Delayed Presentation of Urethral Valves: A Diagnosis That Should Be Suspected Despite a Normal Prenatal Ultrasound

Background. Congenital urethral obstruction occurs most frequently as a result of urethral valves. The diagnosis is usually confirmed pre- or neonatally. Though not ideal, delayed diagnosis can occur in childhood, adolescence, or adulthood. Despite a normal prenatal ultrasound, there may still be a delayed diagnosis of urethral valves. Methods. We present 4 patients with delayed diagnosis of posterior urethral valves (PUV) and 1 patient with delayed diagnosis of anterior urethral valves (AUV) who were evaluated by a pediatric urologist at our Institution. We determined the age and symptoms at presentation, physical examination, micturating cystourethrogram (MCU) findings prior to the valve ablation, renal function before and after valve ablation, type of valve ablation, and urine culture and sensitivity. The urological courses following urethral valve ablation including urodynamic study findings are described. Results. The median age at presentation was 33 months. All 5 patients presented with decreased urine output and urinary retention. All 5 patients underwent a MCU that demonstrated bladder trabeculations (3 cases), vesicoureteral reflux (3 cases), and bladder diverticula (2 cases). A urethral valve ablation was performed in all cases. Four patients underwent a renal function panel prior to this procedure, and their serum BUN/creatinine levels decreased 1 day postoperatively. Conclusion. Pediatricians should consider urethral valves as causing urethral obstruction although the prenatal ultrasound may be normal. Early diagnosis and prompt treatment of urethral valves may mitigate the potentially devastating morbidities such as renal failure, congestive heart failure, and respiratory distress that may ensue.

Once in a Blue Moon, Congenital Anterior Urethral Diverticulum

Anterior urethral valves (AUVs) are rare congenital anomalies causing lower urinary tract obstruction in children. Although they are referred to as valves, these obstructive structures often occur in the form of a diverticulum. Congenital anterior urethral diverticulum (CAUD) is an uncommon/rare entity in children which can lead to obstructive lower urinary tract symptoms and urosepsis. Over the past 20 years, more than 260 cases have been reported. However, most cases are diagnosed on prenatal ultrasonography (USG) in early life if patients present with bilateral hydronephrosis and/or azotemia. Diagnosis is by ureteroscopy and radiological imaging. Surgical treatment can be open or endoscopic. Here, we report a new case of CAUD and discuss its clinical presentation, diagnosis, and management. A brief review of the literature is also presented.

Disorders of the urethra

Most disorders of the urethra in children are congenital in origin and affect boys more commonly than girls. They include; posterior urethral valves (PUV), anterior urethral valves, anterior urethral diverticulum, syringocele, urethral atresia, megalourethra, urethral web, urethral polyp, and urethral duplication. Urethral strictures may be congenital or acquired. Most cases of PUV are now diagnosed prenatally. Postnatal management comprises bladder drainage, correction of any metabolic disturbance, prevention of infection (UTI), and endoscopic valve ablation. Careful follow up is required with the aim of preventing urosepsis and preserving renal function. Persisting bladder dysfunction (‘valve’ bladder) can threaten renal function and should be managed aggressively. Chronic renal failure ultimately affects a third of boys with PUV, of whom 10–20% require renal transplantation during the course of childhood. PUV may also present clinically with recurrent UTI, urinary incontinence, or unexplained lower urinary tract symptoms.

Anterior Urethral Valve: Uncommon Association with Renal Duplicity

Anterior urethral valves (AUVs) is an unusual cause of congenital obstruction of the male urethra, being 15–30 times less common than posterior urethral valves. We present a case of AUV diagnosed at 24th gestational week. Ultrasonography and fetal MRI revealed hydronephrotic kidneys with ureteral duplicity, a distended bladder and perineal cystic mass which confirmed dilated anterior urethra in a male fetus. Diagnosis was confirmed postnatally by voiding cystourethrogram and surgery.