Megaureter and Other Congenital Ureteral Anomalies 

Developmental abnormalities of the ureter encompass a wide range of disorders. Ureteral anomalies are a common cause of morbidity in children and frequently require surgical intervention.

Interestingly, Leonardo da Vinci and Galen were the first to begin to recognize the importance of the ureterovesical junction (UVJ) and to identify vesicoureteral reflux (VUR). Semblinow's 1883 animal experiments renewed enthusiasm for the study of reflux and began the modern era of research to clarify the anatomy, function, and pathophysiology of UVJ anomalies.

Duplex (duplicated) system

The duplex system is kidney with 2 ureters. A patient with a duplication anomaly may have bifid ureters (partial or incomplete duplication) or 2 ureters that empty separately into the bladder (complete duplication). The upper ureter is more likely to be associated with ectopic insertion, ureterocele, and/or obstruction, whereas the lower ureter is more frequently associated with VUR.

The upper pole is one of the components of the duplex kidney. The upper pole ureter drains the upper pole of a duplex kidney. Similarly, the lower pole of the kidney is drained by the lower-pole ureter.

Caudal or medial ectopia describes the ureteral orifice when located at the proximal lip of the bladder neck or more distal.

Ectopic ureter

Ectopic ureter occurs when the ureter drains to an abnormally located (ectopic) orifice.

Bilateral single-system ureteral ectopia is rare and usually coexists with a multitude of other urinary tract abnormalities (eg, VUR, renal dysplasia, rudimentary bladder development).

Megaureter

A megaureter is a wide ureter, greater than 7 mm in diameter.

Megaureters may be classified into 4 categories: obstructed, refluxing, obstructed and refluxing, and nonobstructed/nonrefluxing.

Ureterocele

A ureterocele is a congenital saccular dilatation of the distal segment of the ureter.

This anomaly most frequently involves the upper pole of a duplex system. If the ureterocele extends beyond the bladder into the urethra, it is considered an ectopic ureterocele; whereas a ureterocele contained within the bladder is considered an orthotopic ureterocele.

Ureteroceles are often associated with obstruction and VUR.

Vesicoureteral reflux

VUR is retrograde passage of urine from the bladder into the ureter and/or kidney. Reflux-induced renal injury is usually caused by the association of VUR with urinary tract infection (UTI).

Reflux-induced renal injury was previously thought to occur primarily in children younger than 2 years. However, the risk of renal injury from pyelonephritis associated with VUR may occur in individuals well beyond this age.

Reflux may also be associated with regions of renal dysplasia or hypoplasia in the absence of UTI and is thought to be secondary to abnormal development.

Reflux that is secondary to high bladder pressures such as those occurring in patients with posterior urethral valves (PUV) or bladder outlet obstruction (BOO) is frequently associated with renal injury.

Reflux-induced renal injury may range from clinically silent focal scars to generalized scarring and renal atrophy (reflux nephropathy), which may lead to morbidity during pregnancy, renin-mediated hypertension, renal insufficiency, and even end-stage renal disease.

Frequency

Ureteral duplication

• Ureteral duplication is the most common anomaly of the urinary tract.
• Incidence is as high as 8% in children being evaluated for UTI.
• Incomplete ureteral duplication is observed in approximately 1 in 25 individuals.
• Complete duplication is present in approximately 1 in 125 individuals.
• Complete duplication on one side results in a 40% chance of a complete duplication abnormality on the other side.
• Approximately 10% of siblings may also be affected by complete duplication.

Ectopic ureters

• Ectopic ureters occur in 0.025% of the population.
• Approximately 10% are bilateral.
• Ectopic ureters occur more frequently in females than in males (by a ratio of 6:1).
• In females, more than 80% of ectopic ureters drain duplicated systems. In males, most ectopic ureters drain a single system.
• Approximately 80% of all ectopic ureters drain the upper pole of a duplex kidney.
• In males, the ureters always terminate proximal to the external sphincter and may be found at the bladder neck/prostatic urethra (48%), seminal vesicle (40%), ejaculatory duct (8%), vas deferens (3%), or epididymis (0.5%).
• In females, the ureters may terminate at the bladder neck/urethra (35%), vestibule (30%), vagina (25%), or uterus (5%).

Megaureters

• Bilateral involvement is present in about 20% of patients with primary obstructed megaureters.
• Primary obstructed megaureter has a male-to-female ratio of nearly 4:1.
• The left side is more often affected than the right.

Ureterocele

• Incidence is approximately 1 in 4000 children.
• Approximately 80% occur in females.
• Approximately 10% are bilateral.
• Approximately 80% are associated with duplicated collecting systems with single system ureteroceles being more common in males and adults.

Vesicoureteral reflux

• Incidence in otherwise healthy children is approximately 1%.
• The incidence is approximately 40% in patients undergoing evaluation for UTI.
• The reported risk of reflux in a sibling is 27-43%.
• Approximately 50% of the offspring of women with reflux also have VUR.

A ureteral bud, the early precursor of the ureter, branches off from the caudal portion of the wolffian (mesonephric) duct between the fourth and sixth week of gestation. The cranial portion of the ureteral bud joins with the metanephric blastema and begins to induce nephron formation. The bud subsequently branches into the renal pelvis and the calyces and induces nephron formation. Caudally, the mesonephric duct (along with the ureteral bud) is incorporated into the cloaca as it forms the bladder trigone. Alterations in bud number, position, or time of development result in ureteral anomalies.

