RESUMO
A primigravida in the extremist-affected region of a third-world nation gave birth to a newborn who was remotely consulted through video rounds from the capital of the state. Unfortunately, these abnormalities are often overlooked and left untreated. The baby had multiple limb defects, gastroschisis, exstrophy of the bladder and spina bifida. Tragically, the newborn did not survive due to the lack of clinical and surgical expertise in the area. It is crucial to emphasise the importance of establishing e-clinics for expectant mothers in underserved areas, providing them with access to high-quality anomaly scans.
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Extrofia Vesical , Gastrosquise , Disrafismo Espinal , Humanos , Gastrosquise/cirurgia , Gastrosquise/diagnóstico , Extrofia Vesical/cirurgia , Extrofia Vesical/complicações , Disrafismo Espinal/cirurgia , Disrafismo Espinal/complicações , Disrafismo Espinal/diagnóstico por imagem , Recém-Nascido , Feminino , Anormalidades Múltiplas/cirurgia , Gravidez , Deformidades Congênitas dos Membros/cirurgia , AdultoRESUMO
PURPOSE: To answer the question of whether it is possible to achieve complete corporal covering of the urethral closure using incomplete penile disassembly in classic bladder exstrophy. We hypothesize that mobilization of the corpora under Buck's fascia, their dorsal translocation through the incisions in Buck's fascia and suturing corporal convex sides above the urethra would allow extend corporal covering of the urethra, reducing the risk of urethra-cutaneous fistula formation. MATERIALS AND METHODS: A prospective follow-up on all boys who underwent the modified Cantwell-Ransley primary penile reconstruction was conducted. Inclusion criteria comprised bladder exstrophy closure in our institution, ensuring a postoperative follow-up period of no less than 24 months. The key innovation of the technique lies in a deep dissection of the dependent corpora under Buck's fascia, followed by their dorsal relocation through extended dorsal incisions in Buck's fascia, and limited external corporal rotation 90 degrees only at the base of the penis. RESULTS: Between November 2019 and March 2022, 18 boys aged 11 to 35 months met the inclusion criteria and underwent the modified penile reconstruction. Surgical procedures and postoperative period did not include any major complications. Total corporal covering of the urethral sutures was achieved in 15 of 18 patients. No urethra-cutaneous fistulas were observed within 2 years of follow-up. All individuals demonstrated spontaneous erections, and the absence of dorsal curvature was documented. CONCLUSION: The modified technique of incomplete penile disassembly applied in a homogenous group of patients with classic bladder exstrophy allows penile shaft elongation, improved aesthetic outcomes, preserved erections, and eliminates dorsal curvature. The technique demonstrated feasibility and reliability while maintaining positive effects on tissue circulation. The absence of urethra-cutaneous fistulae is attributed to the complete corporal covering of the urethral sutures and supports the initial hypothesis.
