Practice Essentials
This article discusses some of the more common pediatric bladder anomalies, such as bladder diverticula, as well as some of the uncommon 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]
For many of these bladder anomalies, no overt physical findings are observed: They are elucidated after radiologic imaging for such things as a history of antenatal hydronephrosis or urinary tract infection (UTI). Imaging modalities employed in diagnosis of bladder abnormalities include voiding cystourethrography (VCUG), ultrasonography (US), intravenous pyelography (IVP), sinography, computed tomography (CT), magnetic resonance imaging (MRI), and cystography.
Patients with bladder diverticula generally require surgery when the diverticula are causing obstruction, recurrent UTIs, vesicoureteral reflux (VUR), or stone formation. No treatment is necessary for bladder ears. In megacystis, the massive VUR is corrected by ureteral reimplantation; reduction cystoplasty often is not necessary. Bladder agenesis is treated by means of urinary diversion and subsequent reconstruction. Incomplete bladder duplication often need not be treated; complete bladder duplication more often necessitates surgical correction. For bladder septation, depending on the anatomy, no treatment may be appropriate; surgical treatment is dictated by the individual patient's anatomy.
Urachal sinus can be observed in the first 4-8 weeks of life; surgical correction involves complete removal of the urachus. After adequate treatment of an infected urachal cyst, surgical removal of the entire urachus is warranted. Most patients with a vericourachal diverticulum require no treatment; excision is reserved for large symptomatic diverticula causing recurrent UTIs, stones, or poor emptying. A patent urachus can generally be observed in the first few months of life; persistence after 2 months warrants surgical correction. (See Treatment.)
Pathophysiology
The timing of altered embryogenesis leading to bladder anomalies has been a matter of speculation, and the embryologic cause of these lesions is unknown.
Bladder development occurs during weeks 5-7 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
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. In particular, megacystis, bladder duplication, and bladder septation are quite rare. [2, 3] For example, as of 2021, only about 70 cases of bladder duplication had been reported in the English literature. [4]
Bladder anomalies are generally diagnosed in infancy or childhood. With the advent of antenatal ultrasonography (US), such abnormalities are fully evaluated after birth. When they are not detected by antenatal screening, they are most commonly discovered in the evaluation of a UTI or, in the case of urachal anomalies, periumbilical drainage or redness. All of these anomalies occur in both males and females. The data are insufficient to establish any reasonably accurate frequency differences between the sexes.
Prognosis
The prognoses for the various pediatric bladder anomalies are as follows:
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Bladder diverticula - Excellent
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Urachal sinus - Excellent
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Urachal cyst - Excellent
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Patent urachus - Excellent
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Vesicourachal diverticulum - Excellent
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Bladder ears - Excellent
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Bladder agenesis - Guarded
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Megacystis - Good
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Bladder duplication - Good to excellent
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Bladder septation - Variable, ranging from guarded to good, depending on the anatomy and the degree of renal dysfunction
Most of these pediatric bladder anomalies have a low mortality and are associated with little morbidity.
Bladder diverticula and vesicourachal diverticula often cause no symptoms, but in symptomatic cases, patients most often present with UTI. 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 typically 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. [5] The risk of later adenocarcinoma appears to be low in children with asymptomatic lesions. [6]
Bladder agenesis generally is incompatible with life.
The morbidity of megacystis, bladder duplication, and bladder septation is generally related to associated abnormalities (when present), such as high-grade VUR or renal dysplasia. Megacystis may occur as part of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS; also referred to as Berdon syndrome), for which the prognosis is poor. [7]
Patient Education
In all of these conditions, an element of voiding dysfunction may exist. Parents should be educated regarding frequent and complete bladder emptying after the toilet training period to guard against infrequent and dysfunctional voiding patterns. In general, children should void approximately five or six times per day, which equates to voiding every 3 hours. Infrequent and dysfunctional voiding in the presence of an anatomic abnormality may place the child at increased risk for febrile UTIs and renal damage.
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Voiding cystourethrogram showing bladder diverticulum arising from posterior aspect of bladder.
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Voiding cystourethrogram showing two posteriorly placed bladder diverticula.
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Bladder sonogram showing two posteriorly placed bladder diverticula.
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Duplicated bladder with urethral catheter placed into each bladder.
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Voiding cystourethrogram demonstrating duplicated bladder.
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Intravenous pyelogram demonstrating duplicated bladder. Note how each ureter drains into ipsilateral bladder.
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Urachal cyst at level of umbilicus.
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Bladder anomalies. Sonogram demonstrating urachal cyst. Ultrasound cursors mark extent of cyst.
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Voiding cystourethrogram showing megacystis. Bilateral vesicoureteral reflux is also observed (grade 3 on right, grade 2 on left).
