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1.
JBJS Case Connect ; 14(1)2024 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-38452165

RESUMO

CASE: We present a case of an 8-year-old boy with classical bladder exstrophy and a neglected right hip dislocation, exemplifying the risk of missed developmental dysplasia of the hip (DDH) in patients with exstrophy requiring careful orthopaedic oversight. CONCLUSIONS: When treating patients with bladder exstrophy, physicians and surgeons should be vigilant not to miss associated DDH. If this condition requires surgical treatment, preoperative planning with computed tomography scans is vital to uncovering the complexities arising from abnormal pelvic and acetabular anatomy and ensuring successful treatment outcomes.


Assuntos
Extrofia Vesical , Displasia do Desenvolvimento do Quadril , Luxação Congênita de Quadril , Masculino , Humanos , Criança , Extrofia Vesical/complicações , Extrofia Vesical/diagnóstico por imagem , Extrofia Vesical/cirurgia , Acetábulo , Luxação Congênita de Quadril/complicações , Luxação Congênita de Quadril/diagnóstico por imagem , Luxação Congênita de Quadril/cirurgia , Pelve
2.
BMC Pregnancy Childbirth ; 24(1): 195, 2024 Mar 12.
Artigo em Inglês | MEDLINE | ID: mdl-38475706

RESUMO

Bladder exstrophy (BE) is a congenital genito-urinary malformation where there is a defect in the abdominal wall resulting in a protruding open bladder with exposed mucosa (Resnik R.P. et al. Creasy and Resnik's maternal-fetal medicine: principles and practice. Elsevier, 2019). Several reconstructive procedures are required to correct the anomalies, resulting in an ileal conduit which is an alternate urinary reservoir reconstructed from the terminal ileum (Madersbacher S, et al. J Urol 169(3):985-90, 2003). We describe the care of a pregnant woman with BE and outline the principles of management of her pregnancy with a multidisciplinary team. Timely pre-operative planning is advised to minimise intraoperative complications in the event of a caesarean section. The woman went on to have an uncomplicated classical caesarean section at term by midline laparotomy with a good outcome for both mother and baby.


Assuntos
Extrofia Vesical , Procedimentos de Cirurgia Plástica , Complicações na Gravidez , Humanos , Gravidez , Feminino , Cesárea/efeitos adversos , Extrofia Vesical/complicações , Extrofia Vesical/cirurgia , Complicações na Gravidez/etiologia , Bexiga Urinária
3.
Pediatr Surg Int ; 40(1): 69, 2024 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-38441774

RESUMO

PURPOSE: An overexpression of nerve growth factor (NGF) in the urothelium is discussed to lead to neuronal hyperinnervation of the bladder detrusor. The aim was to assess the sensory and sympathetic innervation of the detrusor in unclosed exstrophic bladders patients with known overexpression of NGF in the urothelium. METHODS: Full-thickness bladder biopsies were prospectively obtained from 34 infants at delayed primary bladder closure between 01/2015 and 04/2020. The bladder biopsies were immunohistochemically stained with antibodies against S100, calcitonin gene-related peptide (anti-CGRP), Neurofilament 200 (anti-NF200), and tyrosine-hydroxylase (anti-TH). Specimens from 6 children with congenital vesicoureterorenal reflux (VUR) served as controls. RESULTS: There was no statistically significant difference in nerve fiber density in any of the immunohistochemical assessments (anti-S100 [p = 0.210], anti-CGRP [p = 0.897], anti-NF200 [p = 0.897]), and anti-TH [p = 0.956]) between patients with BE and patients with VUR. However, we observed a trend toward lower nerve fiber densities in exstrophic detrusor. CONCLUSION: Overall our results showed an unharmed innervation pattern in this cohort but a lower density of nerve fibers in the detrusor compared to controls. Further studies in patients after successful primary closure are needed to clarify the potential impact of the urothelial overexpression of NGF modulating the innervation pattern in exstrophic bladders.


