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• Cortical Cysts • Polycystic Disease of Kidney• Medullary Cysts• Multi-Cystic Renal Dysplasia• Extra-Parenchymal Renal Cysts

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• Simple cortical renal cysts

• Syndromes associated with renal cysts

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• Simple Cortical Renal Cysts

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1. Autosomal Dominant Polycystic Kidney

Disease

2. Autosomal Recessive Polycystic Kidney

Disease

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• Autosomal dominant.

• 1 in 1000 people.

• Third decade of life

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Extrarenal manifestations include:• Liver cysts in 25-50% of patients.

• Pancreatic cysts in 9%.

• Splenic cysts in 5% of patients.

• Other cysts reported include cysts of the thyroid, parathyroid, lung, brain,

pituitary gland, pineal gland, ovary, uterus, testis, seminal vesicles,

epididymis, bladder, and the peritoneum.

• Aneurysms of cerebral arteries (berry aneurysms) in 3-50% of patients.

• A variety of cardiac and aortic abnormalities including aortic root

dilatation, aortic regurgitation, bicuspid aortic valves, coarctation of the

aorta, mitral regurgitation, and abdominal aortic aneurysm.8

• Nephrogram findings may have a

characteristic Swiss cheese

appearance.

IVU:

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• The pelvocaliceal systems

demonstrate bilateral, diffuse

irregularity and splaying

• The collecting system may be

elongated, distorted, and

attenuated.

IVU:

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• Most children with ADPKD have normal ultrasonographic

findings at birth

US:

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• When cysts are seen, they vary in When cysts are seen, they vary in

size and are scattered throughout size and are scattered throughout

the cortex, including medulla and the cortex, including medulla and

cortex.cortex.

US:

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• On CT scans, the cysts are fairly well-

defined round or oval masses with low

attenuation values similar to those of water.

• After the intravenous administration of

iodinated contrast material, cysts do not

enhance but stand out prominently against

normally enhancing background renal

tissue.

CT:

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RAVINE CRITERIA

Age Positive family history Negative family history

< 30 y. 2 cysts bilateral 5 cysts bilateral

30-60 y. 4 cysts bilateral 5 cysts bilateral

> 60 y. 8 cysts bilateral 8 cysts bilateral15

• The most common heritable cystic renal disease The most common heritable cystic renal disease

occurring in infancy and childhood.occurring in infancy and childhood.

• The clinical spectrum shows a wide variability, The clinical spectrum shows a wide variability,

ranging from perinatal death to a milder ranging from perinatal death to a milder

progressive form, which may not be diagnosed progressive form, which may not be diagnosed

until adolescence.until adolescence.

• The Renal And Hepatic Disease Are Inversely The Renal And Hepatic Disease Are Inversely

Proportional To Each Other In Individual Patients.Proportional To Each Other In Individual Patients.

Classification of ARPKD:Classification of ARPKD:

• Category 1 (Perinatal ARPKD).Category 1 (Perinatal ARPKD).

• Category 2 (Neonatal ARPKD).Category 2 (Neonatal ARPKD).

• Category 3 (Infantile ARPKD).Category 3 (Infantile ARPKD).

• Category 4 (Juvenile ARPKD).Category 4 (Juvenile ARPKD).

Category 1 (Perinatal ARPKD) Category 1 (Perinatal ARPKD)

• Approximately 90% of the collecting ducts are dilated with Approximately 90% of the collecting ducts are dilated with

minimal liver involvement. minimal liver involvement.

• Severe renal impairment in utero leads to oligohydramnios and Severe renal impairment in utero leads to oligohydramnios and

subsequent pulmonary hypoplasia. Other clinical findings may subsequent pulmonary hypoplasia. Other clinical findings may

include sequelae of oligohydramnios, such as Potter facies and include sequelae of oligohydramnios, such as Potter facies and

clubfoot. clubfoot.

• Most of these infants do not survive beyond the first week of life. Most of these infants do not survive beyond the first week of life.

Potter Facies: Snubbed nose, low-set and flattened ears, deep eye creases and micrognathia.

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In the neonate: In the neonate:

• Decreased excretion of contrast material.Decreased excretion of contrast material.

• Nephromegaly.Nephromegaly.

• Characteristic striated nephrograms due to dilated Characteristic striated nephrograms due to dilated

collecting tubules.collecting tubules.

• Contrast material may remain in the dilated tubules for days Contrast material may remain in the dilated tubules for days

without visible excretion into the renal calyces. without visible excretion into the renal calyces.

IVUIVU

::

• Striated Nephrograms.Striated Nephrograms.

IVUIVU

::

• Prenatal sonography may Prenatal sonography may

demonstrate echogenic, demonstrate echogenic,

enlarged kidneys, enlarged kidneys,

oligohydramnios, or an oligohydramnios, or an

empty urinary bladder.empty urinary bladder.

IVUIVU

::

• Prenatal sonography may Prenatal sonography may

demonstrate echogenic, demonstrate echogenic,

enlarged kidneys, enlarged kidneys,

oligohydramnios, or an oligohydramnios, or an

empty urinary bladder empty urinary bladder

IVUIVU

::

US US

::

US US

::

CT:CT:

NCCT Kidneys are smooth, enlarged, and low →NCCT Kidneys are smooth, enlarged, and low →

in attenuation. in attenuation.

CT:CT:

CECT Striated pattern of contrast →CECT Striated pattern of contrast →

media excretion.media excretion.

CT:CT:

CT:CT:

• Medullary Sponge Kidney.

• Medullary Cystic Disease.

• Renal Papillary Necrosis.

• Congenital disease in which the renal medulla is replaced with numerous small cysts producing a sponge-like appearance.

• The majority of the cysts communicate with the tubules which may be dilated and tortuous.

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• Multiple small mainly pyramidal cysts that opacify with contrast due to communication with the pelvi-calyceal system

• Multiple medullary or corticomedullary junction cysts

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• Parapelvic cysts

• Perinephric cysts (urinoma)