CONGENITAL MALFORMATIONS OF THE LOWER URINARY TRACT

PROF. D. A. NZEHDEPARTMENT OF RADIOLOGY

UNIVERSITY OF ILORIN

CONGENITAL ANOMALIES OF THE URINARY BLADDER-1

BLADDER EXTROPHY Characterized by absence of the anterior bladder wall. Has a male to female ratio of 3:1. Diagnosis can be made solely by clinical observation. Plain radiograph shows wide separation (diastasis) of

the pubic bones. Symphysis pubis is separated by a gap as wide as the

sacrum. May be associated with epispadias and cloacal

anomalies. IVU is useful to examine the upper renal tract b/c

ureteric obstruction is a known association. The dilated ureters sometimes give a classical “hurley-

stick” (base ball stick) appearance. Differential diagnosis is epispadias which also gives

pubic symphysis diastasis in some cases.

CONGENITAL ANOMALIES OF THE URINARY BLADDER-2

BLADDER DUPLICATION A rare congenital anomaly. When complete, each half of the bladder has its own

normal muscle, ipsilateral ureter and urethra. When duplication is incomplete, the divided bladder

components drain via a single urethra. Septate bladders also drain through a single urethra. Diagnosis is made by cystography.BLADDER AGENESIS Rare and incompatible with life. BLADDER EARS Results from lateral protrusions of the bladder due to

extraperitoneal herniations through the inguinal ring into the inguinal canal.

Associated with a high incidence of inguinal hernia and usually seen in infants.

It is not a true congenital anomaly but a hernial deformity.

Radiological diagnosis is by cystography and IVU.

CONGENITAL ANOMALIES OF THE URINARY BLADDER-3

PRUNE-BELLY SYNDROME (EAGLE-BARRATT SYNDROME)

Consists of absence of anterior abdominal wall (recti) muscles; congenital enlargement of the bladder; hydroureter ; urinary tract dysplasia and undescended testes.

Almost always occurs in males. Has a poor prognosis. Bladder wall is thickened. The trigone is enlarged and this leads

to vesico-ureteric reflux.

CONGENITAL ANOMALIES OF THE URACHUS

PATENT URACHUS Due to persistent communication b/w the bladder and the

umbilicus. Diagnosed clinically by observing a continuously wet

umbilicus in an infant.URACHAL SINUS Due to patency at the umbilical end of the urachus (blind

external type). May be undetected until they become infected and produce

clinical features like fever and sepsis. Radiological diagnosis is by sinography.URACHAL DIVERTICULUM Due to patency at the vesical end of the urachus (blind

internal type). Radiological diagnosis is by MCU and US.URACHAL CYST Due to obstruction of a patent urachus at both ends. Ultrasound helps to outline the cyst.

CONGENITAL ANOMALIES OF THE URETHRA-1

POSTERIOR URETHRAL VALVES The valves are found almost always in males. Valves are located around the verumontanum. Diagnosed by MCU Valves may be unicuspid, bicuspid or membrane-like. MCU shows a dilated posterior urethra with poor stream of urine in

the anterior urethra. The valves may not be visible on MCU but valve stretches like a sail

to obstruct the urethra.

ANTERIOR URETHRAL VALVES Rare congenital anomaly. When present, it produces obstruction with proximal dilatation similar

to that of the posterior urethral valves. The radiolucent defect of the valve may be demonstrated on MCU.

HYPOSPADIAS (PENILE OR PERINEAL) Due to abnormal opening of the external urethral meatus. May be associated with PUJ obstruction. Repair may result in stricture formation which is best demonstrated

on MCU.

CONGENITAL ANOMALIES OF THE URETHRA-2

URETHRAL DIVERTICULUM Can occur in males or females. In females, all diverticula are congenital. In males, most diverticula are acquired after trauma or

infection although some are congenital. Diverticula may be demonstrated by RUCG in males. In females, a diverticulum is best demonstrated by

MCU due to difficulties encountered with RUCG. URETHRAL DUPLICATION Rare congenital anomaly. Usually partial in nature (i.e. incomplete). Stasis of urine predisposes to infection.

CONGENITAL ANOMALIES OF THE URETHRA-3

URETHRAL STENOSIS Cause of narrowing of the urethra. Diagnosed by RUCG.URETHRAL ATRESIA Incompatible with life. Results in fetal death during the fourth month

of gestation when production of urine begins. Fetus may survive if there is a fistulous

connection with the urachus to the bladder or with the rectum.

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