Cystic Renal Disease
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Cystic Renal Disease

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Imaging of cystic renal disease

Imaging of cystic renal disease

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  • Go to school - a common vexer in radiology - probably cyst on US. <br /> Take advantage of opportunities to look at US of known cysts to get better <br /> ie Seek veritas <br />
  • Figure 9.  Multicystic dysplastic kidney. (b) US scan shows a different pattern with larger cysts and more echogenic, dysplastic parenchyma. <br />
  • Multicystic dysplastic kidney in a 23-week-old fetus. (a) Sagittal MR image shows a large, multicystic structure in the left renal fossa (arrowhead). The cysts vary in size and are not connected, findings that are consistent with multicystic dysplastic kidney. The stomach is seen just inferior to the diaphragm (arrow). (b) Sagittal MR image shows a normal right kidney (arrowhead). <br />
  • Hi-res us now shows cystic areas <br />
  • Prenatal us on left with echogenic enlarged kidneys, ct on right in newborn <br />

Cystic Renal Disease Cystic Renal Disease Presentation Transcript

  • Cystic Renal Disease Jud Gash, MD
  • Cystic Renal Disease  Overview         Simple Cyst  complicated  hyperdense  Bosniak classification Cysts and cancer  ACKD, TS & VHL Multicystic dysplastic kidneys Autosomal Dominant and Recessive Polycystic Medullary Sponge Kidney Medullary Cystic Disease Localized Cystic Renal Disease Renal Sinus Cysts
  • Simple Cyst Most common adult renal mass (50% > 50yo)  Cortical; straw colored fluid  usually asymptomatic  BIB them 
  • Simple Cyst  IVP (historical) homogeneous, lucent  thin walls, smooth interface  “beak sign” - implies indolent process  Cannot dx on IVP; must confirm (US or CT) 
  • Simple Cyst  Ultrasound    anechoic thin, smooth wall increased thru transmission
  • Simple Cyst  CT and MRI  near water density (< 10 HU)        Check HU’s of other water thin, smooth wall homogeneous No enhancement MRI>CT Veritas tip – test yourself before ROI Pitfall – beware of Papillary RCC
  • Simple Cyst  Too small to characterize (subcentimeter) renal hypodensity  2 points: OK to say very likely cyst and BIB it  Although cant get water HU’s   A 5 mm cyst is more hypodense to your eye than 5 mm solid lesion
  • Cyst-makers vs Stranders
  • Complicated Renal Cyst   Simple cysts may become infected or hemorrhage/trauma US/CT/MRI   Thick walls; calcifications, septations, debris or increased attenuation And so, overlaps the appearance of cystic RCC  And therefore Bosniak Classification
  • 56 y/o with renal mass Hemorrhagic cyst at surgery
  • 74 with flank pain
  • Hyperdense Cyst   Benign cyst containing blood products (clotted, old not just acute) 50 -90 HU (hyperdense to water or kidney)     if no NCCT, can be isodense or hypodense to kidney on CCT 50% appear cystic( a few echoes), but may not US CT Criteria     < 3cm smooth wall (must extend beyond margin to evaluate) rounded; sharp margins **** NO SIGNIFICANT ENHANCEMENT ****
  • Hyperdense Cyst
  • Hyperdense Cyst
  • Hyperdense Cyst  Must distinguish from RCC if strict CT criteria or US, then some stop (II) or some follow (IIF)  if comes very close (intrarenal, 3.5 cm, elderly) could follow (IIF)  if doesnt meet criteria, “cannot RO RCC”   New Data helpful Too  >70HU noncontrast
  • Bosniak Classification    Most problematic renal masses are cystic Bosniak created a classification scheme to guide proper management Classification I - simple cyst  II - minimally complicated (1 or 2 thin, “nonenhancing”septi, delicate Ca++ in septi or wall; hyperdense)  III - thick walls or Ca++, thick septi, nodules  IV - definitely malignant (necrotic masses, etc.) 
