Minimally invasive management of Zinner's syndrome / Manejo mínimamente invasivo del síndrome de Zinner

Abstract Zinner's syndrome (ZS) is a rare congenital malformation of the seminal vesicles and ipsilateral upper urinary tract which is characterized by the triad of ipsilateral ejaculatory duct obstruction, semi nal vesicle cysts, and renal agenesis. We report an 18-year-old male who presented intermittent gross hematuria and hematospermia and ejaculation volume diminution. ZS was diagnosed with magnetic resonance imaging (MRI) of the prostate, among other complementary studies requested. We performed a minimally laparoscopic resection of the left seminal vesicle. The postoperative was uneventful symptoms resolved, and 3 months later semen analysis showed increase in ejaculation volume. Currently, the minimally invasive approach is feasible and effective for the treatment of the seminal vesicle cysts excision in the ZS. We presented a symptomatic patient treated by a laparoscopic approach with successful middle follow-up results.

Resumen El síndrome de Zinner (ZS) es una malformación congénita rara de las vesículas seminales y del tracto urinario superior ipsolateral que se caracteriza por la tríada de obstrucción del conducto eyaculatorio ipsolateral, quistes de vesículas seminales y agenesia renal. Presentamos el caso de un varón de 18 años que presentó hematuria macroscópica intermitente y hemospermia y disminución del volumen de eyaculación. El diagnóstico de ZS se realizó mediante resonan cia magnética (RM) de próstata, entre otros estudios complementarios solicitados. Realizamos una resección mínimamente laparoscópica de la vesícula seminal izquierda. El postoperatorio se resolvió sin complicaciones y 3 meses después el análisis de semen mostró un aumento en el volumen de eyaculación. Actualmente, el abordaje mínimamente invasivo es factible y efectivo para el tratamiento de la escisión de quistes de vesículas seminales en el ZS. Presentamos un paciente sintomático tratado por vía laparoscópica con seguimiento medio exitoso.

Ectopic ureter associated with Zinners syndrome in a kidney recipient: case report and literature review

SUMMARY INTRODUCTION Zinner's Syndrome is a triad of mesonephric duct anomalies comprising unilateral renal agenesis, seminal vesicle cyst, and ejaculatory duct obstruction. In this study, we present a kidney recipient with ectopic ureter associated with Zinner's syndrome and a literature review. CASE PRESENTATION A 59-year-old male with a history of chronic kidney disease and left renal agenesis underwent deceased donor kidney transplantation. After securing optimal renal functions, the patient underwent abdominal computed tomography (CT) scan for the seroma that occurred under the incision. The final diagnosis was an ectopic distal ureter ending in the seminal vesicle cyst's wall and ipsilateral renal agenesis. The patient was discharged without any complications and the clinical follow up was uneventful. DISCUSSION AND CONCLUSION Congenital seminal vesicle disorders are usually associated with ipsilateral urinary duct anomalies stemming from the same embryonic structure. To our knowledge, this is the first case report that describes kidney transplantation in a patient with ipsilateral renal agenesis and ectopic ureter ending in the seminal vesicle cyst. In patients with renal agenesis, during the ipsilateral urinary tract anastomosis, the possibility of ectopic ureter should be kept in mind otherwise graft loss can occur with a high morbidity rate.

RESUMO INTRODUÇÃO A Síndrome de Zinner é uma tríade de anomalias do ducto mesonéfrico que compreende agenesia renal unilateral, cisto da vesícula seminal e obstrução do ducto ejaculatório. Neste estudo, apresentamos um receptor de rim com ureter ectópico associado à Síndrome de Zinner e revisão da literatura. APRESENTAÇÃO DO CASO Homem de 59 anos com história de doença renal crônica e agenesia renal esquerda foi submetido a transplante de rim de doador falecido. Após função renal ideal, foi realizada tomografia computadorizada do abdome (TC) devido ao seroma sob incisão. O diagnóstico final foi um ureter distal ectópico que termina na parede do cisto da vesícula seminal e agenesia renal ipsilateral. O paciente recebeu alta sem complicações e o acompanhamento clínico ocorreu sem intercorrências. DISCUSSÃO E CONCLUSÃO Os distúrbios congênitos da vesícula seminal geralmente estão associados às anomalias do ducto urinário ipsilateral devido a uma mesma estrutura embrionária. Até onde sabemos, é o primeiro relato de caso que descreve o transplante renal em um paciente com agenesia renal ipsilateral e ureter ectópico terminado no cisto da vesícula seminal. Em pacientes com agenesia renal, durante a anastomose do trato urinário ipsilateral, deve-se ter em mente a possibilidade do ureter ectópico, caso contrário, poderá ocorrer perda do enxerto com alta taxa de morbidade.

