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Diagnostic Ultrasound - Abdomen and Pelvis

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Xanthogranulomatous Pyelonephritis<br />

Diagnoses: Urinary Tract<br />

TERMINOLOGY<br />

• Chronic renal inflammation usually associated with<br />

longst<strong>and</strong>ing urinary calculus obstruction (75%)<br />

• Renal parenchyma is gradually replaced by lipid-laden<br />

macrophages<br />

• Diffuse (> 80%) <strong>and</strong> focal (< 20%) forms<br />

• 3 stages of XGP: Intrarenal → perirenal → perinephric ±<br />

retroperitoneal involvement<br />

IMAGING<br />

• Staghorn calculus with renal enlargement <strong>and</strong> perirenal<br />

fibrofatty proliferation on CT<br />

• Multiple focal low-attenuating renal masses with rim<br />

enhancement on CT<br />

• Renal sinus fat obliterated with large central "staghorn"<br />

calculus on any modality<br />

• Perinephric extension ± adjacent organs or structures may<br />

include sinus tracts or abscesses<br />

KEY FACTS<br />

• Diffusely enlarged kidney with hypoechoic round masses<br />

replacing normal parenchyma on US<br />

• <strong>Ultrasound</strong> ideal at initial investigation; CT good for<br />

assessing excretory function <strong>and</strong> retroperitoneal<br />

involvement<br />

PATHOLOGY<br />

• Lipid-laden "foamy" macrophages, diffuse infiltration of<br />

plasma cells <strong>and</strong> histiocytes<br />

CLINICAL ISSUES<br />

• Flank pain, fever, palpable mass & weight loss<br />

• Rare complications: Hepatic dysfunction, extrarenal<br />

extension, fistulas<br />

• Long-term chronic process with good prognosis if treated,<br />

<strong>and</strong> rare mortality<br />

• Antibiotic treatment is sometimes effective<br />

• Severe disease or perinephric extension usually requires<br />

nephrectomy<br />

(Left) Graphic shows lower<br />

pole XGP with a longst<strong>and</strong>ing<br />

ureteropelvic obstruction by a<br />

large staghorn stone ,<br />

causing replacement of<br />

parenchyma by collections of<br />

foamy macrophages .<br />

(Right) Transverse color<br />

Doppler US shows a dilated<br />

collecting system with<br />

internal debris <strong>and</strong> an<br />

obstructing stone st at the<br />

ureterovesical junction. The<br />

parenchyma is replaced by<br />

cystic spaces containing debris<br />

ſt. Note the lack of<br />

vascularity in the expected<br />

region of renal parenchyma.<br />

(Left) Two ultrasounds in<br />

another patient show cystic<br />

intraparenchymal spaces st<br />

peripheral to calculi (calipers)<br />

in central renal pelvis. Color<br />

Doppler US shows twinkling<br />

artifact with stones. (Right)<br />

Axial CECT shows a large<br />

central calculus , nearcomplete<br />

replacement of<br />

parenchyma by cystic<br />

collections st, <strong>and</strong> formation<br />

of an abscess <strong>and</strong> sinus tract<br />

necessitating into adjacent<br />

abdominal wall. Proliferation<br />

of perinephric fat in this<br />

otherwise cachectic patient is<br />

a response to chronic<br />

inflammation.<br />

488

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