Cystoscopy and mucosectomy: essentials in the management of Persistent müllerian duct syndrome with transverse testicular ectopia / Cistoscopia y Mucosectomia: Manejo del síndrome de persistencia de los conductos mullerianos con ectopia testicular transversa

ABSTRACT

OBJECTIVES: The concurrence of Persistent Müllerian Duct Syndrome and transverse testicular ectopia is rare. The risk of damage to the vas deferens and the deferential blood supply hinders some surgeons from complete excision of potentially malignant Müllerian duct remnants. METHODS: We present a unique surgical technique of Persistent Müllerian Duct Syndrome in a patient with right inguinal hernia accompanying transverse testicular ectopia. RESULTS: During exploration, both testes were detected in the right inguinal canal. When the hernia sac was opened, a primitive uterus and fallopian tubes without fimbria were identified confirming Persistent Müllerian Duct Syndrome. A 4 Fr catheter was placed into the os of the Müllerian duct remnants via the verumontanumorifice, and then a urethral catheter was placed. The full-thickness excision of proximal Müllerian duct remnant swere performed. The distal part of Müllerian duct remnants was layed open and only mucosa was excised for preserving vas deferens. Resection was completed just above its junction with the urethra with the aid of 4Fr catheter marked at centimeter intervals and the cuffwas oversewn. High ligation for right inguinal hernia and bilateral orchidopexy were performed. CONCLUSIONS: Removal of Müllerian duct remnantsis advised in order to reduce the jeopardy of malignancy, urinary tract infections, stones and hematuria. On the other hand, excision down to urethra which can compromise the integrity and vascularity of the vas deferens is diffucult, even in experienced surgical hands. Complete excision of these structures by mucosectomy of the distal part of remnant which lay closed to vas deferens is a safe and effective method. Cystoscopy assistance and placement of a catheter into MDRs were essential for the complete excision of this mucosa. To the best of our knowledge, cystoscopy assisted mucosectomy in Persistent Müllerian Duct Syndrome has not been reported previously

RESUMEN

OBJETIVOS: La presencia de síndrome de persistencia de los conductos mullerianos y ectopia testicular transversa es raro. El riesgo de dañar el conducto deferente y la vascularización diversa hace que muchos cirujanos no realicen una extirpación completa de los conductos mullerianos remanentes con riesgo de malignización. MÉTODOS: Presentamos una técnica quirúrgica única para la resección completa de los conductos mullerianos remanentes en pacientes con hernia inguinal derecha acompañada de ectopia testicular transversa. RESULTADOS: Durante la exploración física se detectaron ambos testículos en el canal inguinal derecho. Cuando abrimos el saco herniario, se observó un útero primitivo con trompas de falopio sin fimbrias confirmando el síndrome de persistencia de los conductos mullerianos. Se colocó un catéter 4 Fr en la punta del remanente mulleriano a través del orificio del verumontanumy a posteriori se colocó una sonda uretral. CONCLUSIONES: La extirpación del remanente del conducto mulleriano esta indicada para evitar la malignización, infecciones urinarias, litiasis y hematuria. Por otro lado, la extirpación hasta la uretra puede comprometer la vascularización y integridad del conducto deferente, siendo dificultosa hasta en manos expertas. La extirpación completa de las estructuras con mucosectomia de la parte distal del remanente es segura y eficaz. La ayuda de la cistoscopia y colocación de un catéter en el remanente son muy importantes para la resección completa. Esta es la primera descripción de mucosectomia asistida por cistoscopia en un síndrome de persistencia del remanente mulleriano hasta la fecha