Congenital megalourethra: outcome after prenatal diagnosis in a series of 4 cases

Abstract

Objective: The purpose of this series is to describe the prenatal sonographic findings and pregnancy outcome in 4 cases of congenital megalourethra.

Methods: Three cases of fusiform-type megalourethra and 1 case of scaphoid-type megalourethtra were diagnosed prenatally by sonography between 20 and 24 weeks' gestation. The key findings for diagnosis included sonographic features of lower urinary tract obstruction in association with dilatation and elongation of the penile urethra in all 4 cases. Severe oligohydramnios was detected at the time of diagnosis in only 1 case. Prenatal chromosome analysis was available in 3 cases and revealed a normal 46,XY karyotype. Information on pregnancy outcome was available in all cases.

Results: The parents opted for elective termination of pregnancy in 1 case when decreased amniotic fluid volume and echogenic kidneys were documented at the follow-up scan. Among the 3 ongoing pregnancies, there were 2 premature cesarean deliveries at 28 and 31 weeks and 1 vaginal delivery at term. All neonates had severe lung hypoplasia and died in the early neonatal period.

Conclusions: Congenital megalourethra is associated with poor perinatal outcome. Because this condition can be diagnosed prenatally in the context of lower urinary tract obstruction, detailed examination of the external genitalia in these cases should be mandatory.