Could Drainage Related Ultrasonography Prevent Unnecessary Interventions in Patients with Wolfram Syndrome?

Abstract

Background: Upper urinary tract dilation, the most common urological manifestation of the Wolfram syndrome (WS), is mainly non-obstructive and secondary to other components of the disease such as diabetes insipidus. Misdiagnosis of the type of the hydroureter in Wolfram patients and encountering them as obstructive uropathies has led to ineffectual surgeries such as ureter re-implantation. Based on previous studies drainage related ultrasonography (DRUS) is a beneficial means of distinction between obstructive and non-obstructive hydroureters. Objectives: To avoid unnecessary interventions in patients with WS by detecting hydroureters’ types using DRUS. Methods: Seven patients (14 ureters) with a mean ± SD age of 24.43 ± 4.25 months who were diagnosed with WS were included in this retrospective study. The definite diagnosis of the non-obstructive type of hydroureter was assessed by appropriate imaging modalities. The maximum diameter of these 14 ureters, before (D1) and after (D2) 3 hours of catheterization were observed by ultrasonography. Values were recorded as D ratio ( [(|D1 - D2|)/D1] × 100) and the cutoff point of 22% for D ratio was set to discriminate the subtypes of the hydroureter. Results: Measurement of maximum diameter of ureter prior to catheterization indicated a mean ± SD diameter of 20.64 ± 2.73 mm; decreasing to 11.07 ± 2.64 mm after 3 hours of catheterization which indicates a significant decrease. Mean D ratio of 14 hydroureters was 45.95 ± 13.01% which indicated significantly higher percentage than 22%, revealing that hydroureters’ type in WS is non-obstructive. Conclusions: DRUS is a useful method for the assessment of the hydroureter’s type in WS and it could prevent performing unnecessary surgeries in WS patients.