SEMINAL VESICLE CYST WITH IPSILATERAL RENAL AGENESIS (ZINNER SYNDROME): CASE REPORT

Jihane Habi, Sophia Berrada, Hind Guerroum, Mariam Kassimi, Nabil Chikhaoui and Mohamed Mahi Department of Radiology, Cheikh Khalifa Hospital / Mohammed VI University of Health Sciences, Casablanca, Morocco. ...................................................................................................................... Manuscript Info Abstract ......................... ........................................................................ Manuscript History Received: 25 January 2020 Final Accepted: 27 February 2020 Published: March 2020


Discussion:-
Zinner's syndrome is an uncommon birth defect, described like a seminal vesicle cyst associated with ipsilateral renal agenesis and obstruction of the ejaculatory duct.
The association between this triad of malformation is due to their common embryological origin (Mesonephric or Wolffian duct).
Maldevelopment of the distal mesonephric duct results in absence of the ureteral bud, and, therefore, ipsilateral renal agenesis or dysplasia in addition to atresia of the ejaculatory duct with subsequent obstruction of the seminal vesicles and formation of cysts. [2] The syndrome was first described by Zinner since 1914 [3]. Since then, it has often been reported on the right and rarely on the left.
This syndrome is the prerogative of patients between the 2nd and the 5th decade, at the beginning of sexual activity by distention of the seminal vesicle or during an assessment of infertility. Its discovery is often fortuitous on clinical examination with digital rectal examination by the palpation of a mass in retro prostatic region or during a radiological exam when the cyst of the seminal vesicle is of size <5cm. If the size of the cyst is > 5cm, it can be discovered by multiple and varied urogenital symptoms (Pain pelvic, dysuria, pain on ejaculation, hemospermia, infertility or even abdominal pain).
Cysts of the seminal vesicles greater than 12 cm have been termed "giant" cysts and often are present with symptoms of bladder and colonic obstruction because of mass effect [4].
Imaging is more than enough to make a positive diagnosis.
Ultrasound provides information on the nature of the cyst, its retro-vesical location, its size and appearance (An anechoic pelvic mass with a thick, irregular wall and occasional wall calcifications, or the mass may contain internal debris reflecting prior hemorrhage or infection [5]. On sonography, ipsilateral kidney is absent, and the obstructed ejaculatory ducts are seen as anechoic structures in the pelvis, though hemorrhage or infection can produce internal low level echoes) [6]. For our patient, pelvic ultrasound could only detect the retro-vesical mass.
Vasovesiculography is an invasive means of imaging which consists of injecting the iodized contrast and checking under scope the passage of the product towards the ectopic ureteral bud testifying to an obstruction of the ejaculatory duct. This examination was not released for our patient due to its very invasive nature.
CT is much more reliable than ultrasound to show ipsilateral renal agenesis. Reported findings have been variable ranging from a cystic pelvic mass with a thick irregular wall to a solid mass and apparent enlargement of the ipsilateral seminal vesicle [7]. The solitary cysts had irregular margins (9/14, 64.3%) or tubulosaccular shape (5/14, 35.7%) [8].The cyst density was slightly greater than that of urine with an average CT value of 8 ± 5 HU (range: 3-15 HU) and no contrast enhancement [8]. It can also have a spontaneously hyperdense content if its content is highly protein like the aspect that we found in our patient.
MRI is the modality of choice for precise delineation of the anatomy of the male genital tract, for demonstrating the seminal vesicles and evaluating anomalies of the mesonephric duct and to differentiate the seminal vesicle cysts from other cystic pelvic masses [9]. The seminal vesicle cyst is generally hyposignal in T1 and hypersignal in T2 without contrast enhancement. Old hemorrhage and a high proteinaceous concentration in the seminal fluid with old spermatozoa are established features of seminal vesicle cysts; these conditions shorten T1 time and account for the high signal intensity [10]. It also shows the termination of the ectopic ureter into the seminal vesicle and the dilation of the ejaculatory duct.

For the treatement:
Asymptomatic patients have conservative treatment. This conservative approach will help to prevent possible side effects (e.g. erectile dysfunction) from surgical intervention. [11] For symptomatic patient, invasive treatments including transurethral resection of the ejaculatory duct (TURED), exploration, laparoscopic and robotic vesiculectomy are suggested [12]. Clogging the ejaculatory duct can solve the problem of infertility.