Congenital urinary bladder anomalies often occur without other genitourinary abnormalities. They may cause infection, retention, incontinence, and reflux. Symptomatic anomalies may require surgery.
A bladder diverticulum is a herniation of the bladder mucosa through a defect in bladder muscle. It predisposes to urinary tract infections (UTIs) Urinary Tract Infection (UTI) in Children Urinary tract infection (UTI) is defined by ≥ 5 × 104 colonies/mL in a catheterized urine specimen or, in older children, by repeated voided specimens with ≥ 105 colonies/mL... read more and may coexist with vesicoureteral reflux Vesicoureteral Reflux (VUR) Vesicoureteral reflux is retrograde passage of urine from the bladder back into the ureter and sometimes also into the renal collecting system, depending on severity. Reflux predisposes to urinary... read more . It is usually discovered during evaluation for fetal hydronephrosis or recurrent UTIs in young children.
Diagnosis of bladder diverticulum is by voiding cystourethrography.
Surgical removal of the diverticulum and reconstruction of the bladder wall may be necessary.
In exstrophy, there is a failure of midline closure from the umbilicus to the perineum, resulting in bladder mucosa continuity with the abdominal skin, separation of the pubic symphysis, and epispadias Epispadias Congenital anomalies of the urethra in boys usually involve anatomic abnormalities of the penis and vice versa. In girls, urethral anomalies may exist without other external genital abnormalities... read more or bifid genitalia. The bladder is open suprapubically, and urine drips from the open bladder rather than through the urethra. Despite the seriousness of the deformity, normal renal function usually is maintained.
The bladder can usually be reconstructed and returned to the pelvis, although vesicoureteral reflux invariably occurs and is managed as needed. Additional surgical intervention may be necessary to treat a bladder reservoir that fails to expand sufficiently or has sphincter insufficiency. Reconstruction of the genitals is required and may be done in the immediate postnatal period or may be delayed.
In this syndrome, a large, thin-walled, smooth bladder without evident outlet obstruction develops, usually in girls. Vesicoureteral reflux Vesicoureteral Reflux (VUR) Vesicoureteral reflux is retrograde passage of urine from the bladder back into the ureter and sometimes also into the renal collecting system, depending on severity. Reflux predisposes to urinary... read more is common.
Megacystis syndrome is poorly understood. The syndrome may be a manifestation of a primary myoneural defect, especially when intestinal obstruction (eg, megacystis-microcolon, intestinal hypoperistalsis syndrome) is also present.
Symptoms are related to urinary tract infections (UTIs) Urinary Tract Infection (UTI) in Children Urinary tract infection (UTI) is defined by ≥ 5 × 104 colonies/mL in a catheterized urine specimen or, in older children, by repeated voided specimens with ≥ 105 colonies/mL... read more .
Ultrasonography with the bladder empty may disclose normal-appearing upper tracts, but voiding cystourethrography may show reflux with massive upper tract dilation.
Ureteral reimplantation may be effective, although some patients benefit from antibacterial prophylaxis, timed voiding with behavioral modification, intermittent catheterization, or a combination.
Neurogenic bladder Neurogenic Bladder Neurogenic bladder is bladder dysfunction (flaccid or spastic) caused by neurologic damage. Symptoms can include overflow incontinence, frequency, urgency, urge incontinence, and retention.... read more is bladder dysfunction caused by neurologic disorders, including spinal cord or central nervous system abnormalities, trauma, or the sequelae of pelvic surgery (eg, for sacrococcygeal teratoma or imperforate anus Anal Atresia Anal atresia is an imperforate anus. (Also see Overview of Congenital Gastrointestinal Anomalies.) In anal atresia, the tissue closing the anus may be several centimeters thick or just a thin... read more ).
The bladder may be flaccid, spastic, or a combination. A flaccid bladder has high-volume, low-pressure, and minimal contractions. A spastic bladder has normal or low-volume, high-pressure, and involuntary contractions. When present, chronically elevated bladder pressure (> 40 cm H2O) often causes progressive kidney damage, even without infection or reflux.
Manifestations include recurrent UTIs Urinary Tract Infection (UTI) in Children Urinary tract infection (UTI) is defined by ≥ 5 × 104 colonies/mL in a catheterized urine specimen or, in older children, by repeated voided specimens with ≥ 105 colonies/mL... read more , urinary retention and/or incontinence Urinary Incontinence in Children Urinary incontinence is defined as involuntary voiding of urine ≥ 2 times/month during the day or night; the incontinence may be intermittent or continuous. Revised terminology for the time... read more , and potentially renal insufficiency.
The underlying neurologic abnormality is usually readily apparent. Usually, postvoiding residual volume is measured, renal ultrasonography is done to detect hydronephrosis, and serum creatinine is measured to assess renal function. Urodynamic testing is often done to confirm diagnosis and to monitor bladder pressures and function. These studies are often repeated at scheduled intervals as the child grows to assess for deterioration of bladder and renal function.
Management goals include lowering risk of infection, maintaining adequate bladder storage pressure and volume, effective bladder emptying, and achieving social continence. Treatment of neurogenic bladder includes drugs (eg, anticholinergics, prophylactic antibiotics), intermittent catheterization, and/or surgical intervention (eg, augmentation cystoplasty, appendicovesicostomy, botulinum toxin injections, neurostimulation). Children with neurogenic bladder often also have a neurogenic bowel with constipation Constipation in Children Constipation is responsible for up to 5% of pediatric office visits. It is defined as delay or difficulty in defecation. Normal frequency and consistency of stool varies with children's age... read more and stool incontinence Stool Incontinence in Children Stool incontinence is the voluntary or involuntary passage of stool in inappropriate places in children > 4 years of age (or developmental equivalent) who do not have an organic defect or... read more that also require proper management.