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Pediatrics: Bladder Anomalies
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== Postnatally Detected Bladder Anomalies == === Urachal Anomalies === * In a series of 176 patients diagnosed with a urachal anomaly and urachal remnants, children mostly presented with umbilical drainage or on physical examination; of the adults, 66% had hematuria or pain. Surgical treatment in children consisted of simple excision, whereas over 50% of adults required partial or radical cystectomy because of malignancy. * Imaging possibilities include ultrasound, CT, and VCUG * Non-resolved urachal remnants should be excised because of the increased risk for later adenocarcinoma formation * '''Patent urachus (most common,''' 50%) ** '''Suspected in the neonatal period by continuous or intermittent drainage of fluid from the umbilicus''' ** '''[SASP] 50% of radiographically confirmed patent urachus will spontaneously close in the first 6 months of life and observation is warranted''' ** '''If required, treatment is complete excision of the patent urachus, including a bladder cuff''' * '''Umbilical-urachus sinus''' (15%) ** Manifestation is similar to that of the patent urachus * '''Urachal cyst''' (30%) ** There is no communication of the cyst with the bladder or umbilicus. ** However, the fluid-filled cyst can drain through the umbilicus or into the bladder intermittently. ** Urachal cysts are found more commonly in the distal part of the urachus and manifest more commonly in adults than in infants or children ** The cyst material consists of desquamated epithelial cells. These cells can become infected; '''staphylococcus aureus has been identified as the most common organism.''' Once infected, urachal cysts can manifest as umbilical abscess formation or bladder infections ** Treatment consists of antibiotics and draining the infected cyst, followed by complete excision of the urachal remnant structures. Conservative treatment with observation is justified in asymptomatic cases because of possible spontaneous resolution * '''Vesicourachal diverticulum''' (3%) === Bladder Diverticulum === * Can be detected on prenatal ultrasound, but the gold standard remains VCUG, which will reveal possible accompanying VUR. * Caused by infravesical obstruction, iatrogenic after bladder surgery, or as a congenital defect. Independent from the cause, all diverticula develop as herniation of bladder mucosa between defects of bladder smooth muscle fibers. The neck of the resulting diverticulum depends on the size of the muscular defect * Primary paraureteral diverticula are seen in smooth-walled bladders, occur isolated with no other diverticula, are intermittent in manifestation, happen in children with no infravesical obstruction, and are most likely caused by a congenitally deficient bladder wall * Secondary paraureteral diverticula are acquired, found in trabeculated bladders as one of many diverticula in the bladder, are always present, and are caused by infravesical obstruction. * '''Congenital diverticula are often found in children with generalized connective tissue diseases such as Ehlers-Danlos, Williams elfin-facies, or Menkes syndrome''' * '''Paraureteral diverticula or diverticula located in the lower part of the bladder can become so large that they compress the bladder neck or posterior urethra. The resulting bladder outlet obstruction starts a vicious circle by continuously filling and expanding the diverticulum. This increases the obstructing and subsequently causes complete urinary retention''' * '''Small, asymptomatic congenital diverticula detected during unrelated workups can be treated conservatively with regular observation.''' * '''In acquired bladder diverticula, the infravesical obstruction has to be eliminated first. After bladder outlet resistance is normalized, the bladder can reshape and diverticulectomy might become unnecessary.''' If symptomatic, especially in conjunction with VUR, the diverticulum should be excised. === Bladder Duplication === * Duplication of the bladder and urethra can be complete or incomplete * '''Can occur in either the coronal or sagittal plane; complete duplication in the sagittal plane the most common''' * Often associated with duplication anomalies of the external genitalia and lower GI tract * Initial treatment is directed toward renal preservation and prevention of infections by relieving possibly obstructed genitourinary tracts. * Long-term goals include achieving continence and reconstructing the internal and external genitalia.
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