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Cystic Renal Disease
Jud Gash, MD
Cystic Renal Disease
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Overview
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Simple Cyst
 complicated
 hyperdense
 Bosniak classification
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 proces...
Simple Cyst


Ultrasound

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anechoic
thin, smooth wall
increased thru transmission
Simple Cyst
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CT and MRI
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near water density (< 10 HU)
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
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Check HU’s of other water

thin, smooth wall
hom...
Simple Cyst


Too small to characterize (subcentimeter) renal
hypodensity


2 points:
OK to say very likely cyst and BIB...
Cyst-makers vs Stranders
Complicated Renal Cyst




Simple cysts may become infected or
hemorrhage/trauma
US/CT/MRI


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Thick walls; calcificat...
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...
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 co...
Bosniak Classification





Most problematic renal masses are cystic
Bosniak created a classification scheme to guide
p...
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 I...
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 d...
Acquired Cystic Renal Disease


Findings


Cysts






Neoplasms






small early; enlarge
and multiply in time;
...
Tuberous Sclerosis


Renal manifestations
occur in majority
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Cysts – 15-50%
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AML’s – 80%
Neoplasm (RCC)
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



...
Von Hippel-Lindau


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Renal manifestations
also common
Findings


Cysts – 50-75%


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RCC - 35%



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BL; Mulitple
b...
Multicystic Dysplastic Kidney





Congenital, non-hereditary dysplasia
characterized by renal parenchyma replaced by
m...
Multicystic Dysplastic Kidney


Types
pelvoinfundibular atresia
(most common) - atretic
renal pelvis and ureter;
cystic r...
Multicystic Dysplastic Kidney


Findings
No function
 multiple noncommunicating cysts replacing kidney
 Usually no larg...
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 C...
Autosomal Dominant Polycystic
Kidney Disease


Manifestation







Renal Cysts
 start small but enlarge over time
...
Autosomal Dominant Polycystic
Kidney Disease


IVP
Bilateral enlarged lobulated kidneys
 swiss cheese nephrogram (multip...
Autosomal Dominant Polycystic
Kidney Disease
Autosomal Dominant Polycystic
Kidney Disease
Autosomal Recessive Polycystic
Kidney Disease


Genetic condition two primary abnormalities
Kidneys - cystic dilatation (...
Autosomal Recessive Polycystic
Kidney Disease


Clinically
Classically classified as perinatal, neonatal, infantile and
j...
Autosomal Recessive Polycystic
Kidney Disease


Imaging


Perinatal form (most common)





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
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massive nephromega...
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
peri...
Medullary Sponge Kidney


Pathology




saccular 1-8mm dilatation of
the distal collecting ducts
with sluggish flow and...
Medullary Sponge Kidney


Imaging (PF, US and CT)


Medullary nephrocalcinosis



bilateral, unilateral or focal
“grow...
Medullary Sponge Kidney
Medullary Sponge Kidney
Medullary Sponge Kidney
Medullary Sponge Kidney
Medullary Cystic Disease
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Clinicopathologic
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Rare inherited disease
Chronic failure in early
adulthood
Medull...
Localized Cystic Disease


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Multiple
conglomerated
cysts with
intervening
normal (or
compressed) renal
tissue
Uncom...
Localized Cystic Disease


DDX
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Abscess
MCDK
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MLCN and CysticRCC


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Kidney functions
normally
Normal internev...
Renal Sinus Cysts


2 types


parapelvic cyst


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peripelvic cyst


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simple parenchymal cyst which projects into th...
Renal Sinus Cyst
(parapelvic)
Renal Sinus Cysts


Imaging


US
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

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cyst in renal
sinus
sinus cyst
(delays needed
to exclude
hydro)

CT

DDX hy...
Renal Sinus Cysts
Renal Sinus Cysts
Cystic Renal Disease


Review











Simple Cyst
 complicated
 hyperdense
 Bosniak classification
Cysts an...
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Cystic Renal Disease

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Imaging of cystic renal disease

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Cystic Renal Disease

  1. 1. Cystic Renal Disease Jud Gash, MD
  2. 2. 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
  3. 3. Simple Cyst Most common adult renal mass (50% > 50yo)  Cortical; straw colored fluid  usually asymptomatic  BIB them 
  4. 4. Simple Cyst  IVP (historical) homogeneous, lucent  thin walls, smooth interface  “beak sign” - implies indolent process  Cannot dx on IVP; must confirm (US or CT) 
  5. 5. Simple Cyst  Ultrasound    anechoic thin, smooth wall increased thru transmission
  6. 6. 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
  7. 7. 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
  8. 8. Cyst-makers vs Stranders
  9. 9. 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
  10. 10. 56 y/o with renal mass Hemorrhagic cyst at surgery
  11. 11. 74 with flank pain
  12. 12. 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 ****
  13. 13. Hyperdense Cyst
  14. 14. Hyperdense Cyst
  15. 15. 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
  16. 16. 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.) 
  17. 17. Bosniak I
  18. 18. Bosniak II
  19. 19. Bosniak II-F
  20. 20. Bosniak III
  21. 21. Bosniak IV
  22. 22. 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 
  23. 23. Cysts and Cancer  3 main areas Acquired cystic kidney disease (ACKD)  Tuberous Sclerosis (TS)  Von-Hipple Lindau (VHL) 
  24. 24. 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
  25. 25. 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
  26. 26. 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.
  27. 27. Von Hippel-Lindau   Renal manifestations also common Findings  Cysts – 50-75%   RCC - 35%    BL; Mulitple bl and multiple Aggressive behavior pancreatic cysts
  28. 28. 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** 
  29. 29. 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 
  30. 30. 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
  31. 31. Multicystic Dysplastic Kidney Mercado-Deane M et al. Radiographics 2002;22:1429-1438
  32. 32. Multicystic Dysplastic Kidney
  33. 33. 23 week fetus with MCDK Huppert B J et al. Radiographics 1999;19:S215-S227
  34. 34. 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
  35. 35. 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
  36. 36. 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
  37. 37. Autosomal Dominant Polycystic Kidney Disease
  38. 38. Autosomal Dominant Polycystic Kidney Disease
  39. 39. 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 
  40. 40. 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 
  41. 41. 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
  42. 42. Autosomal Recessive Polycystic Kidney Disease
  43. 43. Autosomal Recessive Polycystic Kidney Disease
  44. 44. 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)
  45. 45. 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
  46. 46. 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
  47. 47. Medullary Sponge Kidney
  48. 48. Medullary Sponge Kidney
  49. 49. Medullary Sponge Kidney
  50. 50. Medullary Sponge Kidney
  51. 51. 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++
  52. 52. 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
  53. 53. 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
  54. 54. 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 
  55. 55. Renal Sinus Cyst (parapelvic)
  56. 56. Renal Sinus Cysts  Imaging  US     cyst in renal sinus sinus cyst (delays needed to exclude hydro) CT DDX hydronephrosis
  57. 57. Renal Sinus Cysts
  58. 58. Renal Sinus Cysts
  59. 59. 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

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