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Extrofia Vesical , Pênis , Procedimentos de Cirurgia Plástica , Uretra , Procedimentos Cirúrgicos Urológicos Masculinos , Humanos , Masculino , Extrofia Vesical/cirurgia , Estudos Prospectivos , Lactente , Procedimentos Cirúrgicos Urológicos Masculinos/métodos , Pré-Escolar , Uretra/cirurgia , Pênis/cirurgia , Resultado do Tratamento , Procedimentos de Cirurgia Plástica/métodos , Seguimentos , Reprodutibilidade dos Testes , Técnicas de SuturaRESUMO
The bladder exstrophy-epispadias complex includes some of the most challenging conditions treated by pediatric urologists. They are associated with the need for multiple intricate reconstructive procedures, aimed at restoring the anatomy and function of the bladder, urethra and external genitalia. These patients often endure multiple redo reconstructive procedures to improve urinary function, sexual function and cosmesis throughout the first two decades of life. In this article, we present the 30-year experience of a single surgeon performing redo surgery for males born with epispadias and bladder exstrophy. Through detailed documentation of 6 clinical cases, we highlight technical aspects that may contribute to a successful surgical reconstruction in these patients. The article is focused specifically on patients undergoing redo epispadias repair with or without concomitant continence procedures. We make the case for complete penile disassembly with external rotation of the corpora to correct recurrent dorsal curvature; this approach also allows the surgeon to have access to the proximal urethra and bladder neck after opening the intersymphiseal scar/band. This is useful when additional procedures on the bladder, such as bladder neck tailoring, are necessary. We also highlight the importance of avoiding reverse Byars' flaps when performing skin closure, due to the resulting midline scar. Besides being associated with a poor cosmetic outcome, it can also contribute to recurrent dorsal curvature. The authors advocate for rotational skin flaps to cover the penile shaft. Correction of dorsal curvature and improved cosmesis obtained with complete penile disassembly sometimes comes at the expense of the urethra being left as a hypospadias (figure). This will require further surgeries (usually a 2-stage buccal mucosa graft), much like the treatment of proximal hypospadias. Redo epispadias surgery in males remains a challenge. The systematic approach offered by the case scenarios may help guide surgeons dealing with this difficult condition. Patient with complications after repair of classic bladder exstrophy. A) Stone retrieved from posterior urethra after complete penile disassembly. B) After opening the inter-symphiseal scar, the bladder has been opened and the bladder neck tailored. C) Complete penile disassembly has been completed with corporal bodies and urethra individualized. D,E,F) Final appearance of the repair; abdominal wall was closed with anterior rectus sheath flaps, penile skin was closed with rotational flaps and urethra ended up as a hypospadias.
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Extrofia Vesical , Epispadia , Reoperação , Procedimentos Cirúrgicos Urológicos Masculinos , Humanos , Masculino , Epispadia/cirurgia , Extrofia Vesical/cirurgia , Procedimentos Cirúrgicos Urológicos Masculinos/métodos , Criança , Procedimentos de Cirurgia Plástica/métodos , Estética , Adolescente , Complicações Pós-Operatórias/cirurgia , Pré-EscolarAssuntos
Extrofia Vesical , Humanos , Extrofia Vesical/cirurgia , Resultado do Tratamento , Fatores de Tempo , Masculino , Feminino , Seguimentos , CriançaRESUMO
BACKGROUND: In literature studies exploring long-term psychosexual development and intimacy of adults living with the complex genitourinary conditions associated with classic bladder exstrophy (BE) are scarce, with small sample sizes and lacking in methodology. OBJECTIVE: This study aims to examine areas of potential psychosexual distress in adults born with classic BE to develop targeted clinical interventions. STUDY DESIGN: The validated Sexrelation Evaluation Schedule Assessment Monitoring (SESAMO) questionnaire was administered to all BE patients aged ≥18 years operated on in our tertiary referral center during infancy. Z-scores were calculated for each area of interest, considering i) gender (female vs male); ii) committed partnership status (singles vs couples); iii) the voiding technique adopted to empty the bladder. RESULTS: A total of 33 (F:M 12:21; singles: couples 11:22) adults with BE were enrolled in the study at a median age of 39 (32-47) years. Overall, BE adults performed the worst regarding psychosexual identity (z-score:-1.282), pleasure (z-score:-0.915) and desire (z-score:-0.583); singles regarding relational attitude (z-score:-1.751) and imaginative eroticism (z-score:-0.806); couples regarding extramarital sexuality (z-score:-1.175) and sexual communication (z-score:-0.255). When it came to gender, females significantly performed worse than males regarding psychosexual identity (-1.645 vs -1.282; p-value:<0.0001) and areas of pleasure (-1.126 vs -0.359; p-value:<0.001). Single females performed worse than males regarding actual masturbation (-0.763 vs 0.583; p-value:<0.05) and better regarding relational attitude (-1.226 vs -1.751; p-value:<0.05). Females in stable relationships performed worse than males regarding actual masturbation (-1.645 vs 0.306; p-value:<0.05) and better regarding sexual intercourse (1.866 vs -0.565; p-value:<0.01). The voiding techniques used to empty the bladder did not show any influence on these results. CONCLUSION: Adults with BE have a greater likelihood of experiencing a wide range of psychosexual difficulties. Identifying the specific areas of psychological distress can help them cope with their medical experience and actual clinical condition and clinicians plan adequate psychological interventions.
