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    URETER DISEASE

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    ANATOMY

    The ureters are the ducts that carry urine from the kidneys tothe urinary bladder, passing anterior to the psoas major. Theureters are muscular tubes that can propel urine along by themotions ofperistalsis. In the adult, the ureters are usually 25-30cm long.

    In humans, the ureters enter the bladder through the back,running within the wall of the bladder for a few centimetres.There are no valves in the ureters, backflow being prevented by

    pressure from the filling of the bladder, as well as the tone ofthe muscle in the bladder wall.

    In the female, the ureters pass through the mesometrium on theway to the urinary bladder.

    http://en.wikipedia.org/wiki/Urinehttp://en.wikipedia.org/wiki/Kidneyhttp://en.wikipedia.org/wiki/Urinary_bladderhttp://en.wikipedia.org/wiki/Psoas_majorhttp://en.wikipedia.org/wiki/Peristalsishttp://en.wikipedia.org/wiki/Mesometriumhttp://en.wikipedia.org/wiki/Urinary_bladderhttp://en.wikipedia.org/wiki/Urinary_bladderhttp://en.wikipedia.org/wiki/Mesometriumhttp://en.wikipedia.org/wiki/Peristalsishttp://en.wikipedia.org/wiki/Psoas_majorhttp://en.wikipedia.org/wiki/Urinary_bladderhttp://en.wikipedia.org/wiki/Kidneyhttp://en.wikipedia.org/wiki/Urine
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    HISTOLOGY

    The ureter has a diameter of about 3 millimeters, and the lumen isstar-shaped. Like the bladder, it is lined with transitional epithelium,

    and contains layers of smooth muscle. The epithelial cells of the ureter are stratified (in many layers), are

    normally round in shape but become squamous (flat) when stretched.The lamina propria is thick and elastic (as it is important that it isimpermeable).

    There are two spiral layers of smooth muscle in the ureter wall, aninner loose spiral, and an outer tight spiral. The inner loose spiral is

    sometimes described as longitudinal, and the outer as circular, (this isthe opposite to the situation in the gastrointestinal tract). The distalthird of the ureter contains another layer of outer longitudinal muscle.

    The adventitia of the ureter, like elsewhere is composed offibrousconnective tissue, that binds it to adjacent tissues.

    http://en.wikipedia.org/wiki/Transitional_epitheliumhttp://en.wikipedia.org/wiki/Lamina_propriahttp://en.wikipedia.org/wiki/Spiralhttp://en.wikipedia.org/wiki/Gastrointestinal_tracthttp://en.wikipedia.org/wiki/Fibrous_connective_tissuehttp://en.wikipedia.org/wiki/Fibrous_connective_tissuehttp://en.wikipedia.org/wiki/Fibrous_connective_tissuehttp://en.wikipedia.org/wiki/Fibrous_connective_tissuehttp://en.wikipedia.org/wiki/Gastrointestinal_tracthttp://en.wikipedia.org/wiki/Spiralhttp://en.wikipedia.org/wiki/Lamina_propriahttp://en.wikipedia.org/wiki/Transitional_epithelium
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    http://en.wikipedia.org/wiki/Image:Ureter.JPG
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    URETER DISEASE

    Congenital

    Inflamation

    Tumor

    Obstruction

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    Congenital Disease

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    1. Duplex (duplicated) system

    The duplex system is a renal unit in which the kidney has 2pelvicaliceal systems. A patient with a duplication anomalymay have a single ureter with a duplicated collecting

    system, bifid ureters (partial or incomplete duplication), or 2ureters that empty separately into the bladder (completeduplication). The upper ureter is more likely to beassociated with ectopic insertion, ureterocele, and/orobstruction, whereas the lower ureter is frequentlyassociated with vesicoureteral reflux (VUR).

    The upper (lower) pole is one of the components of theduplex kidney. The upper (lower)-pole ureter drains theupper (lower) pole of a duplex kidney.

    Caudal or medial ectopia describes the ureteral orifice whenlocated at the proximal lip of the bladder neck or beyond.