  • Bosniak I
  • Bosniak II
  • Bosniak II-F
  • Bosniak III
  • Bosniak IV
  • Bosniak Classification  Management I - stop  II - stop or follow Up (IIf)  III & IV - surgery    Although most III will be benign, 30% will be cancer Problems Category II lesions (at least one study showed 4/5 malignant)  Interobserver variability between II and III 
  • Cysts and Cancer  3 main areas Acquired cystic kidney disease (ACKD)  Tuberous Sclerosis (TS)  Von-Hipple Lindau (VHL) 
  • Acquired Cystic Renal Disease   Development of cysts and occasional neoplasms in patients with CRF Can occur without dialysis (10%) but very common in patients with dialysis (either hemo or peritoneal)    ? prolongs lifespan so cysts develop 50% at 5 yrs, 90% at 10 years Impact of transplant    controversial cysts - some regress, some dont neoplasms - again controversial
  • Acquired Cystic Renal Disease  Findings  Cysts    Neoplasms    small early; enlarge and multiply in time; can mimic ADPCKD commonly Ca++, hemorrhage definite increased risk with CRF, again esp. dialysis often indolent in nature (papillary RCC) kidneys small; atrophic; hyperechoic; no other cysts
  • Tuberous Sclerosis  Renal manifestations occur in majority  Cysts – 15-50%    AML’s – 80% Neoplasm (RCC)    When occur tend to be in young children Controversial whether increased incidence predisposition suggested by younger age and bilateral nature No extrarenal cysts Renal cysts in a 10-month-old with TS.
  • Von Hippel-Lindau   Renal manifestations also common Findings  Cysts – 50-75%   RCC - 35%    BL; Mulitple bl and multiple Aggressive behavior pancreatic cysts
  • Multicystic Dysplastic Kidney    Congenital, non-hereditary dysplasia characterized by renal parenchyma replaced by multiple cysts due to dysplastic ureteral bud Clinical May present as abdominal mass in child or incidental in adult  ** 10-30% incidence of contralateral anomaly, esp UPJ and reflux** 
  • Multicystic Dysplastic Kidney  Types pelvoinfundibular atresia (most common) - atretic renal pelvis and ureter; cystic renal mass  Hydronephrotic type (rare) - ureteral atresia but dilated pelvis  segmental (rare) - in duplicated system 
  • Multicystic Dysplastic Kidney  Findings No function  multiple noncommunicating cysts replacing kidney  Usually no large central cystic area (UPJ)  large in child  Small to large, often Ca++ in adult (fails to grow)   MUST evaluate contra-lateral side
  • Multicystic Dysplastic Kidney Mercado-Deane M et al. Radiographics 2002;22:1429-1438
  • Multicystic Dysplastic Kidney
  • 23 week fetus with MCDK Huppert B J et al. Radiographics 1999;19:S215-S227
  • Autosomal Dominant Polycystic Kidney Disease  Clinically May present prenatally to elderly  Classically presents with CRF in 30-40 yo  Problems      Renal Failure - often, but not always, progressive HTN, UTI’s and pain SAH Autosomal Dominant condition
  • Autosomal Dominant Polycystic Kidney Disease  Manifestation     Renal Cysts  start small but enlarge over time  enlarge the kidney  often complicated with Ca++, hemorrhage  usually bl, symetric involvement Extrarenal cysts  liver (40-90%)  pancreas (10%)  Spleen (5%) Cerebral aneurysms (15-40%) No increased risk of RCC
  • Autosomal Dominant Polycystic Kidney Disease  IVP Bilateral enlarged lobulated kidneys  swiss cheese nephrogram (multiple lucencies on nephrogram)  arachnoid calyces (elongated, stretched calyces)   US & CT/MRI  bilateral large kidneys with cysts   often with Ca++, hyperdense Extrarenal cysts
  • Autosomal Dominant Polycystic Kidney Disease
  • Autosomal Dominant Polycystic Kidney Disease
