Double trouble: diagnostic dilemma in a rare association of proximal ureteric valve with orthotopic ureterocele—case report

Abstract

Background Association of the ureterocele and proximal ureteral valve is a rare congenital anomaly which can lead to quick detoriation of renal function due to double obstruction. Though ureteral valves have been associated with other ureteral anomalies, this specific association is extremely rare. Case presentation A 7-month-old boy was referred to us for antenatally detected hydronephrosis. Provisional diagnosis of orthotopic ureterocele with pelviureteric junction (PUJ) obstruction was made based on preoperative imaging. Endoscopic incision of ureterocele was done and retrograde pyelography showed tight obstruction at PUJ which necessitated a pyeloplasty. Examination of resected specimen confirmed a ureteral valve. Postoperatively, the patient had good improvement in kidney function. Conclusion To conclude, congenital ureteral valves are a rare cause of ureteric obstruction and its association with ureterocele is rarer still. Identification of an ureterocele should not deter us from searching for an associated pathology like a ureteric valve when imaging shows more severe hydronephrosis suggestive of more proximal obstruction.

Congenital ureteral valve as a diagnostically challenging congenital ureteral defect

“…10) showed no pelvicalyceal or ureteral dilatation. in more than 50% of patients (3,4,6) . In the described cases, none of the patients had an additional urinary tract defect.…”

“…In the described cases, none of the patients had an additional urinary tract defect. Symptoms are non-specific, with abdominal and lumbar pain, as well as urinary tract infections being most common (3,4) . Diagnostic imaging (US of the urinary tract and CTU) is used as an auxiliary investigation (4) and sometimes allows to detect urinary flow obstruction.…”

“…The diagnostic criteria were proposed in 1952 by Wall and Wachter and include: • presence of transverse folds of the ureteric mucosa containing bundles of smooth muscle fibre; • no other evidence of mechanical or functional ob struction; • signs of obstructive disease above the valve with a normal ureter below it (2)(3)(4) . Another classification was proposed by Rabinowitz (3,4) : • type I -smooth muscle present within the folds; • type II -smooth muscle at the base only. Morphologically, three types of ureteral valves can be distinguished: cusp-like, diaphragmatic and annular type (3) .…”

“…It is believed that the valve may be a persistence of Chwalla's membrane or arise as a result of disturbances during embryogenesis (2,3,5) . Valve location in the ureter has been described as 50% in the proximal part, 17% in the middle third, and 33% in the distal third (3)(4)(5) . Cases of bilateral valve have also been reported (2) .…”

“…In the case of proximal location, pelvic ureteroplasty is performed, while ureteral transplantation is used for valves located at the bladder outlet. In the case of mid ureteric valve, the stenotic segment is excised with a simultaneous uretero-ureteral anastomosis (1,4,6) . In recent years, endoscopic treatment with the use of electrocoagulation or a laser has been gaining in importance (7) .…”