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    2. Ectopic ureter

    Ectopic ureter occurs when the ureter drains to anabnormally 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).

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    3. Megaureter

    A megaureter is a wide ureter. Megaureters may be classified as obstructed,

    refluxing, obstructed and refluxing, and

    nonobstructed/nonrefluxing.

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    Etiology of Megaureter

    Primary obstructing megaureter is caused by a structuralalteration in the muscular layers of the distal ureter, which ischaracterized, to varying degrees, by diminished or absentlongitudinal muscle fibers, hypertrophied or hyperplasticcircular muscle fibers, or increased connective tissuedeposition. These changes are defined pathologically andmay represent either an arrest of normal development or anintrauterine response of the ureter to ongoing obstruction.

    Animal models of congenital megaureter are lacking, butMortell et al have developed a rat model of prenataldoxorubicin (Adriamycin) exposure that may help elucidatethe etiology of this developmental defect.

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    Pathophysiology of Megaureter

    Partial obstruction in the abnormal distal segmentof the ureter leads to progressive dilatation.Progression to hydronephrosis (ie, dilation of therenal pelvis and calyces) occurs when the ureter nolonger accommodates resistance to urinarydrainage; pressure is then conveyed moreproximally. Complete obstructions are rare and areinvariably associated with a nonfunctioning renalunit at diagnosis.

    Primary obstructed megaureters enter the bladderin a normal location on the trigone, with the ureteral

    orifice appearing unaffected. This entity should notbe confused with ectopic megaureters that end in

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    Clinical presentation of primary

    megaureter

    Includes the following:

    UTI

    Hematuria

    Pyuria Flank mass

    Failure to thrive

    Sepsis

    Fever of unknown origin

    Abdominal or pelvic pain

    Abdominal mass

    Urolithiasis

    Serendipitous identification on imaging (eg, ultrasonography ; CT scanning;kidneys, ureters, and bladder [KUB] radiography; bone scan)

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    Light microscopic findings in primary obstructive megaureters. A, Operative

    exposure. B, Specimen; note that the obstructed segment often admits a catheter or

    probe. C, Longitudinal section showing no abnormally. D, Reduced muscle bulk seen

    in some megaureters. E, Circular muscle preponderance. F, Thickened adventitia.

    Various abnormalities are encountered with light microscopy.

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    Electron microscopic findings in primary obstructive megaureter. A, Operative

    specimen. B, Muscle cell atrophy, absent nexus, and excessive collagen and

    ground substance in the intercellular space from dilated ureter. C, Abnormal

    collagen fibers between muscle cells reduced from x 4000. D, Abnormality

    reduced from x 17,000 from narrow ureter.

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    Etiology of Uterocele

    The precise embryologic etiology of the ureterocele has beengreatly debated. Several theories exist, including obstruction of theureteral orifice, incomplete muscularization of the intramuralureter, and excessive dilation of the intramural ureter during thedevelopment of the bladder and trigone.

    The most commonly accepted theory behind ureterocele formationis the obstruction of the ureteral orifice during embryogenesis, withincomplete dissolution of the Chwalla membrane. (The Chwalla

    membrane is a primitive thin membrane that separates the ureteralbud from the developing urogenital sinus.) Failure of thismembrane to completely perforate during development of theureteral orifice is thought to explain the occurrence of aureterocele.

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    Clinical symptoms of ureteroceles

    May include the following:

    Urinary tract infection

    Urosepsis

    Obstructive voiding symptoms

    Urinary retention

    Failure to thrive

    Hematuria

    Cyclic abdominal pain

    Ureteral calculus

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    Intravenous urogram demonstrating left hydroureteronephrosis

    due to a ureterocele represented by the round filling defect

    located at the left base of the bladder

    http://www.emedicine.com/med/images/Large/1823ivptwoureteroceles.jpg
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    5. Vesicoureteral reflux

    VUR is defined as retrograde regurgitation of urine from the urinary bladder upthe ureter and into the collecting system of the kidneys. The InternationalReflux Grading system classifies VUR into 5 grades, depending on the degreeof retrograde filling and dilation of the renal collecting system. This system is

    based on the radiographic appearance of the renal pelvis and calyces on avoiding cystogram, as follows:

    Grade I: Urine backs up into the ureter only, and the renal pelvis appearshealthy, with sharp calyces.