Assuntos
Extrofia Vesical , Criança , Humanos , Lactente , Extrofia Vesical/cirurgia , Músculos , Fator de Crescimento Neural , Bexiga Urinária , Urotélio
4.
J Pediatr Orthop ; 44(5): e469-e473, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38477339

RESUMO

OBJECTIVE: Pelvic osteotomies relieve tension of the bladder and fascial closures during bladder exstrophy repair. Multiple techniques for postoperative immobilization of the pelvis and lower extremities have been described. The primary aim of this study was to assess differences in short and long-term changes in pubic rami diastasis when comparing Bryant traction to spica cast immobilization. Secondary aims included a comparison of length of stay, skin-related complications, and urologic outcomes. METHODS: We performed a single-institutional retrospective review of bladder exstrophy patients younger than 18 months of age who underwent posterior pelvic osteotomy and bladder exstrophy closure from April 2005 to April 2020. Short-term and long-term pubic rami diastasis were defined as postoperative measurements ≤6 months and ≥12 months, respectively. Secondary outcomes included length of stay, pressure ulcer, skin rash/abrasion, urethrocutaneous fistula, and bladder or fascial dehiscence rates. Multivariable logistic regression assessed for an association between immobilization type and degree of diastasis while controlling for age at the time of diastasis measurement and sex. RESULTS: Fifteen patients underwent Bryant traction and 36 patients underwent spica cast immobilization. In both the short-term and long-term, there was a greater reduction in pubic diastasis in the spica cast group ( P = 0.002 and P = 0.05, respectively). After adjustments, there were higher odds of having a greater reduction in pubic rami diastasis in both the short-term (odds ratio: 2.71, 95% CI: 1.52-4.86, P = 0.001) and long-term (odds ratio: 2.41, 95% CI: 1.00-5.80, P = 0.05). Length of stay was significantly higher in Bryant's traction group (26 vs 19 d, P < 0.001). Rates of pressure ulcers were higher in the Bryant traction group (26.7% vs 0%, P = 0.005). Rates of skin rash/abrasions, urethrocutaneous fistula, and bladder/fascial dehiscence did not differ. CONCLUSIONS: Spica cast immobilization is a safe and effective immobilization method. Compared with Bryant traction, spica cast immobilization was associated with a greater reduction in postoperative pubic diastasis both short and long-term, along with a shorter length of hospitalization and reduced rate of pressure ulcers. LEVEL OF EVIDENCE: Level III-therapeutic study.


Assuntos
Extrofia Vesical , Exantema , Fístula , Lesão por Pressão , Humanos , Lactente , Extrofia Vesical/cirurgia , Procedimentos Cirúrgicos Urológicos/métodos , Estudos Retrospectivos
5.
Ann Plast Surg ; 92(4): 437-441, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38527352

RESUMO

OBJECTIVE: In this study, we conducted a retrospective analysis of cases involving adult classic bladder exstrophy (CBE) accompanied by the absence of the abdominal wall. Specifically, we focused on the utilization of multilayer flaps for reconstructive purposes. In addition, we aimed to share our clinical treatment experience pertaining to similar challenges, thereby providing valuable insights to complement the surgical management of this rare disease. METHODS: We conducted a retrospective analysis of 12 adult patients diagnosed with CBE who underwent initial treatment between June 2013 and January 2020. All patients underwent multilayer reconstruction to address their abdominal wall defects. This involved utilizing shallow flaps derived from the superficial fascia of the abdomen and incorporating myofascial flaps composed of the anterior sheath of the rectus abdominis and aponeurosis of the external oblique muscle. The flap sizes ranged from 9 × 11 cm to 13 × 15 cm. RESULTS: Abdominal wall reconstruction in the 12 patients with CBE resulted in an absence of wound dehiscence recurrence, urinary obstruction, or urinary tract infection. All patients expressed satisfaction with the aesthetic outcome of their abdominal wall based on self-evaluation. They reported a successful resumption of normal life and work activities without experiencing any restrictions. The married patients expressed contentment with their sexual function. CONCLUSION: The utilization of a multilayered reconstruction technique involving multiple flaps in adults with congenital CBE allows for successful restoration of urinary function, as well as the attainment of sufficient abdominal wall strength to support daily life and work activities, while preserving sexual function. However, it is important to approach the evaluation of surgical outcomes with caution because of the rarity of this condition and the lack of objective assessment measures.