Sindrome de zinner. a propósito de un caso clínico / Zinner syndrome. report of a clinical case

Introducción: El Sindrome de Zinner se caracteriza por dilatación quística unilateral de la vesícula seminal y atrofia o agenesia del riñón ipsilateral, siendo producto de una alteración congénita de los conductos de Wolff. Objetivos: Presentación de caso clínico y revisión bibliográfica. Reporte de caso: Joven de 18 años que consulta por dolor testicular intermitente, sordo y mal localizado de un año de duración, que se acompaña de una disminución del volumen del líquido espermático eyaculado. Se procede a realizar ecografía abdominal, la cual informa agenesia renal derecha y vesícula seminal derecha. Con los hallazgos clínicos e imagenológicos se diagnóstica sindrome de Zinner. Discusión: La mayoría de los pacientes con este grupo de anomalías del conducto mesonéfrico son asintomáticos hasta la tercera o cuarta década de la vida. La ecografía es el método diagnóstico inicial por su accesibilidad y sirve para descartar otras causas de dolor pélvico y demostrar la agenesia renal, así como la imagen quística de la pelvis. La resonancia magnética es el método de elección para evaluar malformaciones del conducto mesonéfrico. (AU)

Introduction: Zinner syndrome is characterized by unilateral cystic dilatation of the seminal vesicle and atrophy or agenesis of ipsilateral kidney, being the product of a congenital abnormality of the Wolffian ducts. Objectives: Presentation of a case and literature review. Case report: 18 year old who complains of intermittent testicular pain, poorly localized, associated with ejaculate seminal fluid volume reduction. We performed an abdominal ultrasound, which reports right renal and right seminal vesicle agenesis. With clinical and image findings, Zinner syndrome is diagnosed .Discussion: Most patients with this group of mesonephric duct abnormalities are asymptomatic until the third or fourth decade of life. Ultrasound is the initial diagnostic method for its accessibility and it serves to rule out other causes of pelvic pain. It proves renal agenesis and cystic image on the pelvis. MRI is the best method to assess mesonephric ducts abnormalities. (AU)

Sindrome de zinner. a propósito de un caso clínico / Zinner syndrome. report of a clinical case

Introducción: El Sindrome de Zinner se caracteriza por dilatación quística unilateral de la vesícula seminal y atrofia o agenesia del riñón ipsilateral, siendo producto de una alteración congénita de los conductos de Wolff. Objetivos: Presentación de caso clínico y revisión bibliográfica. Reporte de caso: Joven de 18 años que consulta por dolor testicular intermitente, sordo y mal localizado de un año de duración, que se acompaña de una disminución del volumen del líquido espermático eyaculado. Se procede a realizar ecografía abdominal, la cual informa agenesia renal derecha y vesícula seminal derecha. Con los hallazgos clínicos e imagenológicos se diagnóstica sindrome de Zinner. Discusión: La mayoría de los pacientes con este grupo de anomalías del conducto mesonéfrico son asintomáticos hasta la tercera o cuarta década de la vida. La ecografía es el método diagnóstico inicial por su accesibilidad y sirve para descartar otras causas de dolor pélvico y demostrar la agenesia renal, así como la imagen quística de la pelvis. La resonancia magnética es el método de elección para evaluar malformaciones del conducto mesonéfrico.

Introduction: Zinner syndrome is characterized by unilateral cystic dilatation of the seminal vesicle and atrophy or agenesis of ipsilateral kidney, being the product of a congenital abnormality of the Wolffian ducts. Objectives: Presentation of a case and literature review. Case report: 18 year old who complains of intermittent testicular pain, poorly localized, associated with ejaculate seminal fluid volume reduction. We performed an abdominal ultrasound, which reports right renal and right seminal vesicle agenesis. With clinical and image findings, Zinner syndrome is diagnosed .Discussion: Most patients with this group of mesonephric duct abnormalities are asymptomatic until the third or fourth decade of life. Ultrasound is the initial diagnostic method for its accessibility and it serves to rule out other causes of pelvic pain. It proves renal agenesis and cystic image on the pelvis. MRI is the best method to assess mesonephric ducts abnormalities.