Background
This article discusses some of the more common bladder anomalies, such as bladder diverticula, and some of the uncommon bladder anomalies, such as bladder ears, congenital hypoplasia of the bladder, megacystis, bladder agenesis, duplication anomalies of the bladder, and finally, bladder septa. A discussion of urachal anomalies (ie, urachal sinus, urachal cyst, urachal diverticulum, patent urachus) is also included.[1, 2]
Pathophysiology
The timing of altered embryogenesis leading to these conditions has been a matter of speculation, and the embryologic cause of these lesions is unknown. Bladder development occurs during the fifth to seventh week of gestational development. Development depends upon many factors, such as proper mesenchymal differentiation, mesenchymal growth, urine production that stimulates bladder expansion, and detrusor contraction. Bladder cycling, the process of sequential expansion and contraction, is important in the anatomic and physiologic development of the normal bladder. Bladder ears are considered a normal variation, not a congenital anomaly.
Epidemiology
Frequency
United States
Bladder diverticula are uncommon, but not rare. In a series of over 5000 children studied, the approximate incidence was 1.7%. All of the other entities are uncommon or rare.
Mortality/Morbidity
Most of these conditions have a low mortality rate and little morbidity.
- Bladder diverticula and vesicourachal diverticula often cause no symptoms, but in symptomatic cases, patients most often present with urinary tract infection. Rarely, bladder diverticula and vesicourachal diverticula may cause bladder outlet obstruction. Death from peritonitis due to a ruptured infected urachal cyst has been reported in a few cases, but most bladder anomalies are incidental findings, and patients are asymptomatic.
- Patent urachal anomalies do not usually cause significant morbidity or mortality. However, adenocarcinoma has been reported in adults in urachal remnants, presumably from chronic inflammation and infection.
- Bladder agenesis generally is incompatible with life.
- Megacystitis, bladder duplication, and bladder septation are rare. Morbidity of these conditions is generally related to associated abnormalities (when present) such as high-grade vesicoureteral reflux or renal dysplasia.
- Only 50 reported cases of bladder duplication exist.[3, 4]
- Bladder septations are equally rare.
Sex
All of these anomalies are infrequent or rare, and each condition occurs in both males and females. Data are insufficient to provide reasonably accurate frequency differences in the sexes.
Age
Bladder anomalies are generally diagnosed in infancy or childhood. With the advent of prenatal ultrasonography, such abnormalities are fully evaluated after birth. When not detected by antenatal screening, most are discovered in the evaluation of a urinary tract infection or, in the case of urachal anomalies, periumbilical drainage or redness.
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