Diagnostic Imaging of Renal Cystic Diseases

1. Genito-Urinary System
Renal Cystic Diseases

2. Mohamed Zaitoun
Assistant Lecturer-Diagnostic Radiology
Department , Zagazig University Hospitals
Egypt
FINR (Fellowship of Interventional
Neuroradiology)-Switzerland
zaitoun82@gmail.com

5. Knowing as much as
possible about your enemy
precedes successful battle
and learning about the
disease process precedes
successful management

6. Renal Cystic Diseases
a) Renal Dysplasia
b) Polycystic Disease
c) Cortical Cysts
d) Medullary Cysts
e) Miscellaneous Intrarenal Cysts
f) Extraparenchymal Renal Cysts

7. a) Renal Dysplasia :
1-Multicystic Dysplastic Kidney (MCDK)
2-Focal & Segmental Cystic Dysplasia
3-Multiple Cysts Associated With Lower
Urinary Tract Obstruction

8. 1-Multicystic Dysplastic Kidney (MCDK) :
a) Incidence
b) Radiographic Features

9. a) Incidence :
-Collection of large noncommunicating cysts
separated by fibrous tissue, there is no
functioning renal parenchyma
-Develops in utero and the diagnosis is often made
either antenatally or in the early neonatal period
-There may be a predisposition for the left kidney ,
a slightly higher incidence in males for unilateral
MDCK and a higher incidence in females for
bilateral MCDK

10. b) Radiographic Features :
-Cystic renal mass with no excretory
function
-Hypertrophy of contralateral kidney
-Thick echogenic fibrous septa between
cysts
-Atretic ureter
-Absence of renal artery

11. Left kidney has multiple cysts without connection between them and
without residual normal parenchyma. Sp = fetal spine

18. 2-Focal & Segmental Cystic Dysplasia :
-Segmental dysplasia is seen in children
with duplex (duplicated) kidney
(incomplete fusion of upper and lower
poles)

19. 7-day-old girl with probable segmental dysplasia of left kidney, partial
sagittal ultrasound view of left kidney shows partially cystic, partially
echogenic mass (calipers), no substantial change was found during
follow-up examinations

20. 3-Multiple Cysts Associated With Lower
Urinary Tract Obstruction :
-Usually with posterior urethral valve in
males

21. b) Polycystic Disease :
1-Autosomal Recessive PKD
2-Autosomal Dominant PKD

23. 1-Autosomal Recessive PKD :
a) Incidence
b) Types
c) Associations
d) Radiographic Features

24. a) Incidence :
-Autosomal recessive polycystic kidney
disease is one of the commonest
inheritable infantile cystic renal diseases,
but is far less common than autosomal
dominant polycystic disease (ADPKD)
which affects adults

25. b) Types :
1-Antenatal Form : 90%
-Oligohydramnios in utero
-75% have death within 24 hours of delivery
2-Neonatal Form :
-Minimal hepatic fibrosis
3-Infantile Form :
-Moderate periportal fibrosis
4-Juvenile Form :
-Gross hepatic fibrosis
-Portal hypertension with splenomegaly and portosystemic
varices

26. c) Associations :
-Caroli disease
-Congenital hepatic fibrosis

27. d) Radiographic Features :
1-Kidneys :
-Enlarged hyperechoic kidneys (hallmark)
-Cysts of 1 to 2 mm are seen
2-U/S in Utero :
-Nonvisualization of urine in bladder
-Enlarged hyperechoic kidneys
-Oligohydramnios (nonfunctioning kidneys)

28. 3-Lungs :
-Pulmonary hypoplasia (because of external
compression)
-Pneumothorax
4-Liver :
-Hepatic fibrosis
-Portal venous hypertension

29. Fetal kidneys in ARPKD, (a) Longitudinal US scan of the left kidney in a 3rd-
trimester fetus shows an enlarged, echogenic kidney (arrows) with loss of
corticomedullary differentiation, note the paucity of amniotic fluid, indicating
oligohydramnios, (b) Transverse US scan of a 26-week-old fetus shows
enlarged kidneys (arrows) that are more echogenic than the liver (*)

