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15:44 - 15:47
S3-4
(CP)
AND IF IT WAS A ZINNER'S SYNDROME?
Massimiliano SILVERI
1
, Antonio ZACCARA
2
, Mauro COLAJACOMO
3
, Ottavio Domenico ADORISIO
1
, Francesca DIOMEDI
CAMASSEI
4
and Mario DE GENNARO
5
1) Bambino Gesù Children's Research Hospital, Department of Surgery and Transplantation Center, Palidoro - Rome,
ITALY - 2) Bambino Gesu' Children's Research Hospital, Department of Nephrology and Urology, Rome, ITALY - 3)
Bambino Gesù Children's Research Hospital, Department of Imaging, Palidoro - Rome, ITALY - 4) Bambino Gesù
Children's Research Hospital, Department of Pathology, Rome, ITALY - 5) Bambino Gesù Children's Research Hospital,
Department of Nephrology and Urology, Rome, ITALY
PURPOSE
First described in 1914 by Zinner, the triad consisting of renal agenesis, ipsilateral seminal vesicle cyst (SVC) and
eiaculatory duct obstruction has been reported in a hundred cases, predominantly adults or adolescents. Since
symptoms related to SVC coincide with the onset of a sexually active life, the majority of the cases reported are
observed in the second and third decade of life.
MATERIAL AND METHODS
A 2-year-old boy with an unremarkable prenatal diagnosis was admitted to the Emergency Department with abdominal
pain and constipation.
RESULTS
Ultrasound (US) showed a large cystic mass between the bladder and the rectum along with absence of the left kidney.
Magnetic resonance (MR) confirmed the findings. Endoscopy showed that the cyst was attributable to the male genital
tract through the presumable outlet of a seminal vesicle into the posterior urethra. A clinical worsening (pain,
constipation, pelvic discomfort) prompted surgical excision of the cystic mass. At surgery, a blind end vas deferens
entering into the SVC was found and the entire mass was excised close to the urethra previously cannulated. Pathology
confirmed SVC with the typical fibromuscular layer lined with cubic epithelium. The patient had an uneventful course and
at 10 month post-operative follow-up is in good health without symptoms.
CONCLUSIONS
Even though diagnosis of Zinner's syndrome usually recalls fertility issues in adolescent and adult age, there is evidence
that such association must be also taken into consideration in the differential diagnosis of congenital anomalies of
internal male genitalia in pediatric age. Treatment may be mandatory with regard to possible onset of bladder
dysfunction or epididymo-orchitis that can arise with a relative frequency and result in a significant morbidity in these
patients. Whether or not treatment and follow-up in pediatric age may prevent impairment of fertility potential later in
life remains to be clarified.