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Kidney transplant is the established treatment for patients with chronic kidney disease but is associated with complications due to the complexity of the procedure. Calyceal fistulas are rare urological complications in transplants caused by arterial occlusion with segmental infarction of the graft. Treatment is based on the extension of the affected area and the clinical status of the patient. For extensive infarctions treated surgically, a total nephrectomy of the transplanted kidney is generally performed. We present a case of a transplanted kidney with polar necrosis and calyceal fistula treated with partial nephrectomy of the affected area, maintaining the graft and preserving kidney function.

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INTRODUCTION: Colorenal fistula is rare in the pediatric population. It may occur at any segment involved by ischemia, chronic inflammation, or necrosis. It is typically associated with a preliminary renal lesion that may arise as a result of interventional procedures, inflammatory conditions, colon tumor, and xanthogranulomatous pyelonephritis, among others. CASE REPORT: 15-year-old female patient diagnosed with acute lymphoblastic leukemia admitted at our institution for baseline condition management. During her stay, she experienced gastrointestinal and urinary infectious events. In the assessment and management of those, a left colorenal fistula was found. Surgical treatment was decided upon. DISCUSSION: Colorenal fistula typically occurs secondary to renal inflammation or infection. Clinical signs are highly variable, and treatment is surgical, with the fistulous tract being resected in all cases.

INTRODUCCION: Las fístulas colorrenales son infrecuentes en la población pediátrica. Pueden desarrollarse en cualquier segmento afectado por isquemia, inflamación crónica o necrosis. Suelen estar asociadas a una lesión primitiva en el riñón que puede producirse por procedimientos intervencionistas, enfermedades inflamatorias, tumorales del colon, pielonefritis xantogranulomatosa, entre otras. CASO CLINICO: Paciente femenina de 15 años, con diagnóstico de leucemia linfoide aguda, ingresa a la institución para recibir manejo de su enfermedad de base. Durante su evolución, desarrolla eventos infecciosos (gastrointestinales y urinarios), y en evaluación y manejo de estos se documenta fístula colorrenal izquierda, motivo por el cual se da un enfoque de tratamiento quirúrgico. COMENTARIOS: La fístula renocólica generalmente se presenta secundaria a procesos inflamatorios o infecciosos renales; su presentación clínica es muy variada, y el tratamiento es quirúrgico, incluyendo siempre la resección del trayecto fistuloso.

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INTRODUCTION: Transposition of the gracilis has been used in a large number of reconstructive procedures. Its advantage is its proximity to these defects and a good blood supply. Traditionally, the gracilis mobilization is performed by open surgery with one or more incisions. We describe our initial experience with the video-endoscopic mobilization of gracilis. METHOD: We described a retrospective review of all patients who underwent gracilis muscle mobilization for treatment of rectourethral fistula, performed by video-endoscopy, between March 2013 and September 2017, for treatment of rectourethral fistula. Also, our surgical technique is described in detail. RESULTS: Three patients, with a mean age of 66.6 years, underwent the procedures. The mean time for mobilization of the gracilis was 107 min (range 60-145). There was no case of donor area infection, no change in the sensitivity of the medial aspect of the thigh or chronic pain. Conversion to open surgery was not necessary in any case. The hospital discharge occurred in average after 4 days. The bladder catheter was removed after 4 weeks after cystography was performed without evidence of leakage. One patient had a recurrence of the fistula. DISCUSSION: The gracilis is an excellent choice of tissue to be interposed in reconstructive procedures of the perineal region, especially in the treatment of rectourinary fistulas. However, endoscopic harvest of the gracilis muscle has not yet found its way into everyday practice. The results in the treatment of rectourinary fistulas are excellent, with a success rate of 87.7%. Our rate of 67% is below, probably due to the small number of cases. In open surgery, complications are uncommon; however, approximately half of the patients expressed concern about the painful scar, which can be reduced by minimally invasive access. CONCLUSION: Video-endoscopic mobilization of gracilis muscle for the treatment of rectourethral fistula is feasible and safe. Studies comparing this technique with the conventional mobilization are required.