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Extrofia Vesical , Humanos , Extrofia Vesical/psicologia , Extrofia Vesical/cirurgia , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Desenvolvimento Psicossexual , Inquéritos e Questionários , Fatores de Tempo , Adaptação Psicológica , Comportamento Sexual/psicologiaRESUMO
AIM: The small bladder in failed exstrophy needs to be augmented and also accommodate bladder neck surgery, ureteric re-implantations and a catheterizable channel. We present a novel method of ileocecal bladder augmentation providing a non-implanted, un-detached appendicular channel, rendered non refluxing by an ileal "fundoplication". The colonic segment could receive ureteric reimplantation when bladder plate is unsuitable. MATERIALS AND METHODS: Operative technique: Bilateral cephalo-trigonal reimplantation and bladder neck surgery is done. The ileocecal segment is isolated and arranged as a ring (doughnut) around the un-detached appendix which is made to pass behind the terminal ileum, through the ileal mesentery and then in front of the ileum as it exits as a catheterizable channel. The bowel segment is opened on the antimesenteric aspect except for the retro appendiceal ileal segment. The opened ileo-caecum is reconfigured as a de-tubularised augmentation pouch while the retro appendiceal ileal segment is wrapped around the appendix like a fundoplication. The colon segment may receive the ureteric reimplantation if required. CASE MATERIAL: 100 children (mean age 6.3 years) with failed exstrophy underwent doughnut augmentation (3 neobladders) in 2006-2021. Ninety eight were incontinent, with 30 open/dehisced bladder plate, 48 units were hydronephrotic (HUN), 105 ureters were refluxing (VUR) and eGFR was subnormal in 20 children. 79 children underwent YDL bladder neck plasty and 23 underwent bladder neck closure (primarily in 18). Three infants with HUN did not undergo bladder neck surgery. 126 ureters were reimplanted into the bladder, 44 into the colon segment while 30 were not reimplanted. RESULTS: 95 children are continent on CIC, which was gradually introduced starting with once or twice daily change of indwelling Mitrofanoff catheter leading to regular 3-4 hourly CIC as the child became confident. The appendicular channel was easily catheterizable without any leak in 95, had minor issues in 4 while one necrosed and replaced by a gastric tube. HUN recovered (25/48) or stabilized with a significant reduction in the grade of hydronephrosis (p = 0.001). VUR resolved in 90% of bladder reimplants, 85% of colonic reimplants and 70% of non-reimplanted ureters. In the 20 children with compromised renal function eGFR improved (71-106 ml/m2/min) significantly (p = 0.036) at follow up (mean 5 years). Urodynamics study (N = 33) showed a mean capacity of 95% of expected bladder capacity (EBC) with end filling pressure less than 25 cm of water (Mean end filling cystometric pressure = 15 cm of water). Complications included adhesive intestinal obstruction (N = 16) and bladder calculi (N = 12). CONCLUSION: The doughnut ileocecal augmentation provides a easily catheterizable, non-refluxing, appendicular channel, not requiring isolation of the appendix nor its reimplantation into the bladder. The colonic segment can provide a bed for ureteric reimplantation. Dryness with a good capacity bladder and preserved upper tract is achieved.
