Congenital Mid Ureteric Valve Stenosis Revisited: Case Report and Review of the Literature

Abstract

Congenital mid ureteric valve (MUV) stenosis is a very rare entity. Definitive preoperative diagnosis is clinically challenging, and most patients are misdiagnosed preoperatively. Intraoperative identification is therefore very important. Curative treatment consists of excision of the involved ureteric segment and anastomosis. This report describes the clinical findings in a patient with congenital mid ureteric valve stenosis, including radiological and histological workup and operative management. Routine intraoperative retrograde pyelography is important in the diagnosis of such rare pathologies.

Bilateral aniridia and congenital ureteral valve: Role of genetic testing

“…Ureteral valves represent a unique and exceedingly rare finding (Elifranji et al, 2019;Rossi et al, 2007). Ureteral valves are often accompanied by other genitourinary abnormalities such as renal duplication, ectopic ureter, VUR, pelviureteric junction obstruction, renal agenesis, and horseshoe kidney (Rossi et al, 2007;Sant et al, 1985).…”

“…Hydronephrosis is frequently observed on prenatal US with ureteral valves, and an intravenous and retrograde pyelogram confirms the diagnosis (Rossi et al, 2007). Surgical excision of the ureteric segment containing the valve followed by ureteral anastomosis is often performed (Elifranji et al, 2019;Rossi et al, 2007). The differential diagnoses include ureteral stricture due a previous ureteral insult (infection or trauma) or primary megaureter that both cause ureteral dilatation (Elifranji et al, 2019).…”

“…Surgical excision of the ureteric segment containing the valve followed by ureteral anastomosis is often performed (Elifranji et al, 2019;Rossi et al, 2007). The differential diagnoses include ureteral stricture due a previous ureteral insult (infection or trauma) or primary megaureter that both cause ureteral dilatation (Elifranji et al, 2019).…”

“…Ureteral valves are exceedingly rare causes of obstructive uropathy that are usually diagnosed at surgery or at fetal or newborn autopsies (Rossi, Salas, Aucatoma, Munoz, & Fochs, 2007;Sant, Barbalias, & Klauber, 1985). Symptoms associated with ureteral valves include urinary tract obstruction often caused by hydronephrosis, urinary tract infection, hematuria, a flank mass, or flank pain (Elifranji, Elkadahi, Charles, & Abbas, 2019;Reinberg, Aliabadi, Johnson, & Gonzalez, 1987;Rossi et al, 2007). Wall and Watcher established three criteria to confirm ureteral valves: (a) Anatomically demonstrable transverse folds of transitional epithelium containing smooth muscle bundles; (b) Secondary obstructive changes above the ureteral valve with a normal ureter distally; and (c) No other mechanical or functional obstruction (Wall & Wachter, 1952).…”