Assuntos
Parede Abdominal , Extrofia Vesical , Procedimentos de Cirurgia Plástica , Adulto , Humanos , Extrofia Vesical/cirurgia , Parede Abdominal/cirurgia , Estudos Retrospectivos , Retalhos Cirúrgicos/cirurgia
6.
Urology ; 185: 91-93, 2024 03.
Artigo em Inglês | MEDLINE | ID: mdl-38281666

RESUMO

Variations from classic bladder exstrophy (BE) are extremely uncommon, resulting in distinctive challenges in both diagnosis and management. The supravesical fissure variant of BE is exceptionally rare and has only been reported in male patients to date. Herein, we report the case and surgical management of a supravesical fissure variant of BE presentation in a newborn female patient and provide a literature review of this exstrophy variant.


Assuntos
Extrofia Vesical , Recém-Nascido , Humanos , Masculino , Feminino , Extrofia Vesical/cirurgia
7.
J Pediatr Surg ; 59(2): 225-229, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37968156

RESUMO

BACKGROUND: Cloacal exstrophy (CE) represents a rare sub-group of anorectal malformations. Traditionally managed with a permanent colostomy, colonic pull-through (PT) has emerged to allow cleanliness without a life-long stoma. We sought to understand outcomes of PT in a large multi-center CE population. METHODS: We performed a retrospective study involving eleven pediatric hospitals. We gathered data on demographics, outcomes, and anatomical factors including colon length. Continuous variables were analyzed with Wilcoxon rank-sum tests and categorial variables with Fisher's exact tests. RESULTS: There were 98 patients, of which the majority (n = 70, 71.4 %) never underwent PT. There were no differences in exstrophy type, demographics, or associated anomalies. Median age at PT was 1.3 years (IQR 0.3-3.7). Of the cohort that continue to use their PT, the majority (n = 16, 69.6 %) are not clean. In total, 7.1 % (n = 7) of the cohort is clean with a PT, and only one patient is continent. Clean patients have a longer colon length than those who are not clean or opt for re-do ostomy (64.0 cm [IQR 46.0-82.0] vs 26.5 cm [IQR 11.6-41.2], p = 0.005). CONCLUSION: Overall, we demonstrate that most children born with CE will keep their stoma. Only a small percentage who elect to undergo colonic PT are clean for stool. Greater colon length correlates with success. This suggests that multiple factors, including colon length, are important when considering PT in a child with CE. LEVEL OF EVIDENCE: III.


Assuntos
Malformações Anorretais , Extrofia Vesical , Pré-Escolar , Humanos , Lactente , Malformações Anorretais/cirurgia , Extrofia Vesical/cirurgia , Colo/cirurgia , Colo/anormalidades , Colostomia , Estudos Retrospectivos
8.
Urology ; 183: 212-214, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37972897

RESUMO

LUMBAR syndrome is rare with a multitude of features that requires a high index of suspicion for timely diagnosis and appropriate management. We present on a newborn female whose untreated segmental infantile hemangioma lead to poor healing of her bladder exstrophy closure. The objective of this report is to describe bladder exstrophy as a urogenital anomaly in patients with LUMBAR syndrome and the importance of balancing management of infantile hemangioma and time to surgery.


Assuntos
Extrofia Vesical , Hemangioma , Anormalidades Urogenitais , Humanos , Recém-Nascido , Feminino , Extrofia Vesical/complicações , Extrofia Vesical/diagnóstico , Extrofia Vesical/cirurgia , Estudos Retrospectivos , Procedimentos Cirúrgicos Urológicos
9.
Pediatr Nephrol ; 39(2): 371-382, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37410166

