PUJO aetiology

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A ureteropelvic junction (UPJ) obstruction can be thought of as a restriction to flow of urine, from the renal pelvis to the ureter, which, if left uncorrected, leads to progressive renal deterioration The response to obstruction is the development of renal pelvic hypertrophy, in which the kidney 4/14/12

Congenital UPJ obstruction most often results from intrinsic disease. A frequently found defect is the presence of an aperistaltic segment of the ureter, perhaps similar to that found in primary obstructive megaureter
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UPJ obstruction may also result from acquired lesions. In children, vesicoureteral reflux can lead to upper tract dilatation with subsequent elongation, tortuosity, and kinking of the ureter. true UPJ obstruction may definitely coexist with 4/14/12 vesicoureteral reflux, although it

UPJ obstruction occurs in all pediatric age groups, but there tends to be a clustering in the neonatal period because of the detection of antenatal hydronephrosis today the majority of cases are identified and diagnosed in the 4/14/12 perinatal period .

Beyond the neonatal period, UPJ obstruction is seen during childhood and adolescence but to a lesser degree. Often, the cause is an aberrant lower pole parenchymal vessel that crosses over the UPJ Obstruction occurs more commonly in boys than in girls ; 4/14/12 especially in the newborn period,

ETIOLOGY The precise cause of UPJ obstruction remains elusive despite investigation along a number of lines: ? embryologic ,? anatomic A narrowing of the UPJ is often

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The typical findinsg a narrowed segment of the ureter at the UPJ that is probe patent . This finding may be the result of an interruption in the development of the circular musculature of the UPJ or an alteration of the collagen 4/14/12 fibers and composition between

transforming growth factor-, epidermal growth factor expression, nitric oxide, and neuropeptide Y increased UPJ stenosis A less frequent intrinsic cause of congenital UPJ obstruction is true ureteral stricture. Such congenital ureteral 4/14/12 strictures are most frequently

Other causes of intrinsic UPJ obstruction valvular mucosal folds

persistent fetal convolutions upper ureteral polyps

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Intrinsic obstruction at the UPJ may also result from kinks or valves produced by infoldings of the ureteral mucosa and musculature . The presence of these kinks, valves, bands, or adhesions may also produce angulation of the ureter at the lower margin of the renal pelvis in such a manner 4/14/12

some cases, the high insertion itself is likely the primary obstructing lesion, because this phenomenon is found more frequently in the presence of renal ectopia or fusion anomalies

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Congenital folds are a common finding in the upper ureter of fetuses after the fourth month of development and may persist until the newborn period. Such folds are mucosal infolds with an axial offshoot and adventitia that does not flatten out when the ureter is distended or stretched. 4/14/12

This provides a length reserve for the ureter, which traverses a shorter distance in the newborn than in the adult stling thought that these folds were a precursor of UPJ obstruction because they frequently were discovered in babies who had a contralateral UPJ obstruction. This concept has evolved, and

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Extrinsic An aberrant, accessory, or earlybranching lower pole vessel is the most common cause of extrinsic UPJ obstruction These vessels pass anteriorly to the UPJ or proximal ureter and contribute to mechanical obstruction.
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Nixon (1953) reported that 25 of

Whether the aberrant vessel causes obstruction or is a covariable that exists along with an intrinsic narrowing is unclear when an aberrant or accessory renal artery to the lower pole of the kidney is present and the ureter courses behind it, the ureter may angulate at both the UPJ and the point at which it 4/14/12

Other acquired causes of obstruction at the UPJ include benign tumors such as fibroepithelial polyps urothelial malignancy, stone disease, postinflammatory or postoperative scarring or ischemia 4/14/12

UPJ obstruction, although most often a congenital problem, can present clinically at any time of life. the most common presentation in neonates and infants was the finding of a palpable flank mass asymptomatic newborns being diagnosed with hydronephrosis, 4/14/12 many of whom are subsequently

In older children or adults, intermittent abdominal or flank pain, at times associated with nausea or vomiting, Hematuria, either spontaneous or associated with otherwise relatively minor trauma, . Laboratory findings of microhematuria, pyuria, or frank 4/14/12 urinary tract infection

Lower Pole Ureteral Pelvic Junction Obstruction Lower pole UPJ obstruction can occur in kidneys with an associated incomplete renal duplication . Hydronephrosis of the lower pole moiety can be delineated with a well-performed sonogram
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. Such a finding can suggest the

Associated Anomalies Congenital renal malformations are commonly seen in association with UPJ obstruction . Other urologic abnormalities may be found in 50% of affected infants UPJ obstruction is the most common anomaly encountered in 4/14/12 the opposite kidney; it occurs in

UPJ obstruction was noted in 21% of children with the VATER (vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia) association VUR has been found in as many as 40% of affected children This degree of reflux is often low 4/14/12 grade, not contributing to upper

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