Recurrent urinary tract infections in a child with ambiguous genitalia

Ambiguous genitalia is described as the external genitalia that do not have the typical anatomic appearance of either a boy or a girl. after providing clinical and metabolic stability in these children, distinct problems such as recurrent urinary tract infections can also be a cause of irritability. here, a 22-month-old infant with the diagnosis of xlag syndrome (x-linked lissencephaly and ambiguous genitalia, with 46xy genotype) who suffered from recurrent urinary tract infections due to many different microorganisms including bacteria and fungi, is presented. previous radiologic evaluations of the patient, when he was 10-month-old, such as urinary ultrasonography (us), voiding cystourethrogram (vcug), and 99mtc-dmsa renal scintigraphy were reported as normal. however, when previous vcug examination was re-evaluated, entire urethra could not be seen in the images. a new vcug examination which performed as retrograde urethrography showed prostatic utricle as a cause of recurrent urinary tract infections. although vcug is sufficient for the evaluation of the anatomy and abnormalities of the bladder and urethra, a detailed imaging of the urethra is usually considered less than the bladder. we want to emphasize that this case is a reminder in depicting the importance of urethral anomalies which can be a cause of recurrent urinary tract infections in children with ambiguous genitalia.