RESUMO

Individuals with bladder exstrophy-epispadias complex (EEC) need long-term integrated medical/surgical and psychosocial care. These individuals are at risk for medical and surgical complications and experience social and psychological obstacles related to their genitourinary anomaly. This care needs to be accessible, comprehensive, and coordinated. Multiple surgical interventions, reoccurring hospitalizations, urinary and fecal incontinence, extensive treatment regimens for continent diversions, genital differences, and sexual health implications affect the quality of life for the EEC patient. Interventions must include psychosocial support, medical literacy initiatives, behavioral health services, school and educational consultation, peer-to-peer opportunities, referrals to disease-specific camps, mitigation of adverse childhood events (ACEs), formal transition of care to adult providers, family and teen advisory opportunities, and clinical care coordination. The priority of long-term kidney health will necessitate strong collaboration among urology and nephrology teams. Given the rarity of these conditions, multi-center and global efforts are paramount in the trajectory of improving care for the EEC population. To achieve the highest standards of care and ensure that individuals with EEC can thrive in their environment, multidisciplinary and integrated medical/surgical and psychosocial services are imperative.


Assuntos
Extrofia Vesical , Epispadia , Adulto , Adolescente , Humanos , Criança , Extrofia Vesical/complicações , Extrofia Vesical/cirurgia , Extrofia Vesical/psicologia , Epispadia/complicações , Epispadia/cirurgia , Epispadia/psicologia , Qualidade de Vida , Bexiga Urinária
10.
Urology ; 183: 204-208, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37666328

RESUMO

There is a broad range of variant phenotypes that can occur within the bladder exstrophy and epispadias complex spectrum. Accurate prenatal detection helps prepare families and to coordinate subspecialty resources. Here, we present the case of a patient with prenatally diagnosed patient with covered cloacal exstrophy variant along with four additional cases illustrating the nonlinear spectrum from isolated epispadias to cloacal exstrophy. Given the rarity of these variants overall and of each subtype within the spectrum, there is a need for long-term multi-institutional outcomes data to improve detection, characterization, and prognostication for these patients.


Assuntos
Malformações Anorretais , Extrofia Vesical , Epispadia , Gravidez , Feminino , Humanos , Epispadia/diagnóstico , Epispadia/cirurgia , Extrofia Vesical/diagnóstico , Extrofia Vesical/cirurgia
11.
J Pediatr Surg ; 59(1): 26-30, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37838618

RESUMO

INTRODUCTION: Cloacal exstrophy (CE) is the most severe malformation of the exstrophy-epispadias complex. This study aims to discuss long-term sequela in a single major institution with a high volume of CE patients. MATERIALS AND METHODS: A prospectively maintained database of 1490 patients on the exstrophy epispadias spectrum (145 cloacal exstrophy) from 1974 to 2023. The patient database was reviewed for CE patients >10 years of age for genitourinary, gastrointestinal, orthopedic, and psychosocial outcomes. RESULTS: A total of 63 patients (43.4%) with ≥10 years of follow up were included for analysis. Thirty-nine (61.9%) patients were 18 years or older. Twenty-two (34.9%) patients were female and 39 (61.9%) male, 14 of whom were gender converted at birth. Two female patients conceived naturally and delivered via cesarean section. No male born CE patients had biological children. Catheterizable channels were common (45/63, 71.4%) and most (88.9%) were continent. Gastrointestinal diversion was managed mostly by colostomy (37/63, 58.7%). Three out of five (60.0%) patients who underwent PSARP were continent of stool. Twenty-two (34.9%) patients were wheelchair-bound. Psychosocial diagnoses included 52.4% (33/63) patients with anxiety/depression and 27.0% with chronic pain. Out of 56 patients evaluated by physical therapy, 75% were independent in ADL performance. Of patients older than 18, 79.5% (31/39) had attended college and 82.1% (32/39) were gainfully employed. CONCLUSION: Advances in critical care, nutrition, gastrointestinal, orthopedic, and urologic management have resulted in survival rates approaching 100% among patients with CE. While these children face long-term sequela spanning various organ systems, many lead independent and fully-functional lives. TYPE OF STUDY: Prognosis Study. LEVEL OF EVIDENCE: Level IV.