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ABSTRACT Purpose: To evaluate efficacy of urorectal fistula (URF) repair using different approaches and the clinical factor determinant of success, and also the morbidity associated to the procedure and health-related quality of life (HRQoL) in male survivors of pelvic malignancies. Material and Methods: Retrospective evaluation of 39 patients with URF primarily intervened in three institutions using different surgical approaches. Success was defined as effective fistula closure. Variables evaluated included demographics, previous treatments, surgical approach, ancillary surgeries, complications and HRQoL by using a standardized non-validated specific questionnaire. Median follow-up from surgery to interview was 55 months (interquartile range 49, range 4-112). Factors determinant of success were investigated using logistic regression. Safety of the procedure was evaluated by Clavien-Dindo scale. Deterioration of continence and erectile function and other HRQoL issues were evaluated. Results: Prostate cancer treatment was the predominant etiology. The success rate for fistula repair was 89.5%. The surgical approach was not related to failed repair (p=0.35) or complications (p=0.29). Factors associated with failure were complications (p=0.025), radiotherapy (p=0.03), fistula location (p=0.04) and fistula size (p=0.007). Multivariate analysis revealed fistula size was the only independent determinant of failure (OR 6.904, 1.01-47.75). Complications occurred in 46.2% and severe complications in 12.8%. The mortality related to the procedure was 2.6%. Urinary incontinence was present before repair in 26.3% and erectile dysfunction in 89.5%. Fistula repair caused de novo urinary incontinence in 7.9% and deterioration of erectile status in 44.7%. Globally 79% were satisfied after repair and only 7.9% rated HRQoL as unhappy. Trans-sphincteric approach was related to less deterioration of erectile function (p=0.003), and higher perceived satisfaction in QoL (p=0.04). Conclusions: The surgical approach elected to correct URF is not determinant of success nor of complications. Fistula size appears as independent determinant for failure. Transsphincteric approach could be advantageous over other procedures regarding HRQoL issues.

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PURPOSE: To evaluate efficacy of urorectal fistula (URF) repair using different approaches and the clinical factor determinant of success, and also the morbidity associated to the procedure and health-related quality of life (HRQoL) in male survivors of pelvic malignancies. MATERIAL AND METHODS: Retrospective evaluation of 39 patients with URF primarily intervened in three institutions using different surgical approaches. Success was defined as effective fistula closure. Variables evaluated included demographics, previous treatments, surgical approach, ancillary surgeries, complications and HRQoL by using a standardized non-validated specific questionnaire. Median follow-up from surgery to interview was 55 months (interquartile range 49, range 4-112). Factors determinant of success were investigated using logistic regression. Safety of the procedure was evaluated by Clavien-Dindo scale. Deterioration of continence and erectile function and other HRQoL issues were evaluated. RESULTS: Prostate cancer treatment was the predominant etiology. The success rate for fistula repair was 89.5%. The surgical approach was not related to failed repair (p=0.35) or complications (p=0.29). Factors associated with failure were complications (p=0.025), radiotherapy (p=0.03), fistula location (p=0.04) and fistula size (p=0.007). Multivariate analysis revealed fistula size was the only independent determinant of failure (OR 6.904, 1.01-47.75). Complications occurred in 46.2% and severe complications in 12.8%. The mortality related to the procedure was 2.6%. Urinary incontinence was present before repair in 26.3% and erectile dysfunction in 89.5%. Fistula repair caused de novo urinary incontinence in 7.9% and deterioration of erectile status in 44.7%. Globally 79% were satisfied after repair and only 7.9% rated HRQoL as unhappy. Trans-sphincteric approach was related to less deterioration of erectile function (p=0.003), and higher perceived satisfaction in QoL (p=0.04). CONCLUSIONS: The surgical approach elected to correct URF is not determinant of success nor of complications. Fistula size appears as independent determinant for failure. Trans-sphincteric approach could be advantageous over other procedures regarding HRQoL issues.

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Introducción: La eliminación de orina que con tiene grandes cantidades de material quiloso y adquiere un aspecto lechoso se conoce con el nombre de quiluria. Salvo en las regiones donde es endémica, la filariasis linfática, principal etiología, se considera en general como un trastorno raro. Objetivo: Describir un caso de quiluria asociada al embarazo. Caso clínico: Paciente femenina de 32 años de edad, con múltiples ingresos en el Servicio de Nefrología del Hospital Celia Sánchez Manduley desde el año 2007 por presentar orinas de color blanquecino, cuadro que comenzó con el primer embarazo, el síntoma desapareció y luego reapareció con el segundo embarazo, primero de forma intermitente y después, adquirió carácter permanente, con hematuria, proteinuria, edemas y otras alteraciones clínicas y bioquímicas. Conclusiones: La nefrectomía está indicada en la quiluria, cuando pone en riesgo la vida del paciente o afecta su calidad de vida, si no se tienen otras alternativas terapéuticas(AU)

