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Weigert-Meyer law

Dr Henry Knipe and Radswiki et al. The Weigert-Meyer law describes the relationship of the upper and lower renal moieties induplicated collecting systems to their drainage inferiorly. Embryologically, duplication occurs when two separate ureteric buds arise from a single Wolffian duct. Due to the future lower pole ureter separating from Wolffian duct earlier, it migrates superiorly and laterally as the urogenital sinus grows and becomes the upper pole moiety. Despite this migration of the upper tract, the insertion inferiorly maintains the original embryologic relationship, and thus the upper pole moiety ureter drains infero-medial to the normal lower moiety ureter. The ectopic insertion often has a ureterocoele which obstructs its own collecting system, and can distort the orthotopic lower pole moiety insertion such that it is prone to reflux.

Bifid ureter
Dr Henry Knipe et al.

A bifid ureter (or ureter fissus) is an example of incomplete duplication of a duplex collecting system.

Epidemiology
Present in ~5% (range 1-10%) of the population
1-2

Gross anatomy
A bifid ureter is formed when there is a duplex kidney (separate pelvicalyceal collecting systems) drain into separate ureters, but the ureters unite before draining into the bladder at a single ureteric orifice 1. Rarely, one of the branches of a bifid ureter will be blind-ending and will not unite 2.

Differential diagnosis
double ureter - persistent of ureters to drain at separate ureteric orifices in the bladder

Circumcaval ureter
Dr Yuranga Weerakkody and Radswiki et al.

A circumcaval ureter (or retrocaval ureter) is a developmental anomaly of the inferior vena cava. Unfortunately both terms suggest that the ureter is at fault, whereas in reality it is the IVC.

Epidemiology
This abnormality is congenital.

Clinical presentation
Many patients with this anomaly are asymptomatic. Depending on the degree of compression patients may develop partial right ureteral obstruction or recurrent urinary tract infections due to urinary stasis.

Embryology
This abnormality occurs as a result of the right supracardinal system failing to develop normally. The right posterior cardinal vein persists and therefore ends up passing infront of the ureter. With one reported exception, the anomaly always occurs on the right side, as this is the side of the normal IVC.

Radiographic features
The proximal ureter courses posterior to the IVC, then emerges to the right of the aorta, coming to lie anterior to the right iliac vessels.

Treatment and prognosis


If treatment is required, surgical relocation of the ureter anterior to the IVC can be performed.

Duplex collecting system


Dr Henry Knipe and Dr Jeremy Jones et al. A duplex collecting system (or duplicated collecting system) is one of the most common congenital renal tract abnormalities 4-5. It is characterised by

incomplete fusion of upper and lower pole moieties resulting in complete or incomplete duplication of the collecting system (seeClassification below).

Epidemiology
Duplex collecting systems are seen in 0.7% of the normal adult population and in 2-4% of patients investigated for urinary tract symptoms 7.

Clinical presentation
Most duplicated systems asymptomatic and diagnosed incidentally. However, where symptoms do occur (infection, reflux or obstruction) the patient is likely to have completely duplicated ureters. Occasionally hydronephrosis can be severe enough to result in flank discomfort or even a palpable mass.

Associations

Fanconi anaemia 3

Classification
Duplex collecting system or duplex kidney anomalies can be classified into the following categories depending on the level or lack of of fusion 9-10: duplex kidney - two separate pelvicalcyeal systems draining a single renal parenchyma duplex collecting system - a duplex kidney draining into: o single ureter - i.e. duplex kidney's duplication pelvicalcyeal systems uniting at the pevi-ureteric junction (PUJ) o bifid ureter (ureter fissus) - two ureters that unite before emptying into the bladder o double ureter (complete duplication) bifid collecting system - refers to a duplex kidney with the two separate pelvicalcyeal collecting systems uniting at the PUJ or as bifid ureters double/duplicated ureters (or collecting system) - two ureters that drain separately into the bladder or genital tract

Pathology
Embryologically, duplication occurs when two separate ureteric buds arise from a single Wolffian duct. Interestingly, and explaining the Weigert-Meyer rule, the

future lower pole ureter separates from Wolffian duct earlier and thus migrates superiorly and laterally as the urogenital sinus grows. Duplication can be variable. At one end of the spectrum there is merely duplication of the renal pelvis, draining via a single ureter. At the other extreme, two separate collecting systems drain independently into the bladder or ectopically (see below) Duplex systems may be unilateral or bilateral and can be associated with a variety of other congenital abnormalities of the urinary tract, e.g. ureterocoele.

Radiographic features
As the abnormality is an anatomic alteration, all modalities able to image the renal tract may be able to visualise the typical features. General features include: duplicated ureters extending a variable distance down to the bladder obstruction of the upper pole moiety down to the bladder, often with a ureterocoele vesico-ureteric reflux into the lower pole moiety, often due to distortion in its insertion by the aforementioned ureterocoele ectopic insertion of the upper pole moiety e.g. into the prostatic urethra in males or vaginal vault in females Additionally, if reflux is significant, evidence of reflux nephropathy may be evident.

Fluoroscopy
Excretory urography (IVP) Although able to elegantly image both collecting systems, one should be aware that a poorly functioning system may not excrete contrast. In such a situation the functioning lower pole moiety will be inferiorly displaced, taking on the so called Drooping lily appearance1. The differential for such an appearance is that of an upper pole mass or cyst.

Ultrasound
Ultrasound, when no obstruction/hydronephrosis is present can be suboptimal in the detection of a duplicated system and will especially struggle to distinguish between partial and complete duplication. It is certainly able to detect ureterocoeles if present. It provides excellent anatomic information but does not necessarily differentiate a bifid renal pelvis from a bifid ureter or from two complete ureters.