32. ARPKD with caroli disease

33. ARPKD in a 13-year-old boy who was diagnosed as an infant, (a) US of the liver
demonstrates the “central dot sign” (arrowhead), which represents the portal vein and
hepatic artery branches completely surrounded by a dilated intrahepatic bile duct,
(b) CT shows periportal hypoattenuation representing periportal fibrosis and edema
(white arrow), cystic biliary dilatation with the central dot sign (white arrowhead), and
splenomegaly due to portal hypertension, ascites is noted, note the expanded
medullae with delayed transit of contrast material into the tubules of a segment of the
upper pole of the right kidney (black arrowhead), the hypoattenuating focus in the
spleen represents a splenic infarct (black arrow)

34. Respiratory distress in a newborn due to ARPKD with the oligohydramnios
sequence, chest and abdominal radiograph demonstrates bilateral flank
masses from enlarged kidneys that displace gas-filled bowel loops centrally
(*), the lungs are small with a bell-shaped thorax and left pneumothorax
(arrowhead) related to pulmonary hypoplasia

35. 2-Autosomal Dominant PKD :
a) Incidence
b) Associations
c) Radiographic Features

36. a) Incidence :
-Referred to as adult polycystic kidney disease
-Autosomal dominant polycystic kidney disease is
one of the most common serious hereditary
disease
-The kidneys are normal at birth and with time
develop multiple cysts , at the age of 30 years
approximately 68% of patients will have visible
cysts by ultrasound

37. b) Associations :
-Hepatic cysts , 70%
-Intracranial berry aneurysm , 20%
-Cysts in pancreas and spleen , <5%
-Hypertension
-Colonic diverticulosis

38. c) Radiographic Features :
-Kidneys are enlarged and contain innumerable
cysts
-Simple renal cysts will appear anechoic with well
defined imperceptible walls and posterior
acoustic enhancement, cysts with hemorrhage
or infection will demonstrate echogenic material
within the cyst

39. Ultrasound images of ADPKD, longitudinal view of right mid-kidney of an
advanced case (A), with the poles extending well beyond the image,
innumerable cysts of varying size and shape are present with very little
intervening parenchyma, (B) longitudinal images of right kidney of a child
with early, mild changes, several small cysts are present (arrows) and there
is a reduction in sinus fat due to expansion of the cortex

41. -CT : Simple cysts appear as rounded
structures with near water attenuation (HU
~ 0) , the wall are very thin and regular,
and are often imperceptible , cysts which
have had internal complications may be
hyperattenuating with internal non-
enhancing septations and / or
calcifications

45. With hepatic cysts

46. (a) Coronal T2 shows enlargement of both kidneys with multiple expansile renal
cysts (arrows), as well as multiple hepatic cysts (arrowheads), (b) Axial T2
fat-saturated obtained in another patient shows similar enlargement of the
kidneys, with multiple cysts (arrows)

47. c) Cortical Cysts :
1-Simple Cyst
2-Complicated Cyst
3-Multilocular Cystic Nephroma
4-Syndromes Associated With Cysts
5-End Stage Renal Disease &
Haemodyalisis

48. 1-Simple Cyst :
-Are benign and fluid filled
-There incidence increases with advancing age
and they are found in at least 50% of people >
50 years of age
-U/S :
*Anechoic
*Acoustic enhancement
*Sharply defined , imperceptible , smooth wall
*Round or ovoid shape

52. 2-Complicated Cyst :
-Are those that don’t meet the strict criteria of a simple
renal cyst
-These include cysts containing :
a) Internal echoes
b) Septations
c) Calcification
d) Perceptible defined wall and mural nodularity
a) Internal echoes :
*Internal echoes within a cyst are usually the result of
hemorrhage or infection

55. b) Septations :
*May be seen within a renal cyst and they
often occur following hemorrhage ,
infection and percutaneous aspiration

58. c) Calcification :
*Calcification of the renal cysts may be fine
and linear or amorphous and thick

62. d) Perceptible defined thickened wall or
mural nodularity :
*Essentially excludes a diagnosis of benign
cyst , these lesions will all require surgical
removal to exclude malignancy