Introduction: The elimination of urine containing large amounts of chyllous material and acquiring a milky appearance is known as quiluria. Except in regions where it is endemic, lymphatic filariasis, the main etiology, is generally considered to be a rare disorder. Objective: To describe a case of chyluria associated with pregnancy. Clinical case: 32-year-old female patient with multiple admissions to the nephrology service at Hospital Celia Sánchez Manduley since 2007 for presenting whitish urine, a clinical picture that began with the first pregnancy. The symptom disappeared, then reappeared with the second pregnancy, first intermittently and then became permanent with hematuria, proteinuria, edema and other clinical and biochemical alterations. Conclusions: Nephrectomy is indicated in chyluria when it puts the patient's life at risk and affects the quality of life, if there are no other therapeutic alternatives(AU)

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BACKGROUND: Bladder catheterization duration after urinary obstetric fistula surgery varies widely. OBJECTIVE: To assess the effect of bladder catheterization duration after urinary obstetric fistula surgery. SEARCH STRATEGY: Medline, EMBASE, CINAHL, GIM, and POPLINE databases were searched, without language restrictions, using "obstetric urinary fistula" and "catheterization" from inception to September 30, 2017. SELECTION CRITERIA: Randomized controlled trials comparing shorter versus longer (>10 days) bladder catheterization after urinary obstetric fistula repair were included. DATA COLLECTION AND ANALYSIS: Data were extracted and meta-analyses were conducted. The GRADE system was used to assess evidence quality. MAIN RESULTS: Two unblinded non-inferiority trials (684 patients combined) were included. There were no differences between shorter and longer bladder catheterization in the risk of fistula repair breakdown either before (relative risk [RR] 1.14; 95% confidence interval [CI] 0.49-2.64) or after (RR 1.64; 95% CI 0.81-3.31) hospital discharge. Similarly, urinary infection (RR 5.18; 95% CI 0.25-107.44); urinary incontinence before (RR 1.15; 95% CI 0.54-2.43) or after (RR 1.16; 95% CI 0.62-2.18) discharge; urinary retention (RR 1.34; 95% CI 0.79-2.27); or extended hospital stay (RR 9.33; 95% CI 0.51-172.41) were not associated with duration of catheterization. Evidence quality was low or moderate. CONCLUSIONS: Shorter, compared to longer, bladder catheterization duration after urinary obstetric fistula surgery was not associated with significant outcome differences.

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A doença do trato urinário inferior dos felinos, DTUIF, acomete gatos e tem como sinais clínicos disúria, hematúria e polaciúria. Tais sinais podem estar relacionados a obstrução uretral parcial ou total. Machos tem maior predisposição do que as fêmeas por possuírem a uretra peniana mais estreita, a idade de maior ocorrência é entre dois e seis anos, e ainda animais castrados são mais susceptíveis a desenvolver a doença. Nestes casos a indicação é a desobstrução por meio de cateterização da uretra peniana sob anestesia geral. Em casos recorrentes deve-se indicar a cirurgia de uretrostomia. Este procedimento cirúrgico tem o objetivo de criar uma fístula ligando diretamente a uretra e a pele. O resultado pós-cirúrgico é excelente evitando novas obstruções, mas também é passível de complicações como estenose que ocorre devido ao processo de retração cicatricial, mas pode ser excessiva por falta de cuidados pós-operatórios Este relato tem o objetivo de descrever um caso de estenose após a uretrostomia em um gato que apresentou complicações pós-operatórias e a nova cirurgia para refazer a fístula.

Feline disease of inferior urinary tract affects cats and its clinical signs are dysuria, hematuria and pollakiuria. This signs are related a parcial or total urethral obstruction. Males have more predisposition than females because their penile urethra are thin. Cats between two and six years old and castrated animals are more susceptible to developing the disease. In this cases is indicated the opening with aid of a probe under general anesthesia. In obstruction recurring cases it should be point a perineal urethrostomy surgery. This objective surgery is create a fistula between the urethra and the skin. The post-operative result are excellent and avoid new obstructions, but can have complications such as stenosis. The stenosis is a normal fact in a healing process, but can be excessive when the lack of care in the post-operative period that allow the animal stir in the wound before the complete healing. This case report presents a urethral stenosis after the urethrostomy surgery in a cat and your treatment.