  • Autosomal Recessive Polycystic Kidney Disease  Genetic condition two primary abnormalities Kidneys - cystic dilatation (1-8mm) of the collecting ducts -> renal failure  Biliary - biliary ectasia/periportal fibrosis -> portal hypertension; liver failure 
  • Autosomal Recessive Polycystic Kidney Disease  Clinically Classically classified as perinatal, neonatal, infantile and juvenile; now felt to be too rigid  Spectrum of expression with predominately renal and minimal hepatic disease when presentation in perinatal period; and predominately hepatic and milder renal disease when presenting in the older child  An individual can present anywhere along the spectrum 
  • Autosomal Recessive Polycystic Kidney Disease  Imaging  Perinatal form (most common)       massive nephromegaly usually without discrete cysts dominates the imaging Prenatal US - enlarged hyperechoic kidneys; decreased or absent bladder; oligohydramnios PF - flank masses; pulm. hypoplasia with ptx US - enlarged echogenic kidneys with loss of CMJ CT - nehpromegaly; striated prolonged nephrogram Liver US - usually normal or slightly hyperechoic
  • Autosomal Recessive Polycystic Kidney Disease
  • Autosomal Recessive Polycystic Kidney Disease
  • Autosomal Recessive Polycystic Kidney Disease  Imaging  Older child   The kidneys appear normal or similar to perinatal findings, only milder; occ. discrete cysts Liver US - hyperechoic; may see cystic dilatation of the biliary tree; changes of portal HTN (HSM; varices)
  • Medullary Sponge Kidney  Pathology   saccular 1-8mm dilatation of the distal collecting ducts with sluggish flow and predisposition to stone formation Clinical     Young adults usually asymptomatic except stones non-hereditary; nonprogressive assoc. with Caroli’s, ARPCKD; hemihypertrophy
  • Medullary Sponge Kidney  Imaging (PF, US and CT)  Medullary nephrocalcinosis   bilateral, unilateral or focal “growing calculus sign” Urolithiasis  Discrete linear collections in papilla on IVP   should be distinguished papillary blush - a nondiscrete blush of contrast within the papillary which can be normal
  • Medullary Sponge Kidney
  • Medullary Sponge Kidney
  • Medullary Sponge Kidney
  • Medullary Sponge Kidney
  • Medullary Cystic Disease  Clinicopathologic      Rare inherited disease Chronic failure in early adulthood Medullary cysts two types Imaging     Medullar cysts small, smooth kidneys with poor function Occasionally see dilated, contrast filled tubules in medulla (mimics MSK) No ca++
  • Localized Cystic Disease     Multiple conglomerated cysts with intervening normal (or compressed) renal tissue Uncommon and benign Unilateral (other kidney normal) Part or all of kidney AJR 2001; 176:843-849
  • Localized Cystic Disease  DDX   Abscess MCDK   MLCN and CysticRCC   Kidney functions normally Normal interneving tissue and lack of capsule Should Follow  Can Grow Multilocular cystic RCC – capsule with enhancing tumorous septa AJR 2001; 176:843-849
  • Renal Sinus Cysts  2 types  parapelvic cyst   peripelvic cyst   simple parenchymal cyst which projects into the sinus extraparenchymal cyst arising in sinus (?origin; ? congenital and lymphatic etiology) Imaging (peripelvic) May be multiple and bilateral, unilocular or multilocular; usually small and insinuating  smooth splaying of collecting system  rarely results in obstruction and hydronephrosis 
  • Renal Sinus Cyst (parapelvic)
  • Renal Sinus Cysts  Imaging  US     cyst in renal sinus sinus cyst (delays needed to exclude hydro) CT DDX hydronephrosis
  • Renal Sinus Cysts
  • Renal Sinus Cysts
  • Cystic Renal Disease  Review         Simple Cyst  complicated  hyperdense  Bosniak classification Cysts and cancer  ACKD, TS & VHL Multicystic dysplastic kidneys Autosomal Dominant and Recessive Polycystic Medullary Sponge Kidney Medullary Cystic Disease Localized Cystic Disease Renal Sinus Cysts