Zinner syndrome in pediatric patients: rare disease leading to challenging management

Abstract

Introduction: Zinner syndrome (ZS) is the association of seminal vesicle cysts, ipsilateral ejaculatory duct obstruction, and ipsilateral renal agenesis. This condition is very rare in children and both diagnosis and treatment may be challenging. We reviewed the clinical presentation and treatment describing our experience with a series of three patients.

Methods: From January 2016 to January 2021, three patients (patients 1, 2, and 3) with symptomatic ZS, aged 2, 15, and 17 years, respectively, were diagnosed and treated. All three patients were symptomatic, manifesting pelvic pain and dysuria. The diagnosis was made by physical examination, ultrasonography, and abdominopelvic MRI. Patient 1 underwent open surgery, while for patients 2 and 3, laparoscopic excision was performed.

Results: The renal agenesis regarded the left side in patients 1 and 3, and the right side in patient 2. In all cases, the cystic complex was excised. The mean operating time was 4 h and the mean hospitalization time was 5 days (range 4-6 days). The mean follow-up period was 5 years (range 2-5 years). Patients 1 and 3 showed a complete resolution of the symptoms during postoperative follow-up. In patient 2, clinical symptoms relapsed because of the persistence of a 9 mm cyst requiring a redo laparoscopic excision.

Conclusions: Seminal vesicle cyst with ipsilateral renal agenesis, even if rare in pediatric age, should be suspected in young male patients presenting with pelvic cystic masses, pelvic pain, dysuria, and ipsilateral renal absence. Conservative management should be reversed to asymptomatic patients. Surgical treatment is mandatory in symptomatic cases and the preferred approach is minimally invasive surgery to magnify the operating field to spare anatomical structures, primarily the contralateral vas deferens. Radicality is crucial to avoid the persistence of symptoms and the need for reintervention.

Keywords: Zinner syndrome; ejaculatory duct; pelvic pain; renal agenesis; seminal vesicle.

Figure 1

(A) US study showed a 36 mm × 16 mm right retrovesical fluid-filled cyst; (B) MRI showing the retrovesical SVC; (C) 3D MRI reconstruction; (D) postoperative US showing; the persistence of the 4 mm × 14 mm cyst.

Figure 2

(A) US showing the presence of a 10 mm × 50 mm unilocular dilatation of the seminal vesicle (white arrows); (B) MRI confirming the presence of the retrovesical cyst (white arrows); (C) laparoscopic view of the cystic lesion; (D) intraoperative view of the SVC (white arrow) and vas deferens (black arrow); (E) postoperative US showing the residual SVC; (F) MRI showing the sagittal view of the residual SVC.

Figure 3

(A,B) MRI showing the SVC (white arrows); (C) genitography performed using a 3Ch ureteral catheter inserted into the left ejaculatory duct contrasting the left SVC.