La enfermedad del trato urinário inferior de los felinos acomete gatos y tiene como señales clínicos disúria, hematuria y polaciúria. Tales señales pueden estar relaciuonados a La obstrução uretral parcial o total. Machos tienen mayor predisposição que las hembras por tener la uretra peniana mas estrecha, la edad de mayor ocurrencia es entre dos y seis años, y aún animales castrados son más susceptibles a desarrollar la enfermedad. En estos casos la indicación es la desobstrución por médio de cateterizacion de la uretra peniana bajo anestesia general. En casos recurrentes se debe indicar la cirugía de uretrostomia. Este procedimento tiene el objetivo de crear una fístula conectando directamente la uretra y la piel. El resultado post cirúrgico es excelente evitando nuevas obstruciones, pero también es pasible de complicaciones como estenosis que ocurre debido al proceso de retracion cicatricial, pero puede se excesiva por falta de cuidados post operatórios. Este relato tiene el objetivo de describir un caso de estenosis después de la uretrostomia en un gato que presentó complicaciones post operatórias y la nueva cirugía para rehacer la fístula.

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A doença do trato urinário inferior dos felinos, DTUIF, acomete gatos e tem como sinais clínicos disúria, hematúria e polaciúria. Tais sinais podem estar relacionados a obstrução uretral parcial ou total. Machos tem maior predisposição do que as fêmeas por possuírem a uretra peniana mais estreita, a idade de maior ocorrência é entre dois e seis anos, e ainda animais castrados são mais susceptíveis a desenvolver a doença. Nestes casos a indicação é a desobstrução por meio de cateterização da uretra peniana sob anestesia geral. Em casos recorrentes deve-se indicar a cirurgia de uretrostomia. Este procedimento cirúrgico tem o objetivo de criar uma fístula ligando diretamente a uretra e a pele. O resultado pós-cirúrgico é excelente evitando novas obstruções, mas também é passível de complicações como estenose que ocorre devido ao processo de retração cicatricial, mas pode ser excessiva por falta de cuidados pós-operatórios Este relato tem o objetivo de descrever um caso de estenose após a uretrostomia em um gato que apresentou complicações pós-operatórias e a nova cirurgia para refazer a fístula.(AU)

Feline disease of inferior urinary tract affects cats and its clinical signs are dysuria, hematuria and pollakiuria. This signs are related a parcial or total urethral obstruction. Males have more predisposition than females because their penile urethra are thin. Cats between two and six years old and castrated animals are more susceptible to developing the disease. In this cases is indicated the opening with aid of a probe under general anesthesia. In obstruction recurring cases it should be point a perineal urethrostomy surgery. This objective surgery is create a fistula between the urethra and the skin. The post-operative result are excellent and avoid new obstructions, but can have complications such as stenosis. The stenosis is a normal fact in a healing process, but can be excessive when the lack of care in the post-operative period that allow the animal stir in the wound before the complete healing. This case report presents a urethral stenosis after the urethrostomy surgery in a cat and your treatment.(AU)

La enfermedad del trato urinário inferior de los felinos acomete gatos y tiene como señales clínicos disúria, hematuria y polaciúria. Tales señales pueden estar relaciuonados a La obstrução uretral parcial o total. Machos tienen mayor predisposição que las hembras por tener la uretra peniana mas estrecha, la edad de mayor ocurrencia es entre dos y seis años, y aún animales castrados son más susceptibles a desarrollar la enfermedad. En estos casos la indicación es la desobstrución por médio de cateterizacion de la uretra peniana bajo anestesia general. En casos recurrentes se debe indicar la cirugía de uretrostomia. Este procedimento tiene el objetivo de crear una fístula conectando directamente la uretra y la piel. El resultado post cirúrgico es excelente evitando nuevas obstruciones, pero también es pasible de complicaciones como estenosis que ocurre debido al proceso de retracion cicatricial, pero puede se excesiva por falta de cuidados post operatórios. Este relato tiene el objetivo de describir un caso de estenosis después de la uretrostomia en un gato que presentó complicaciones post operatórias y la nueva cirugía para rehacer la fístula.(AU)