Assuntos
Extrofia Vesical , Epispadia , Urologia , Gravidez , Criança , Recém-Nascido , Humanos , Masculino , Feminino , Epispadia/cirurgia , Cesárea , Extrofia Vesical/cirurgia , Estudos Retrospectivos
12.
J Med Case Rep ; 17(1): 450, 2023 Oct 29.
Artigo em Inglês | MEDLINE | ID: mdl-37898815

RESUMO

BACKGROUND: With improved operative techniques pregnancy rates have been rising in patients with anomalies of the extrophy-epispadias-complex, including also female patients with bladder extrophy. Specific risks around pregnancy need to be addressed sufficiently beforehand. CASE PRESENTATION: An unplanned pregnancy was detected at 34 weeks in a 39-year old White female patient with former complex bladder extrophy. Decades after her operation she had not received any follow-up medical care and believed to be unable to conceive due to her anomaly. Thus no contraceptive matters were taken. The patient had lived in a stable relationship with regular sexual intercourse for many years. Until 34 weeks the pregnancy was uncomplicated, but then uterine prolapse and signs of beginning pre-eclampsia appeared, and a healthy girl was born with cesarean section. CONCLUSION: As patients with bladder extrophy and other anomalies from the extrophy-epispadias-complex reach adolescence/adulthood, they need continuous medical follow-up and transition of care to adult surgery and gynecology in order to address specific aspects of sexual health, reproduction, contraception, and also cancer screening. In the presented case lack of transition of care resulted in an unplanned and complicated pregnancy.


Assuntos
Extrofia Vesical , Epispadia , Complicações na Gravidez , Adulto , Adolescente , Humanos , Gravidez , Feminino , Lactente , Extrofia Vesical/cirurgia , Extrofia Vesical/complicações , Gravidez não Planejada , Cesárea/efeitos adversos , Epispadia/complicações , Epispadia/cirurgia , Complicações na Gravidez/etiologia
13.
Pediatr Med Chir ; 45(2)2023 Aug 29.
Artigo em Inglês | MEDLINE | ID: mdl-37667897

RESUMO

Primary closure techniques that have been updated and longterm follow-up for CBE (classic bladder exstrophy) may be out of reach for many patients living in resource-limited settings. Late referrals to medical care and primary closures that lack the necessary skills and facilities for comprehensive treatment are still common. Alternative and long-term surgical solutions may improve the lives of these unfortunate patients. During surgical outreach missions, patients with CBE, either non-operated or with a previous unsuccessful bladder closure, who were referred from vast under-resourced rural areas to three Eastern African hospitals, were studied. The following information is provided: mode of presentation, clinical history, diagnostic workout, management, and outcome. There were 25 cases (M/F ratio 17/8) ranging in age from two days to twenty years. Five of the seventeen patients who were not treated (35%) were under 120 days old and eligible for primary closure in a qualified tertiary center when one was available in the country. There were twelve late referred cases (ranging from 120 days to 20 years). Between the ages of ten months and twelve years, eight children arrived following a failed primary closure. In all of them, the bladder plate was too altered to allow closure. Following a preoperative diagnostic workout, a Mainz II continent internal diversion was proposed to fourteen patients with acceptable bowel control and postponed in the other three. Three cases were lost before treatment because parents refused the procedure. Twelve cases ranging in age from three to twenty years (mean seven years) were operated on. Eight people were followed for a total of 53.87 months (range: 36-120). Except for three people who complained of occasional night soiling, day and night continence were good. The average voiding frequency during the day was four and 1.3 at night. There was no evidence of a metabolic imbalance, urinary infection, or significant upper urinary tract dilatation. Two fatalities could not be linked to urinary diversion. Four patients were not followed up on. Due to the limited number of specialist surgical facilities, CBE late referral or failed closure is to be expected in a resource-limited context. In lieu of the primary closure, a continent internal diversion will be proposed and encouraged even at the level of a non-specialist hospital to improve the quality of life of these unfortunate patients. It is recommended that patients be warned about the procedure's potential long-term risks, which will necessitate a limited but regular follow-up.


