Screening for Zinner Syndrome in Patients With a Congenitally Solitary Kidney: Lessons Learned

Abstract

Purpose:We determined if serial screening ultrasounds are beneficial in evaluating for the development of Zinner syndrome in males with a congenital solitary kidney.Materials and Methods:All patients included had their congenital solitary kidney diagnosed at <20 years of age and had to be ≥20 at their last visit. Individuals were seen annually, with pelvic ultrasounds to screen for mesonephric duct cysts obtained at birth and every year of age, divisible by 5.Results:At a median follow-up of 38 years of age (range 20-57), 17% (20/121) developed Zinner syndrome, with 60% (12/20) developing clinical symptoms. The yield for screening ultrasound studies was significantly higher in patients ≤20 years of age at 3.5% (12/340), compared to 0.33% (1/296) in patients >20 years of age (P = .004). Serial ultrasounds reveal the onset of lower urinary tract and cyst-related pain symptoms are associated with the growth of the seminal vesicle cyst to ≥5 cm (P = .0198). Of symptomatic patients, 75% (8/12) had abnormal uroflows. Complete urodynamic studies revealed findings consistent with bladder outlet obstruction in 38% (3/8), equivocal for obstruction in 24% (2/8), and detrusor underactivity in 38% (3/8). Cyst excision effectively resolved voiding symptoms that were obstructive in etiology but failed to resolve symptoms in patients with detrusor underactivity.Conclusions:Serial ultrasound evaluations reveal that cyst growth to ≥5 cm in size is highly related to the onset of clinical symptoms, with the resolution of voiding symptoms by cyst excision directly associated with urodynamic findings.

A Rare Variant of Zinner Syndrome Involving Ectopic Ureteral Implantation into the Seminal Vesicle Causing Recurrent Epididymitis

“…There are no agreed-upon guidelines for routine screening of conditions related to Zinner syndrome. Although overall rare, the incidence of Zinner syndrome in patients with congenital solitary kidneys is approximately 10-12%, making it reasonable to routinely screen patients in this setting [ 12 ]. In a large, single-center study of patients with congenital solitary kidneys, the authors recommended routine pelvic ultrasound between 1 and 3 months of age and at every year of age divisible by 5, as well as annual follow-ups to review medical history, measure serum creatinine, obtain a urinalysis for proteinuria, and to perform a physical examination [ 12 ].…”

“…Although overall rare, the incidence of Zinner syndrome in patients with congenital solitary kidneys is approximately 10-12%, making it reasonable to routinely screen patients in this setting [ 12 ]. In a large, single-center study of patients with congenital solitary kidneys, the authors recommended routine pelvic ultrasound between 1 and 3 months of age and at every year of age divisible by 5, as well as annual follow-ups to review medical history, measure serum creatinine, obtain a urinalysis for proteinuria, and to perform a physical examination [ 12 ]. Lower urinary tract symptoms related to Zinner syndrome are thought to be directly associated with cystic accumulation and progressive cystic growth, and prompt diagnoses and treatment of cystic burden showed good prognosis in 75% of cases [ 12 ].…”

Diseases and syndromes associated with unilateral renal agenesis in children

“…Синдром Циннера -редкая врождённая аномалия развития, характеризующаяся триадой признаков: наличием кисты семенного пузырька, ипсилатеральной аплазией почки или АгП и обструкцией семявыносящего протока. Эта аномалия протекает, как правило, бессимптомно и приводит к грозному осложнению -мужскому бесплодию [7,[38][39][40].…”