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Background: The anorectal anomalies consist in a complex group of birth defects. Laparoscopic-assisted anorectoplasty improved visualization of the rectal fistula and the ability to place the pull-through segment within the elevator muscle complex with minimal dissection. There is no consensus on how the fistula should be managed. Aim: To evaluate the laparoscopic-assisted anorectoplasty and the treatment of the rectal urinary fistula by a bipolar sealing device. Method: It was performed according to the original description by Georgeson1. Was used 10 mm infraumbilical access portal for 30º optics. The pneumoperitoneum was established with pressure 8-10 cm H2O. Two additional trocars of 5 mm were placed on the right and left of the umbilicus. The dissection started on peritoneal reflection using Ligasure(r). With the reduction in the diameter of the distal rectum was identified the fistula to the urinary tract. The location of the new anus was defined by the location of the external anal sphincter muscle complex, using electro muscle stimulator externally. Finally, it was made an anastomosis between the rectum and the new location of the anus. A Foley urethral probe was left for seven days. Results: Seven males were operated, six with rectoprostatic and one with rectovesical fistula. The follow-up period ranged from one to four years. The last two patients operated underwent bipolar sealing of the fistula between the rectum and urethra without sutures or surgical ligation. No evidence of urethral leaks was identified. Conclusion: There are benefits of the laparoscopic-assisted anorectoplasty for the treatment of anorectal anomaly. The use of a bipolar energy source that seals the rectal urinary fistula has provided a significant decrease in the operating time and made the procedure be more elegant.

Racional: As anomalias anorretais consistem de um grupo complexo de defeitos congênitos. A anorretoplastia laparoscópica permite melhor visualização da fístula retourinária e propicia o posicionamento do reto abaixado dentro do complexo muscular do elevador do ânus com mínima dissecção. Não há consenso na literatura sobre o melhor tratamento dessa fístula. Objetivo: Avaliar a anorretoplastia laparoscópica e o selamento bipolar da fístula retourinária. Método: Ela foi realizada de acordo com a descrição original de Georgeson1. Utilizou-se o acesso infraumbilical com portal de 10 mm para a ótica de 30º. O pneumoperitônio foi estabelecido com pressão de 8-10 cm de H2O. Dois trocárteres adicionais de 5 mm foram colocados à direita e à esquerda da cicatriz umbilical. A dissecção foi iniciada na reflexão peritoneal usando Ligasure(r). Com a redução do calibre do reto distalmente, foi identificada a fístula para a o trato urinário. O local do novo ânus foi definido por meio da localização do complexo muscular do esfíncter anal externo, utilizando-se estimulador eletro muscular externamente. Por fim, foi confeccionada uma anastomose entre o reto e o novo local do ânus. Uma sonda uretral de Foley foi deixada durante sete dias. Resultados: Sete meninos foram operados, seis com fístula retoprostática e um retovesical. O período de seguimento variou de um a quatro anos. Os dois últimos pacientes operados foram submetidos ao selamento bipolar da fístula entre o reto e a uretra, sem suturas ou ligadura cirúrgica com pontos. No seguimento em longo prazo não houve evidências de fístulas urinárias. Conclusão: Há benefícios da anorretoplastia laparoscópica para o tratamento de anomalia anorretal. O uso de uma fonte de energia bipolar que promova o selamento da fístula retourinária propiciou redução significativa do tempo cirúrgico e tornou o procedimento mais elegante.

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ABSTRACT Background: The anorectal anomalies consist in a complex group of birth defects. Laparoscopic-assisted anorectoplasty improved visualization of the rectal fistula and the ability to place the pull-through segment within the elevator muscle complex with minimal dissection. There is no consensus on how the fistula should be managed. Aim: To evaluate the laparoscopic-assisted anorectoplasty and the treatment of the rectal urinary fistula by a bipolar sealing device. Method: It was performed according to the original description by Georgeson1. Was used 10 mm infraumbilical access portal for 30º optics. The pneumoperitoneum was established with pressure 8-10 cm H2O. Two additional trocars of 5 mm were placed on the right and left of the umbilicus. The dissection started on peritoneal reflection using Ligasure(r). With the reduction in the diameter of the distal rectum was identified the fistula to the urinary tract. The location of the new anus was defined by the location of the external anal sphincter muscle complex, using electro muscle stimulator externally. Finally, it was made an anastomosis between the rectum and the new location of the anus. A Foley urethral probe was left for seven days. Results: Seven males were operated, six with rectoprostatic and one with rectovesical fistula. The follow-up period ranged from one to four years. The last two patients operated underwent bipolar sealing of the fistula between the rectum and urethra without sutures or surgical ligation. No evidence of urethral leaks was identified. Conclusion: There are benefits of the laparoscopic-assisted anorectoplasty for the treatment of anorectal anomaly. The use of a bipolar energy source that seals the rectal urinary fistula has provided a significant decrease in the operating time and made the procedure be more elegant.