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Extrofia Vesical , Epispadia , Íleo , Humanos , Extrofia Vesical/cirurgia , Masculino , Feminino , Íleo/cirurgia , Íleo/transplante , Pré-Escolar , Epispadia/cirurgia , Bexiga Urinária/cirurgia , Criança , Lactente , Ceco/cirurgia , Procedimentos Cirúrgicos Urológicos/métodos , Estudos Retrospectivos , Procedimentos de Cirurgia Plástica/métodos , Resultado do TratamentoRESUMO
INTRODUCTION: Achieving urinary continence is a key goal in children born with the bladder exstrophy-epispadias complex (BEEC). Unfortunately, this goal is only moderately achieved despite sometimes extensive surgical treatment. Undergoing repeated hospitalization and operations may consequently have a negative impact on quality of life. We therefore believe that other, conservative treatment options should be explored in an earlier stage of incontinence treatment in BEEC patients. As part of this, an intensive urotherapy program based on was offered to patients with persistent incontinence after reconstructive surgery for BEEC. OBJECTIVE: The aim of this study is to evaluate the benefits of intensive urotherapy on incontinence after reconstructive surgery in children with BEEC. STUDY DESIGN: A retrospective chart study was performed including all children who were enrolled in an intensive urotherapy program because of persistent incontinence after reconstructive surgery for BEEC. Urotherapy consisted of a ten-day inpatient training program based on cognitive behavioral therapy, with intensive follow-up by experienced urotherapists. Main outcome measurement was continence, expressed as the percentage of children that achieved complete continence (good result; 100% dry) or 50-99% decrease of wet days a week (improved result) after treatment. RESULTS: Data of 33 patients with a mean age of 10.6 years were analyzed. In 61% of cases (20/33) an improved or good result was reported on incontinence after urotherapy. Children with classic bladder exstrophy more often achieved a good or improved result (13/16; 81%), compared to children with epispadias (6/16; 38%). The only patient with a cloacal exstrophy completed treatment with an improved result. From the group of patients with persistent incontinence, 75% (12/16) reported that the complaints were socially acceptable at the end of follow-up. DISCUSSION: By following our intensive urotherapy program the majority of patients achieved complete continence or improved incontinence. In addition, our results show that the inpatient training program has a positive impact on acceptance in cases of persistent incontinence. The urotherapists offer individualized care and clear guidance, which we deem essential elements of successful treatment. Considering that repeated surgery may impede progress and offers no guarantee of continence, we recommend giving preference to conservative treatment options. CONCLUSION: Our results show that 37% (12/33) of patients with BEEC who were enrolled in our intensive urotherapy program because of persistent incontinence after reconstructive surgery, achieved complete continence after urotherapy and 63% (21/33) still experienced some degree of incontinence. 75% of patients who did not achieve complete continence, described the remaining incontinence as socially acceptable. These findings strongly support counselling patients with BEEC to consider conservative treatment before opting for further surgery.
Assuntos
Extrofia Vesical , Epispadia , Incontinência Urinária , Humanos , Extrofia Vesical/cirurgia , Extrofia Vesical/terapia , Extrofia Vesical/complicações , Epispadia/complicações , Epispadia/terapia , Epispadia/cirurgia , Estudos Retrospectivos , Feminino , Masculino , Incontinência Urinária/terapia , Incontinência Urinária/etiologia , Criança , Pré-Escolar , Procedimentos de Cirurgia Plástica/métodos , Resultado do Tratamento , Seguimentos , Qualidade de Vida , Procedimentos Cirúrgicos Urológicos/métodos , AdolescenteRESUMO
INTRODUCTION: The decreased penile length in patients born with bladder exstrophy (BE) results partly from pubic symphysis diastasis and the separation of the corporal bodies. Also, intrinsic shortening of anterior corporal compartment, residual penile dorsal curvature, and postsurgical scarred skin share in creation of short penile length. OBJECTIVE: The goal of this study was to look at whether adult men who had bladder exstrophy (BE) surgery as babies needed and benefited from penile reconstruction, which included penile lengthening and repair of any redo penile pathology that was present at the same time. STUDY DESIGN: We reviewed the records of 31 repaired BE patients with mean age of 21.4 ± 3.7 years. The patients complained of their dissatisfaction with short penile length, residual dorsal penile curvature, distal dorsal or hypospadiac urethral opening and scared penopubic skin. The penile lengthening was performed by sub-periosteal detachment of the corporal bodies from the pubic rami in all cases. In 8 patients full thickness dermal grafts were used to penile resurfacing after its lengthening. Twelve patients underwent coronal or glanular urethroplasty. Phalloplasty was performed in one patient using forearm free graft. RESULTS: Subjective evaluation by the patient reported satisfactory results in 25/31 (80.6 %). The degree of penile lengthening measured at 6 months and one year postoperatively showed increased length which varied between 50 % and 150 % of the preoperative penile length. DISCUSSION: We hypothesize that congenital causes, such as short anterior corporeal compartment, wide pubic rami diastasis, and short penile urethra, as well as iatrogenic causes, such as post-surgical peno-pubic scars, could account for the coexisting multifactorial causes of penile shortening in male adults with BE. Penile lengthening is permitted, in our opinion, provided that the crura from the pubic rami is carefully and partially mobilized. CONCLUSIONS: The short phallus, residual dorsal chordee and distal urethroplasty can be corrected successfully in the majority of patients. Adult males with BE may have short penis that requires another reconstructive stage. The short phallus, residual chordee and distal urethroplasty can be corrected` successfully in the majority of patients.
