for urine and feces during daytime and only one child occasionally lost small portions of urine at night. An independent evacuation of urine and feces was accomplished in 22 patients (81.5%). Continued bicarbonate supplementation was necessary in 15 patients (55.6%). During the follow-up period six patients (22.2%) had a single urinary tract infection and four patients (14.8%) calculi of the urinary tract. No urinary tract abnormalities-especially no vesicoureteral reflux (VUR) or stenosis-were detected during follow-up ultrasound examination. In two children, a preoperatively known hydronephrosis decreased after CPP. CONCLUSION: CPP is a novel technique that yields excellent results concerning continence. In contrast to other forms of rectosigmoid urinary diversion, functional separation of defecation and urination can be achieved in most patients.


Assuntos
Canal Anal/cirurgia , Extrofia Vesical/cirurgia , Epispadia/cirurgia , Bexiga Urinária/cirurgia , Coletores de Urina , Anastomose Cirúrgica/métodos , Criança , Pré-Escolar , Colo Sigmoide/transplante , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Derivação Urinária/métodos
20.
J Pediatr Urol ; 14(5): 430.e1-430.e6, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29914824

RESUMO

BACKGROUND: Primary bladder closure of classic bladder exstrophy (CBE) is a major operation that occasionally requires intraoperative or postoperative (within 72 h) blood transfusions. OBJECTIVE: This study reported perioperative transfusion rates, risk factors for transfusion, and outcomes from a high-volume exstrophy center in primary bladder closure of CBE patients. STUDY DESIGN: A prospectively maintained, institutional exstrophy-epispadias complex database of 1305 patients was reviewed for primary CBE closures performed at the authors' institution (Johns Hopkins Hospital) between 1993 and 2017. Patient and surgical factors were analyzed to determine transfusion rates, risk factors for transfusions, and outcomes. Patients were subdivided into two groups based upon the time of closure: neonatal and delayed closure. RESULTS: A total of 116 patients had a primary bladder closure during 1993-2017. Seventy-three patients were closed in the neonatal period, and 43 were delayed closures. In total, 64 (55%) patients received perioperative transfusions. No transfusion reactions were observed. Twenty-five transfusions were in the neonatal closure group, yielding a transfusion rate of 34%. In comparison, 39 patients were transfused in the delayed closure group, giving a transfusion rate of 91%. Pelvic osteotomy, delayed bladder closure, higher estimated blood loss (EBL), larger pubic diastasis, and longer operative time were all associated with blood transfusion. In multivariable logistic regression, pelvic osteotomy (OR 5.4; 95% CI 1.3-22.8; P < 0.001), higher EBL-to-weight ratio (OR 1.3; 95% CI 1.1-1.6; P = 0.029), and more recent years of primary closure (OR 1.1; 95% CI 1.0-1.2; P = 0.018) remained independent predictors of receiving a transfusion (Summary Table). No adverse transfusion reactions or complications were observed. DISCUSSION: This was the first study from a single high-volume exstrophy center to explore factors that contribute to perioperative blood transfusions. Pelvic osteotomy as a risk factor was unsurprising, as the osteotomy may bleed both during and immediately after closure. However, it is important to use osteotomy for successful closure, despite the increased transfusion risk. The risks accompanying contemporary transfusions are minimal and osteotomies are imperative for successful bladder closure. CONCLUSIONS: More than half of CBE patients undergoing primary closure at a single institution received perioperative blood transfusions. While there was an association between transfusions and osteotomy, delayed primary closure, larger diastasis, increased operative time, and increased length of stay, only the use of pelvic osteotomy, higher EBL-to-weight ratio, and recent year of closure independently increased the odds of receiving a transfusion on multivariate analysis.


Assuntos
Extrofia Vesical/cirurgia , Transfusão de Sangue/estatística & dados numéricos , Feminino , Previsões , Humanos , Recém-Nascido , Masculino , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
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