Early branching of a single ureteral bud results in incomplete (partial) duplication, with a single ureteral orifice and bifid proximal ureters. An accessory ureteral bud creates complete duplication, with the upper ureter usually protruding into the bladder more medially and inferiorly than the lower ureter. Ectopic termination of a single system or of the ureter of a duplex system is the result of the high (cranial) origin of the ureteral bud from the mesonephric duct. Because of the delayed incorporation of the ureteral bud into the bladder, the resulting position of the ureteral orifice is more caudal and medial or in more severe cases it inserts into one of the Wolffian duct structures as noted above.

The function of the ureter is to effectively transport the urinary bolus from the minor calyces to the urinary bladder at acceptably low pressures. The efficiency of this task depends on adequate coaptation of the ureteral wall to propel the urinary bolus. If the ureter fails to propagate the peristaltic wave, the static urine distends the upper urinary tract and reduces luminal coaptation. Other factors that may affect ureteral transport include urinary volume and bladder pressure.

Megaureters

The term megaureter refers to an enlarged ureter. The 4 categories of megaureters are refluxing, obstructing, refluxing/obstructing, and nonrefluxing/nonobstructing. Each category is further divided into primary or secondary, based on either intrinsic or extrinsic causes for their appearance.

• Primary obstructed megaureter is most commonly caused by an adynamic juxtavesical segment of the ureter that fails to effectively propagate urine flow.
• Secondary obstructed megaureter occurs usually when ureteral dilatation is the result of a functional ureteral obstruction associated with elevated bladder pressures secondary to PUV or an NGB that impedes ureteral emptying.
• Primary refluxing megaureter is associated with severe VUR that alters ureteral efficiency by ureteral distention. The megaureter-megacystis syndrome is an extreme form of the primary refluxing megaureters in which massive reflux prevents effective bladder emptying because urine is passed back and forth between the ureters and bladder.
• Secondary refluxing megaureter occurs secondary to PUV or NGB when elevated bladder pressures cause decompensation of the UVJ.
• Primary nonrefluxing/nonobstructed megaureter is diagnosed when no evidence of obstruction or reflux can be demonstrated (diagnosis of exclusion).
• Secondary nonrefluxing/nonobstructed megaureter occurs secondary to diabetes insipidus, in which high urinary flow rates may overwhelm the maximum transport capacity of the ureter by peristalsis or as the result of ureteral atony accompanying a gram-negative UTI.
• Primary refluxing obstructed megaureter occurs in the presence of an incompetent VUJ that allows reflux through an adynamic distal segment.

Vesicoureteral reflux

A sufficient tunnel length of the submucosal ureter is the most important component of a competent UVJ, which provides a predominantly passive valve mechanism for ureteral compression and prevents retrograde urine passage. Factors that affect marginal tunnel pressure, causing either primary or secondary reflux, include the loss of UVJ compliance (during UTI), structural weaknesses in the detrusor floor (bladder diverticulum, ureterocele), or excessively high intravesical pressure resulting from neurovesical dysfunction or BOO. Reflux resulting from a congenitally deficient UVJ is referred to as primary reflux; reflux resulting from a BOO or NGB is referred to as secondary reflux.

No specific clinical signs are associated with ureteral anomalies. Most children present with an abnormal finding on routine prenatal ultrasound before associated symptoms or infection develops. Some patients present with UTI, abdominal mass or hematuria. Children with primary megaureters may also present with cyclic abdominal pain/flank pain, or, less commonly, in acute pain crisis.^[1] Patients may present with a cystic mass at the urethral meatus representing a prolapsed ureterocele. In other patients, the diagnosis is incidental after imaging studies for unrelated symptomatology.

Ureteral anomalies may be discovered during the evaluation of a patient with hypertension, proteinuria, or even renal insufficiency (in rare cases of severe bilateral anomalies).

Approximately 50% of females with ectopic ureters present with constant urinary incontinence or vaginal discharge. Consideration of an ectopic ureteral insertion should be given in prepubertal boys with recurrent epididymitis. Postpubertal males with ectopic ureters most commonly present with complaints of chronic prostatitis and painful intercourse and ejaculation. In males, incontinence is never due to an ectopic ureter because the ectopic ureter never inserts distal to the external urethral sphincter.

Megaureter

• Increasing hydroureteronephrosis
• Decrease in renal function of involved kidney
• Development of UTI or recurrent pain

Ureterocele

• Obstruction
• Urosepsis or compromised renal function may necessitate urgent decompression prior to definitive surgical reconstruction.

Vesicoureteral reflux (absolute indications)

• Progressive renal injury
• Documented failure of renal growth
• Breakthrough pyelonephritis
• Intolerance or noncompliance with antibiotic suppression
• Parental preference

Vesicoureteral reflux (relative indications)

• Pubertal age
• High-grade (IV or V) VUR
• Failure to resolve

Ureters are paired muscular tubes that run from the renal pelvis to the bladder and travel through retroperitoneal connective tissue in a serpentine fashion. The ureters run through 3 natural areas of narrowing: the ureteropelvic junction, the crossing of the iliac vessels, and the UVJ. From the renal pelvis to the iliac vessels, the ureter is referred to as the abdominal ureter. From the iliac vessels to the bladder, the ureter is called the pelvic ureter.

The UVJ may be divided into 3 sections: the terminal portion (juxtavesical ureter), the intramural portion, and the submucosal portion (under the bladder mucosa).

The presence of an acute UTI, especially with bullous edema of the bladder mucosa, may be a contraindication to definitive reconstruction. Urinary diversion (eg, ureterostomy, vesicostomy) or drainage may be necessary.