Zinner syndrome—a rare developmental anomaly of the mesonephric duct diagnosed on magnetic resonance imaging

Abstract

Developmental anomalies of the urogenital tract are rare but often encountered. Zinner's syndrome is a rare congenital abnormality of mesonephric (Wolffian) duct consisting of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction due to developmental arrest in early embryogenesis affecting the caudal end of Mullerian duct and only approximately a 100 cases have been reported so far. Radiologic modalities such as intravenous pyelography, ultrasonography, vasovesiculography, contrast enhanced computed tomography, and magnetic resonance imaging are all helpful in diagnosis of this unusual entity. We present here an extremely rare developmental anomaly involving the Mullerian ducts, which would remain undiagnosed but for radiologic imaging. The patient presented with symptoms of lower urinary tract irritation.

Prenatal Evaluation and Postnatal Follow-Up of Ureteral Ectopic Insertion in Multicystic Dysplastic Kidneys

“…In boys, ureteral ectopic insertion mostly occurs into the seminal vesicles, but can also be encountered into the lower bladder. Zinner syndrome associates seminal vesicle cysts and ectopic ureter, ejaculatory duct obstruction, and renal agenesis [7,9]. Most probably, it can also include dysplastic kidneys as shown by our series.…”

“…The two smooth cysts described in male fetuses (cases 12 and 14) corresponded to an ectopic insertion into a seminal vesicle cyst in 1 case and into a "pseudobladder diverticulum" in the second one, which may have resulted from an ectopic intravesical ureteral insertion. Lobulated cysts were more frequent in male fetuses (4 out of 5 cases), suggesting distended seminal vesicles [7]. This hypothesis was confirmed postnatally in 3 out of 4 cases.…”

“…In boys, ectopic ureteral insertion into the seminal vesicles may lead to recurrent epididymitis or dysuria and possible subsequent oligozoospermia. Preventive surgery is required [7]. In girls, ectopic insertion into a blind hemivagina does not require surgery during infancy and childhood.…”

“…The association between ureteral ectopic insertion and renal dysplasia is probably linked to a common developmental anomaly. Moreover, the association between ureteral ectopic insertion and genital anomalies is well known, either in the context of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome in girls [4,5] or Zinner syndrome in boys [6,7].…”

Zinner’s Syndrome: Case Report of a Rare Maldevelopment in the Male Genitourinary Tract

“…This syndrome is considered to be the male version of Mayer-Rokitansky-Kuster-Hauser syndrome (5). The diagnosis is usually made between the 2 nd and the 5 th decades of life, concomitant with the period of maximum sexual activity, when cysts start to become more apparent (1).…”