Assuntos
Extrofia Vesical , Criança , Humanos , Lactente , Recém-Nascido , Extrofia Vesical/diagnóstico , Extrofia Vesical/cirurgia , Região de Recursos Limitados , Qualidade de Vida , Encaminhamento e Consulta , Bexiga Urinária/cirurgia
14.
Urology ; 181: 128-132, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37696307

RESUMO

OBJECTIVE: To review the outcomes of classic bladder exstrophy (CBE) closure without the use of osteotomy or lower extremity/pelvic immobilization. METHODS: A prospectively maintained institutional approved exstrophy-epispadias complex database of 1487 patients was reviewed for patients with CBE who had undergone closure without osteotomy nor immobilization. All patients were referred to the authors' institution for reconstruction later in life or for failed closure. RESULTS: Of a total of 1016 CBE patients, 56 closure events were identified that met inclusion with a total of 47 unique patients. Thirty-eight closures were completed prior to 1990 (67.9%). Forty-five closure events developed eventual failure (45/56, 80.4%) (Table 1). Thirteen closure events were secondary closures (13/56, 23.2%). The primary closure failure rate was 83.7% (36/43) while the secondary closure failure rate was 69.2% (9/13). Failures were attributed to one or more of dehiscence, bladder prolapse, and vesicocutaneous fistula (25/45, 55.6%) (23/45, 51.1%) (6/45, 13.3%), respectively. Thirty-seven patients developed social continence (37/47, 78.7%), while only 8 patients developed spontaneous voided continence (7/47,17.0%) (Table 2). The most common methods of voiding were continent catheterizable channels (25/47, 53.2%) of which all were socially continent. CONCLUSION: These results illustrate the critical role osteotomy and postoperative immobilization can play in both primary and secondary exstrophy closure. While this is a historical case series, the authors believe that these results remain relevant to contemporary exstrophy surgeons.


Assuntos
Extrofia Vesical , Anormalidades do Sistema Digestório , Humanos , Extrofia Vesical/cirurgia , Futilidade Médica , Procedimentos Cirúrgicos Urológicos/efeitos adversos , Exercício Físico , Osteotomia/efeitos adversos
15.
J Pediatr Surg ; 58(12): 2308-2312, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37777362

RESUMO

PURPOSE: Staged pelvic osteotomy has been shown in the past to be an effective tool in the closure of the extreme pubic diastasis of cloacal exstrophy. The authors sought to compare orthopedic complications between non-staged pelvic osteotomies and staged pelvic osteotomies in cloacal exstrophy. METHODS: A prospectively maintained exstrophy-epispadias complex database of 1510 patients was reviewed for cloacal exstrophy bladder closure events performed with osteotomy at the authors' institution. Bladder closure failure was defined as any fascial dehiscence, bladder prolapse, or vesicocutaneous fistula within one year of closure. There was a total of 172 cloacal exstrophy and cloacal exstrophy variant patients within the database and only closures at the authors' institution were included. RESULTS: 64 closure events fitting the inclusion criteria were identified in 61 unique patients. Staged osteotomy was performed in 42 closure events and non-staged in 22 closures. Complications occurred in 46/64 closure events, with 16 grade III/IV complications. There were no associations between staged osteotomy and overall complication or grade III/IV complications (p = 0.6344 and p = 0.1286, respectively). Of the 46 total complications, 12 were orthopedic complications with 6 complications being grade III/IV. Staged osteotomy closure events experienced 10/42 orthopedic complications while non-staged osteotomy closures experienced 2/22 orthopedic complications, however this did not reach significance (p = 0.1519). Of the 64 closure events, 57 resulted in successful closure with 6 failures and one closure with planned cystectomy. CONCLUSION: This study confirms, in a larger series, superior outcomes when using staged pelvic osteotomy in cloacal exstrophy bladder closure. Staged osteotomy was shown to be a safe alternative to non-staged osteotomy that can decrease the risk of closure failure in this group. Staged pelvic osteotomy should be considered in all patients undergoing cloacal exstrophy bladder closure. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III.