    Grade II: Urine backs up into the ureter, renal pelvis, and calyces. The renalpelvis appears healthy and has sharp calyces.

    Grade III: Urine backs up into the ureter and collecting system. The ureter and

    pelvis appear mildly dilated, and the calyces are mildly blunted. Grade IV: Urine backs up into the ureter and collecting system. The ureter and

    pelvis appear moderately dilated, and the calyces are moderately blunted.

    Grade V: Urine backs up into the ureter and collecting system. The pelvisseverely dilates, the ureter appears tortuous, and the calyces are severelyblunted.

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    Megaureter with reflux. A, Light microscopy shows attenuated musculature of

    intravesical ureter. B, Electron microscopy shows normal muscle cells of the

    juxtavesical ureter reduced from x 3000. C, Electron microscopy shows

    intravesical ureter with decreased muscle cell population.

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    Ureteritis is infection of one or both ureters, thetubes that connect the kidneys to the bladder.

    The spread of an infection from the kidneys orbladder is the most common cause of ureteritis.Another cause is a slowing of the flow of urinebecause of a defective nerve supply to part of theureter. An underlying kidney or bladder infection

    is treated with an antibiotic. The sections of theureter in which nerves are defective may need tobe removed surgically.

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    Ureteritis cystica

    In this example of ureteritis cystica, the ureteral lumen has been opened

    longitudinally. Several fluid-filled vesicles are seen on the urothelialsurface.

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    Frequency

    The mean age of occurrence is 65 years.

    Incidence of TCC increases with age. Moreover, tumors rarely arefound at autopsy.

    Upper tract urothelial tumors occur more frequently in men, with amale-to-female ratio of 3:1.

    Upper tract urothelial tumors occur more frequently in white people ascompared to black people, with a white-to-black ratio of 2:1.

    An association exists between upper tract TCC and Balkannephropathy. Balkan nephropathy is a degenerative interstitialnephropathy that confers a 100- to 200-times greater increase in theincidence of upper tract TCC in individuals from rural Balkan areasrelative to that observed in individuals residing in neighboringcommunities. Tumors generally are low grade, multiple, and bilateralcompared with TCC of other etiologies.

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    Etiology

    Tobacco smoking is the factor most strongly associated with uppertract TCC and confers a greater than 3-fold increase in risk. Estimatespoint to smoking as the cause of 70% of upper tract tumors in men and

    40% in women. Drinking coffee causes a slight increase in risk of upper tract TCC.

    This tends to be observed in people who consume more than 7 cupsof coffee per day.

    Analgesic abuse also is a risk factor for urothelial malignancy. It isindependent and synergistic with renal papillary necrosis. Long-termexposure to analgesics induces a nephropathy that is associated with

    an incidence of upper tract TCC as high as 70%. The pathognomonicfinding for analgesic abuse, called capillarosclerosis, is found in 15%of patients with upper tract tumors.

    Occupational exposure to agents utilized in the petrochemical, plastic,and tar industries has been linked to increased risk of TCC.

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    Chronic infections, irritation, and calculi also may predispose apatient to develop squamous cell carcinoma and, lesscommonly, adenocarcinoma of the upper urinary tract.

    Cyclophosphamide has been linked to the development ofurothelial tumors. More specifically, a breakdown metabolitecalled acrolein is thought to be the causative agent. Whenimplicated, the associated tumors tend to be high grade.

    Finally, heredity may play a part in the development of TCC. Anassociation exists with Lynch syndrome II, a syndrome that

    includes the early onset of proximal colonic nonpolyposistumors, numerous synchronous and metachronous

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    Problem

    Upper tract urothelial tumors of the renal pelvis and

    ureters are relatively rare. Tumors of the renal pelvis

    account for approximately 10% of all renal tumorsand approximately 5% of all urothelial tumors.