CT
CT is able to delineate essentially all abnormalities, especially when performed during the excretory (IVP) phase (contrast outlining the collecting systems). In an unobstructed system, the diagnosis can be difficult. A duplicated renal collecting system can be suspected by identifying the so-called faceless kidney.

Nuclear medicine
Renal scintigraphy is significantly impaired in its ability to identify non-obstructed systems, as the spacial resolution is poor. However, it is able to evaluate renal function, and is particularly useful in planing corrective surgery.

Treatment and prognosis


Duplex kidney usually does not require any treatment per se however complications may necessitate intervention: vesicoureteric reflux into lower pole moiety marked hydronephrosis of upper pole moiety may have mass effect or become infected

Ureterocoele
Dr Henry Knipe and Dr Frank Gaillard et al. A ureterocoele is a congenital dilatation of the distal-most portion of the ureter. The dilated portion of the ureter may herniate into the bladder secondary to the abnormal structure ofvesicoureteric junction (VUJ).

Epidemiology
Most ureterocoeles are congenital, usually associated with ectopic insertion of the ureter with associated duplicated collecting system (see below). In a minority of cases, ureteroceles are an isolated abnormality, and these are usually seen in adults (see below).

Pathology
Genetics
Most cases have a sporadic occurrence.

Location and classification


There are two main types of ureterocoele, both of which are the result of cystic ectasia of the subepithelial portion of the ureter as it inters the bladder. simple - a ureterocoele that occurs at a VUJ in a normal position ectopic - that which occurs at a VUJ whose site is abnormal They pose a challenge for diagnosis and treatment because of the wide variety of anatomical abnormalties that may exist and the non-specific symptoms that patients present with. Simple ureterocoele: ~ 25 % Considerably less common that the ectopic variety and is almost always confined to the adult population. There is a congenital prolapse of a dilated distal ureter into the bladder lumen. Where they do do occur in children, they usually cause symptoms. Bilateral in about 30% of cases 2. Ectopic ureterocoele: ~ 75 % Almost always associated with a duplicated collecting system and the result of abnormal embryogenesis. There is abnormality in the early development of the intravesicular ureter, the ipsilateral kidney and its collecting system 1. It is significantly more common than the simple type. Approximately 80% of cases are unilateral and may cause obstruction to the entire renal tract because of prolapse into the bladder neck causing bladder outlet obstruction. Additionally, ureterocoeles may contain calculi.

Radiographic features
Ultrasound
A ureterocoele appears as a cystic structure projecting into the bladder, often near the normal location of the vesicoureteric junction (VUJ) - note that insertion can be ectopic. The associated ureter is usually noticeably dilated.

Fluoroscopy
IVP Detectable findings include

filling defect in the bladder (cobra head sign) which may be distended, collapsed or even everted radiolucent halo effect an everted ureterocoele will appear like a bladder diverticulum

MCUG

may show a round or oval lucency near the trigone there may be effacement of the defect with increasing filling of the bladder

Complications

progression to obstructive cystic renal dysplasia

Treatment and prognosis


The prognosis of ureterocoeles are related to the degree of associated reflux or obstruction. Depending on the size and position of a ureterocoele, they may prolapse into the ureter causing complete bladder obstruction.

Differential diagnosis
General imaging differential considerations include

pseudoureterocoele o obstruction and dilatation of an otherwise normal intramural ureter bladder diverticulum o the ureterocoele is everted

Pathophysiology[edit]
Ureteral development begins in the human fetus around the 4th week of embryonic development. A ureteric bud, arising from the mesonephric (or Wolffian) duct, gives rise to the ureter, as well as other parts of the collective system. n the case of a duplicated ureter, the ureteric bud either splits or arises twice. n most cases, the kidney is divided into two parts, an upper and lower lobe, with some overlap due to intermingling of collecting tubules. !owever, in some cases the division is so complete as to give rise to two separate parts, each with its own renal pelvis and ureter.

Classification[edit]

"ne of a series of #$ films at %& minutes after in'ection of contrast medium showed incomplete duplication of left collecting system.

Ureteral duplication is either( Partial ) i.e. the two ureters drain into the bladder via a single common ureter. $artial, or incomplete, ureteral duplication is rarely clinically significant. *+, or Complete ) in which the two ureters drain separately. -omplete ureteral duplication may result in one ureter opening normally into the bladder, and the other being ectopic, ending in the vagina, the urethra or the vulval vestibule. .hese cases occur when the ureteric bud arises twice (rather than splitting).*/,

Prevalence[edit]
0uplicated ureter is the most common renal abnormality, occurring in appro1imately %2 of the population.*+, Race( 0uplicated ureter is more common in -aucasians than in African) Americans. Sex( 0uplicated ureter is more common in females. !owever, this may be due to the higher fre3uency of urinary tract infections in females, leading to a higher rate of diagnosis of duplicated ureter.

Clinical Presentation[edit]
$renatally diagnosed hydronephrosis (fluid)filled kidneys) suggest post)natal follow)up e1amination. .he strongest neo)natal presentation is urinary tract infection. A hydronephrotic kidney may present as a palpable abdominal mass in the newborn, and may suggest an ectopic ureter or ureterocele. n older children, ureteral duplication may present as( Urinary tract infection ) most commonly due to vesicoureteral reflu1 (flow of urine from the bladder into the ureter, rather than vice versa). Urinary incontinence in females occurs in cases of ectopic ureter entering the vagina, urethra or vestibule.