Assuntos
Extrofia Vesical , Epispadia , Humanos , Extrofia Vesical/cirurgia , Epispadia/cirurgia , Procedimentos Cirúrgicos Urológicos/métodos , Osteotomia/métodos , Cistectomia , Estudos Retrospectivos , Resultado do Tratamento
17.
J Pediatr Urol ; 19(6): 696.e1-696.e6, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37607850

RESUMO

INTRODUCTION: Continence after bladder exstrophy (BE) repair remains a major debatable challenge to pediatric urologists, together with the lack of standard definitions and long-term results in large series. OBJECTIVE: We assessed the long-term urinary continence in 142 toilet-trained cases after one (1-) stage of complete primary repair of bladder exstrophy (CPRE) and consequent procedures to achieve this goal in a single tertiary referral center. STUDY DESIGN: The current retrospective study included 123 boys and 19 girls with BE that were repaired by (1-) stage CPRE. The Mean age at (BE) repair was 9.5 ± 2.6 weeks. Complete penile disassembly (CPD) was used for epispadias repair in 42 (34.1%) and modified Cantwell-Ransley repair (MCR) was used in 81 (65.9%) boys. Bilateral anterior transverse innominate osteotomies (ATIO) were applied in all. Urinary continence was expressed in terms of the dry interval (DI). Continence procedures were afforded if CPRE failed to achieve DI ≥ 3 h (hrs.), those were in the form of endoscopic bladder neck injection (BNI), bladder neck reconstruction (BNR), and bladder neck closure (BNC) with catheterizable stoma. RESULTS: The mean age at follow up was 12.1 ± 5.2 years. DI ≥ 3 h was gained in 23 (16.2%) after CPRE alone, while complementary post-CPRE continence procedures were required to reach this goal in the remaining patients. Deflux injection was reported in 10 (7%), CIC in 8 (5.6%), BNR in 32 (22.5), and BNC with catheterizable stoma alone in 37 (26.1%), or with Charleston pouch in 32 (22.5%). DISCUSSION: We think that ≥3 h DI with voiding represents an appropriate definition of continence after BE repair. According to the results in the current series, we think that successful anatomical closure of BE is achievable, but the functional outcome in terms of continence and its evaluation is tricky. Results of continence were reported to change with age of the child, and it is difficult to evaluate both before toilet training age and long-term follow up. CONCLUSIONS: Long-term follow up of CPRE with bilateral ATIO alone or with BNI results in ≥3 h DI in a few cases; BNR after CPRE can provide a good chance for continence; otherwise, BNC with catheterizable stoma is a valid option.


Assuntos
Extrofia Vesical , Bexiga Urinária , Masculino , Feminino , Humanos , Criança , Lactente , Adolescente , Bexiga Urinária/cirurgia , Extrofia Vesical/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Procedimentos Cirúrgicos Urológicos/métodos
18.
Afr J Paediatr Surg ; 20(3): 233-237, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37470562

RESUMO

Classical bladder exstrophy is a congenital anomaly whose management and outcome has advanced over years. Management and outcome are better when management starts at the newborn period. This was the management of a neglected bladder exstrophy in a male presenting at 16 years of age. We report our challenges, management and outcome to highlight the rarity of this presentation, and the adaptation to the usual protocol of care. The patient presented at 16 years of age with classic bladder exstrophy. The bladder plate was contracted and had cystitis. The patient had a modification of complete primary repair of exstrophy (CPRE) with bilateral pelvic osteotomy stabilised with a 7-hole plate and 4 screws, then bladder neck reconstruction + bladder augmentation + cross-trigonal neocystoureterostomy in a 12-h procedure. He had surgical site infection, superficial wound breakdown and vesicocutaneous fistula that all healed with dressing and prolonged suprapubic cystostomy drainage. He achieved some degree of urinary continence and ability to void, though he still has stress incontinence and frequency at 6 months of follow-up. He has a micturition interval of 60-120 min, and is expected to improve. Presentation and repair of classic bladder exstrophy in the adolescent is very rare in the literature and therefore no known standard of care. This report adds to the body of knowledge. Again, this experience lends credence to the proponents of CPRE in reducing the number of procedures required to treat exstrophy.