    Ureteral tumors are even more uncommon,

    occurring with one quarter the incidence of renal

    pelvis tumors. Transitional cell carcinoma (TCC)accounts for more than 90% of upper tract urothelial

    tumors.

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    Pathophysiology

    Upper tract tumor types

    TCC is the most common histology observed, accounting for greaterthan 90% of upper tract urothelial tumors. TCCs are strongly

    associated with smoking. Squamous cell carcinoma comprises 1-7% of upper tract urothelial

    tumors. Squamous cell carcinoma frequently is associated withinfected staghorn calculi that have been present for a long duration.Affected patients frequently present with moderately to poorlydifferentiated tumors and advanced disease.

    Adenocarcinoma is observed in less than 1% of upper tract tumors.

    Patients with adenocarcinoma of the upper tracts also may haveassociated calculi and long-term obstruction, suggesting an etiologicorigin for these processes.

    Inverted papilloma is an unusual lesion. This generally is considered abenign histologic lesion, but it may harbor foci of malignant change.

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    Urothelial Carcinoma involving Ureter

    A nephroureterectomy specimen showing bulbous expansion of proximal

    ureter near the renal pelvis caused by papillary urothelial carcinoma.

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    Transitional cell carcinoma (TCC)

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    Ureteropelvic Junction Obstruction

    Problem: UPJ obstruction is defined as anobstruction of the flow of urine from the renal pelvis

    to the proximal ureter. The resultant back pressurewithin the renal pelvis may lead to progressive renaldamage and deterioration.

    UPJ obstruction presents most frequently inchildhood, but adults and elderly individuals also canpresent with a primary obstructive lesion. In adults,other etiologies for ureteral obstruction must beconsidered, including stones, ureteral compressionfrom extrinsic processes, retroperitoneal fibrosis, andother inflammatory processes.

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    ETIOLOGY

    Possible etiologies for UPJ obstruction include the following:

    Intrinsic obstruction may occur secondary to stenosis from scarring ofureteral valves.

    Ureteral hypoplasia may result in abnormal peristalsis through theUPJ. Asymmetry of ureteral wall musculature may inhibit the naturalperistaltic emptying of the renal pelvis into the ureter.

    Abnormal or a high insertion of the ureter into the renal pelvis maycause an altered configuration and impaired drainage of urine. Thismay be an effect rather than a cause because the 2 etiologiesmentioned previously may present with a high-insertion variant seen

    on imaging studies. Crossing lower pole renal vessel(s) or entrapment of the ureter by a

    vessel can prohibit urinary flow down the ureter. Vessels that wraparound the UPJ may be associated with obstruction or can be aproduct of renal dilation and hydronephrosis that distorts renalvascular architecture.

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    Rotation of the kidney, such as renal ectopy, and renal hypermobilitycan cause intermittent obstruction that is solely dependent on theposition of the kidney relative to the ureter. This was once a very

    popular diagnosis, but today, the other aforementioned etiologies aremore prevalent and this cause is particularly rare.

    Secondary UPJ obstruction can be caused by prior surgicalintervention for other disorders, such as renal stone disease or failedrepair of a primary UPJ obstruction. This obstructive lesion mostcommonly is secondary to ureteral wall and periureteral scarformation.

    The above abnormalities all cause impaired drainage of urine from thekidney into the ureter, resulting in elevated intrarenal back pressure,dilation of the collecting system, and hydronephrosis.

    http://www.emedicine.com/med/images/Large/698269gitlin1.jpg
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    Intravenous pyelogram

    demonstrating a ureteropelvic

    junction obstruction with dilationof the collecting system and lack

    of excretion of contrast

    Retrograde pyelogram

    demonstrating a

    ureteropelvic junction

    obstruction secondary to

    annular stricture.

    http://www.emedicine.com/med/images/Large/757269gitlin2.jpghttp://www.emedicine.com/med/images/Large/698269gitlin1.jpg
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