Megaureter Megaureter is not a diagnosis but a descriptive term for a dilated ureter.

Normal ureteral diameter in children is rarely greater than 5 mm. Ureters greater than 7 mm can be considered megaureters.73 The ultrasonographic appearance of the dilated and tortuous ureter is usually striking (Fig. 55-8). Pelvicalyceal dilation and parenchymal scarring or thinning depend on the primary disease process. These ureters can be classified as refluxing, obstructed, and nonrefluxing, nonobstructed.74 Some ureters also have reflux and simultaneous obstruction. 75 Table 55-1 gives clinical examples of each classification. Any normal ureter will dilate if the volume of urine exceeds emptying capacity. Moreover, bacterial endotoxins and infection alone can cause dilation that will resolve after treatment of the infection.76,77 Primary obstructive megaureter is most commonly caused by a distal adynamic ureteral segment, but ureteral valves33 and ectopic ureteral insertion also cause obstruction. Proximal smooth muscle hypertrophy and hyperplasia are seen. A normal-caliber catheter will usually pass through the distal 3- to 4-mm segment, but the peristaltic wave does not propel urine across this area. This absent peristalsis is not a result of a ganglionic abnormality as seen in megacolon.78 The distal ureter has a variety of histologic appearances, but the common finding is a disruption of muscular continuity that prevents muscular propulsion of urine.14,79-81 As with UPJ obstruction, the majority of megaureters are now detected prenatally, although infection may also be a presentation for refluxing and obstructed megauters.82-84 Megaureter is now the second most common urinary tract abnormality detected prenatally. 2 These children are typically asymptomatic without any physical findings or laboratory abnormalities. Despite the variety of possibilities, standard imaging allows classification and appropriate management. The diagnosis of a nonobstructed, nonrefluxing megaureter is the hardest to make and is established only when the secondary causes of megaureter have been excluded and diagnostic tests do not show obstruction. For years it was assumed that a dilated ureter that did not reflux was obstructed,85 but developmental ureteral dilation can occur in ureters that are not obstructed.86 Diagnostic imaging begins with an ultrasound study,

which almost always distinguishes megaureters from UPJ obstruction. The degree of distal ureteral dilation is often much more pronounced than the degree of renal pelvic dilation or caliectasis. A VCUG should be obtained in all patients. If significant reflux is found, delayed drainage films must be obtained to exclude simultaneous obstruction with a normal-caliber distal ureteral segment. In a partially obstructed system, the contrast density in the ureter is decreased because of dilution related to stasis in the ureter (see Fig. 55-8). Diuretic renography is used to assess function and drainage. The markedly dilated ureters can be a significant source of stasis, and determination of drainage half-time can be difficult. Diuretic administration must be delayed because the system is so capacious and may take 60 to 90 minutes to fill. A washout time of longer than 20 minutes is historically indicative of obstruction. Treatment Nonoperative management is based on clearance halftime and relative renal function of the hydronephrotic and contralateral kidneys. If observation is chosen, the children are given preventive antibiotics and followed with serial ultrasound and renal scans. Neonatal megaureter with obstruction suggested by renography but with preserved function can be safely observed. Most ureters will become radiographically normal over time.82,83,87,88 Surgical correction for decreasing function or recurrent infections will be needed in only 10% to 25% of patients at age 7 years. Evidence of delayed obstruction after normalization of radiographs has not been seen in these children. The initial attempts at surgical repair resulted in significant reflux and recurrent infections. Now, it can be performed with a high expectation of success and low morbidity.89 Ureteral excisional tapering with preservation of the ureteral blood supply was popularized in the early 1970s.85,90 A lngitudinal segment of ureter is excised and then closed over a 10- to 12-Fr catheter. When the ureter is tunneled submucosally, the suture line is positioned against the detrusor to decrease the chance of fistula formation. Initial repairs involved tailoring the entire ureter, but this was found to be unnecessary because the upper ureteral tortuosity and dilatation often disappears after tapering

the distal ureter alone.91 Ureteral folding techniques have been popularized because they theoretically decrease the risk of ischemic injury while achieving the decreased intraluminal diameter necessary for a successful reimplant.92,93 The increased bulk is usually not a technical problem. Although dissection is usually both intravesical and extravesical, solely extravesical reimplants have been described and may be associated with lower morbidity.94 A vesicopsoas hitch is a useful adjunct that helps achieve a longer submucosal tunnel length without risking ureteral kinking, although excisional tailoring usually eliminates the need for this adjunctive procedure. A nonrefluxing, nonobstructed reimplantation can be achieved in 85% to 95% of patients with megaureters.84,93 Recognized complications include persistent obstruction, reflux, and urinary extravasation. Most of these can be managed nonoperatively with drainage tubes. Lower grades of postoperative VUR will often resolve. Primary reconstruction is preferred when indicated, but temporary cutaneous diversion may be beneficial in a neonate or infant in whom the chance of successful reimplantation of a bulky ureter into a small bladder is diminished. Diversion may decrease the ureteral diameter and decrease the need for tailoring at the time of reimplantation. An end-cutaneous ureterostomy is preferred because a high diversion may require two or more procedures for correction.