66. -Bosniak Classification :
*Type 1 : Simple cyst
*Type 2 : Minimally complicated cyst
-Thin septation , calcium in wall
*Type 2F : Minimally complicated cyst
-Increased number of septa, minimally thickened
with nodular or thick calcifications
*Type 3 : Intermediate lesion
-Multiple thick septa , internal echos , mural
nodules
*Type 4 : Clearly malignant
-Solid mass component

68. Type 1

69. Type 2

70. Type 2F

71. Type 3

72. Type 4

73. 3-Multilocular Cystic Nephroma (MLCN) :
a) Incidence
b) Radiographic Features

74. a) Incidence :
-Congenital renal lesion characterized by
large (>10 cm) cystic spaces
-It occurs in children ages 2 months to 4
years with 75% male predilection and in
adults >40 years of age with 95% female
predilection
-In Differential Diagnosis of Pediatric renal
masses

75. b) Radiographic Features :
-CT+C shows a well-defined intrarenal
multilocular mass that compresses or
displaces the adjacent renal parenchyma ,
the septations enhance but the cysts do
not
-Thick septae, nodules or a large solid
component suggest Wilms tumor with
cystic degeneration

76. Sagittal US image of the left renal fossa demonstrates a multicystic
mass with variably sized cystic components and thin echogenic
septa

80. 4-Syndromes Associated With Cysts :
-VHL disease
-Tuberous Sclerosis
-Zellweger’s Syndrome
-Turner’s Syndrome
-Triosomy 13 & 18

81. Von Hippel-Lindau disease, CT+C depicts multiple cysts in both kidneys
(arrows) and an enhancing mass in the right kidney (arrowhead), the mass
has attenuation similar to that in soft tissue, a finding suggestive of clear cell
renal cell carcinoma

82. Tuberous sclerosis complex, CT+C shows multiple cysts in both
kidneys (arrowheads) in a patient with diagnosed tuberous sclerosis

83. Neonate with Zellweger syndrome, (a) Ultrasound image of kidney in 1-day-old
boy after birth shows multiple bilateral subcapsular cysts in diffusely
echogenic kidneys and no corticomedullary differentiation, (b) MR image of
trunk shows diffuse subcapsular cysts suggesting glomerulocystic kidneys

84. 5-End Stage Renal Disease &
Haemodyalisis :
-8-13 % in patients with renal failure not on
dialysis
-> 90 % of patents on dialysis after 5 years
-At least 3-5 cysts in each kidney

86. d) Medullary Cysts :
1-Medullary Sponge Kidney
2-Medullary Cystic Disease
3-Calyceal Cyst (Diverticulum)

87. 1-Medullary Sponge Kidney :
-Bilateral in 60-80 %
-Multiple small mainly pyramidal cysts
-Medullary nephrocalcinosis occurs in the
majority of cases (80%)

92. 2-Medullary Cystic Disease :
-Belongs to group of pediatric cystic renal
diseases
-Normal to small kidneys with multiple small
(1.5cm>) medullary cysts (sometimes
cysts are too small to visualize)
-Loss of CMD and increased parenchymal
echogenicity

94. 3-Calyceal Cyst (Diverticulum) :
-Small usually solitary cyst communicating
via an isthmus with the fornix of a calyx

97. e) Miscellaneous Intrarenal Cysts :
1-Traumatic
2-Inflammatory
3-Neoplastic
4-Cystic Hamartoma

98. 1-Traumatic :
-Intrarenal hematoma
2-Inflammatory :
-T.B.
-Calculus Disease
-Hydatid

99. 3-Neoplastic :
-Cystic Degeneration of a carcinoma
-5% of renal cell carcinomas are cystic
4-Cystic Hamartoma :
-Usually large with thick capsule and
septations

100. f) Extraparenchymal Renal Cysts :
1-Parapelvic Cyst
2-Perinephric Cyst

101. 1-Parapelvic Cyst :
-Located in or near the hilum but doesn’t
communicate with the renal pelvis and
therefore doesn’t opacify during
uropgraphy

104. 2-Perinephric Cyst :
-Beneath the capsule or between the
capsule and perinephric fat
-2ry to trauma , obstruction or replacement
of a hematoma