Assuntos
Extrofia Vesical , Pênis , Procedimentos de Cirurgia Plástica , Procedimentos Cirúrgicos Urológicos Masculinos , Humanos , Extrofia Vesical/cirurgia , Masculino , Pênis/cirurgia , Pênis/anormalidades , Procedimentos de Cirurgia Plástica/métodos , Procedimentos Cirúrgicos Urológicos Masculinos/métodos , Adulto Jovem , Adulto , Estudos Retrospectivos , Adolescente , Resultado do Tratamento , SeguimentosRESUMO
PURPOSE: Bladder exstrophy (BE) poses challenges both during the surgical repair and throughout follow-up. In 2013, a multi-institutional BE consortium was initiated, which included utilization of unified surgical principles for the complete primary repair of exstrophy (CPRE), real-time coaching, ongoing video capture and review of video footage, prospective data collection, and routine patient data analysis, with the goal of optimizing the surgical procedure to minimize devastating complications such as glans ischemia and bladder dehiscence while maximizing the rate of volitional voiding with continence and long-term protection of the upper tracts. This study reports on our short-term complications and intermediate-term continence outcomes. MATERIALS AND METHODS: A single prospective database for all patients undergoing surgery with a BE epispadias complex diagnosis at 3 institutions since February 2013 was used. For this study, data for children with a diagnosis of classic BE who underwent primary CPRE from February 2013 to February 2021 were collected. Data recorded included sex, age at CPRE, adjunct surgeries including ureteral reimplantations and hernia repairs at the time of CPRE, osteotomies, and immobilization techniques, and subsequent surgeries. Data on short-term postoperative outcomes, defined as those occurring within the first 90 days after surgery, were abstracted. In addition, intermediate-term outcomes were obtained for patients operated on between February 2013 and February 2017 to maintain a minimum follow-up of 4 years. Outcomes included upper tract dilation on renal and bladder ultrasound, presence of vesicoureteral reflux, cortical defects on nuclear scintigraphy, and continence status. Bladder emptying was assessed with respect to spontaneous voiding ability, need for clean intermittent catheterization, and duration of dry intervals. All operating room encounters that occurred subsequent to initial CPRE were recorded. RESULTS: CPRE was performed in 92 classic BE patients in the first 8 years of the collaboration (62 boys), including 46 (29 boys) during the first 4 years. In the complete cohort, the median (interquartile range) age at CPRE was 79 (50.3) days. Bilateral iliac osteotomies were performed in 89 (97%) patients (42 anterior and 47 posterior). Of those undergoing osteotomies 84 were immobilized in a spica cast (including the 3 patients who did not have an osteotomy), 6 in modified Bryant's traction, and 2 in external fixation with Buck's traction. Sixteen (17%) patients underwent bilateral ureteral reimplantations at the time of CPRE. Nineteen (21%) underwent hernia repair at the time of CPRE, 6 of which were associated with orchiopexy. Short-term complications within 90 days occurred in 31 (34%), and there were 13 subsequent surgeries within the first 90 days. Intermediate-term outcomes were available for 40 of the 46 patients, who have between 4 and 8 years of follow-up, at a median of 5.7 year old. Thirty-three patients void volitionally, with variable dry intervals. CONCLUSIONS: Cumulative efforts of prospective data collection have provided granular data for evaluation. Short-term outcomes demonstrate no devastating complications, that is, penile injury or bladder dehiscence, but there were other significant complications requiring further surgeries. Intermediate-term data show that boys in particular show encouraging spontaneous voiding and continence status post CPRE, while girls have required modification of the surgical technique over time to address concerns with urinary retention. Overall, 40% of children with at least 4 years of follow-up are voiding with dry intervals of > 1 hour.