Assuntos
Extrofia Vesical , Epispadia , Procedimentos de Cirurgia Plástica , Incontinência Urinária , Recém-Nascido , Humanos , Masculino , Adolescente , Extrofia Vesical/cirurgia , Extrofia Vesical/complicações , Incontinência Urinária/etiologia , Incontinência Urinária/cirurgia , Procedimentos Cirúrgicos Urológicos/métodos , Micção , Resultado do Tratamento , Epispadia/complicações , Epispadia/cirurgia
19.
Pediatr Surg Int ; 39(1): 232, 2023 Jul 12.
Artigo em Inglês | MEDLINE | ID: mdl-37436662

RESUMO

PURPOSE: Cloacal exstrophy (CE) patients may need bladder reconstruction after initially undergoing surgery to obtain continence and improve their quality of life. This study attempts to clarify the clinical features of CE patients who underwent bladder augmentation (BA) and their urinary functional outcomes based on a nationwide survey in Japan. METHODS: A questionnaire survey was conducted, and 150 CE patients were enrolled. Their clinical characteristics and urinary outcomes were reviewed. RESULTS: BA was performed in 52 patients (34.7%). Most cases underwent early bladder closure at initial surgery in neonate period. The age at the BA was performed 6.4 [6-9.0] years. Among them, the most used organ for BA was ileum (n = 30, 57.7%). Regarding the outcomes, the age when the renal function was evaluated was 14.0 [10.0-20.5] years and the serum creatinine level was 0.44 [0.36-0.60] (mg/dl). Clean intermittent catheterization was required in 37 (71.2%) patients. On the other hand, no dialysis or kidney transplantation was necessary in any of these patients. CONCLUSION: The renal function and conditions of patients who underwent BA were relatively well preserved. Individualized management with a stepwise surgical approach for CE patients should thus be considered in the future.


Assuntos
Malformações Anorretais , Extrofia Vesical , Recém-Nascido , Humanos , Criança , Adolescente , Adulto Jovem , Adulto , Bexiga Urinária/cirurgia , Japão , Qualidade de Vida , Extrofia Vesical/cirurgia , Inquéritos e Questionários , Estudos Retrospectivos
20.
Urology ; 180: 235-241, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37473922

RESUMO

OBJECTIVE: To determine intrapelvic angles and distances in pure epispadias patients and compare them to normal values. METHODS: Using three-dimensional computed tomography, 17 intrapelvic dimensions (8 angles and 9 distances) were measured in 26 patients with pure epispadias (21 boys and 5 girls). These values were compared to normal values obtained from 6 patients (5 boys and 1 girl) who underwent either pelvic or abdominopelvic computed tomography for purposes unrelated to their bony pelvis. RESULTS: Significant differences were observed in five angles (sacroiliac joint angle, S1 tilt angle, sacral curvature, superior-inferior rotation of the pelvis, and pubococcygeal angle; P-value=.016, .044, .011, .020, and .001, respectively); these show less sacral rotation toward the axial plane, more sacral curvature, inferior rotation of the pelvis, and sacroiliac joints' rotation toward the coronal plane in epispadias compared to controls. Also, two distances (pubic diastasis and anterior segment length of the pelvis; P-value=.002 and .012, respectively) had significant differences, showing wider pubic diastasis and shorter anterior segment in epispadias. However, the differences between other intrapelvic angles and distances were not statistically significant between the two groups. CONCLUSION: In addition to the explanations hypothesized for the embryology of the exstrophy-epispadias complex, there can be other etiologies for both epispadias and bladder exstrophy to explain the differences between bony anatomies of the pelvis in these patients.


Assuntos
Extrofia Vesical , Epispadia , Ossos Pélvicos , Masculino , Feminino , Humanos , Epispadia/diagnóstico por imagem , Epispadia/cirurgia , Ossos Pélvicos/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Extrofia Vesical/diagnóstico por imagem , Extrofia Vesical/cirurgia , Diafragma da Pelve
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