RESUMO Racional: As anomalias anorretais consistem de um grupo complexo de defeitos congênitos. A anorretoplastia laparoscópica permite melhor visualização da fístula retourinária e propicia o posicionamento do reto abaixado dentro do complexo muscular do elevador do ânus com mínima dissecção. Não há consenso na literatura sobre o melhor tratamento dessa fístula. Objetivo: Avaliar a anorretoplastia laparoscópica e o selamento bipolar da fístula retourinária. Método: Ela foi realizada de acordo com a descrição original de Georgeson1. Utilizou-se o acesso infraumbilical com portal de 10 mm para a ótica de 30º. O pneumoperitônio foi estabelecido com pressão de 8-10 cm de H2O. Dois trocárteres adicionais de 5 mm foram colocados à direita e à esquerda da cicatriz umbilical. A dissecção foi iniciada na reflexão peritoneal usando Ligasure(r). Com a redução do calibre do reto distalmente, foi identificada a fístula para a o trato urinário. O local do novo ânus foi definido por meio da localização do complexo muscular do esfíncter anal externo, utilizando-se estimulador eletro muscular externamente. Por fim, foi confeccionada uma anastomose entre o reto e o novo local do ânus. Uma sonda uretral de Foley foi deixada durante sete dias. Resultados: Sete meninos foram operados, seis com fístula retoprostática e um retovesical. O período de seguimento variou de um a quatro anos. Os dois últimos pacientes operados foram submetidos ao selamento bipolar da fístula entre o reto e a uretra, sem suturas ou ligadura cirúrgica com pontos. No seguimento em longo prazo não houve evidências de fístulas urinárias. Conclusão: Há benefícios da anorretoplastia laparoscópica para o tratamento de anomalia anorretal. O uso de uma fonte de energia bipolar que promova o selamento da fístula retourinária propiciou redução significativa do tempo cirúrgico e tornou o procedimento mais elegante.

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While uncommon, ureteral arterial fistula (UAF) should be a differential diagnosis for persistent hematuria, as management involves coordinated treatment with a multidisciplinary team. Despite various diagnostic modalities available, accuracy in diagnosis remains a challenge. We present a patient with known UAF risk factors, including chronic ureteral stent, history of radiation, and vascular procedures. Despite multiple negative imaging studies, UAF was ultimately diagnosed and successfully managed by an endovascular approach, with resolution of her hematuria.

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PURPOSE OF REVIEW: Urological fistulas are an underestimated problem worldwide and have devastating consequences for patients. Many urological fistulas result from surgical complications and/or inadequate perinatal obstetric healthcare. Surgical correction is the standard treatment. This article reviews minimally invasive surgical approaches to manage urological fistulas with a particular emphasis on the robotic techniques of fistula correction. RECENT FINDINGS: In recent years, many surgeons have explored a minimally invasive approach for the management of urological fistulas. Several studies have demonstrated the feasibility of laparoscopic surgery and the reproducibility of reconstructive surgery techniques. Introduction of the robotic platform has provided significant advantages given the improved dexterity and exceptional vision that it confers. SUMMARY: Fistulas are a concern worldwide. Laparoscopic surgery correction has been developed through the efforts of several authors, and difficulties such as the increased learning curve have been overcome with innovations, including the robotic platform. Although minimally invasive surgery offers numerous advantages, the most successful approach remains the one with the surgeon is most familiar.

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We present the case of a male patient who required treaatment due to anorectal agenesis with recto urethral fistula and penoscrotal transposition with perineal hypospadias, associated with a perineal tumor. The perineal tumor was found strongly adhered and contiguous to the rectum which makes it compatible with an exstrophy of rectal duplication. Surgical reconstruction of the birth defect was performed in stages until acceptable biological function and esthetic results were obtained.

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