Assuntos
Extrofia Vesical , Procedimentos Cirúrgicos Urológicos , Humanos , Extrofia Vesical/cirurgia , Masculino , Feminino , Lactente , Procedimentos Cirúrgicos Urológicos/métodos , Procedimentos Cirúrgicos Urológicos/efeitos adversos , Resultado do Tratamento , Pré-Escolar , Estudos Prospectivos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Fatores de Tempo , Seguimentos , CriançaRESUMO
BACKGROUND: Abdominal and pelvic closure remains a challenge during bladder exstrophy initial repair. We aimed to report on the feasibility and results of a novel technique of bilateral obturator osteotomy. METHODS: Retrospective study of prospective collected data of children who underwent single-stage delayed bladder exstrophy closure combined with RSTM (Radical Soft Tissue Mobilization) for BEEC (Bladder Exstrophy Epispadias Complex) by the same team at different institutions between December 2017 and May 2021. When pubic approximation was not feasible at the end of the procedure, bilateral obturator osteotomy was performed through the same approach, consisting in bilateral divisions of the ilio-pubic rami, ischio-pubic rami, obturator membrane, and detachment of the internal obturator muscle. Pubic bone fragments were approximated together on the midline. Immobilization in a thermoformed posterior splint was indicated for 3 weeks. The main outcome criterion was the bladder dehiscence rate at 6 months, assessed by physical inspection. Secondary outcome criteria included neurovascular obturator pedicle injury, analyzed during orthopedic physical examination, wound or bone infections, gait acquisition, reported by parents and evaluated during medical examination, and vascular penile impairment, judged by penile and glans coloration. RESULTS: 17 children (11 males, 6 females) were included, at a median age of 2 months [1-33]; and representing 29% (17/58) of the children with bladder exstrophy who underwent the same surgical approach during the time of study. There was no postoperative bladder dehiscence with a median follow-up of 34 months [6-47]. No complication was observed. Pelvic X-rays showed bilateral normal ossification process. Neither gait abnormality, nor clinical indication of obturator nerve deficiency was observed during follow-up. CONCLUSION: When pubic bones approximation is not possible, bilateral obturator osteotomy is a useful adjunct in bladder exstrophy closure, feasible by the pediatric urologist through the same approach, and not requiring external fixator. LEVEL OF EVIDENCE: IV.
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Extrofia Vesical , Osteotomia , Humanos , Extrofia Vesical/cirurgia , Osteotomia/métodos , Feminino , Masculino , Estudos Retrospectivos , Lactente , Estudos de Viabilidade , Epispadia/cirurgia , Osso Púbico/cirurgia , Recém-Nascido , Procedimentos Cirúrgicos Urológicos/métodos , Resultado do TratamentoRESUMO
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.
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Parede Abdominal , Extrofia Vesical , Procedimentos de Cirurgia Plástica , Adulto , Humanos , Extrofia Vesical/cirurgia , Parede Abdominal/cirurgia , Estudos Retrospectivos , Retalhos Cirúrgicos/cirurgiaRESUMO
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.
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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 UrináriaRESUMO
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 , PelveRESUMO
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.
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Extrofia Vesical , Criança , Humanos , Lactente , Extrofia Vesical/cirurgia , Músculos , Fator de Crescimento Neural , Bexiga Urinária , UrotélioRESUMO
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.