Cystic Diseases of the Kidney

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Cystic Diseases of the Kidney

• Dr. Raid Jastania

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Objectives

• By the end of this session the students should be
  able to
• List the common causes of renal cysts
• List the types of polycystic kidney disease
• List the types of renal stones and their
  implications
• Understand the pathogenesis of hydronephrosis

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Case Presentation
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• A 40-year-old Chinese man underwent surveillance
  ultrasonography (US) of the kidneys. He was
  suffering from chronic renal failure and was on
  regular haemodialysis for the past six years. The
  patient was otherwise asymptomatic and his
  dialysis program was unremarkable.

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• US scans showed a largely cystic lesion at the
  lower pole of the right kidney . However, there
  was a suspicious solid component at the rim of
  the lesion. Previous US scan performed 6 years
  ago revealed bilateral small and atrophic kidneys.

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• Unenhanced CT scan showed a low-density lesion at
  the lower pole of the right kidney, with a small
  spike of calcification at its margin.
• After contrast injection, the lesion was found to
  be predominantly cystic, but an enhancing solid
  component was noted at the rim of the cystic
  wall. Other CT sections confirmed the presence of
  bilateral atrophic kidneys with multiple cystic
  changes, compatible with acquired cystic disease
  of the kidney.

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• The patient underwent radical nephrectomy and he
  made an uneventful recovery.
• Histopathological examination confirmed a renal
  cell carcinoma in a background of acquired cystic
  disease of the kidney.

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Back toCystic Diseases of Kidney
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Cystic Diseases of the Kidney

• Hereditary
• Developmental
• Acquired

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Simple cyst

• Simple or multiple
• Usually cortical
• 1-5 cm
• Smooth surface
• Incidental finding

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Dialysis-Associated acquired cyst

• End-stage kidney, on hemodialysis
• Cortex and medulla
• May cause hematuria
• Increased risk of tumors
• Occasional adenoma, and renal cell carcinoma

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Adult Polycystic kidney

• Autosomal dominant
• PKD1 (polycystin-1) on Chromosome 16
• 90 of cases
• Cell-cell, cell-matrix adhesion molecule
• Both alleles must be lost (second somatic hit)
• PKD2 (polycystin-2) on chromosome 4, 10 of cases
• Multiple expanding cysts
• Destruction of renal parencyma
• 10 of chronic renal failure cases

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Adult polycystic Kidney

• Morphology
• Large up to 4 kg
• Masses of cysts
• Filled with fluid, clear, turbid, hemorrhagic
• Micro
• Cysts lined by single cuboidal epithelium
• Atrophy of the kidney

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Adult polycystic Kidney

• Clinical
• Present in the 4th decade
• Flank pain
• Mass
• intermittent hematuria
• Hypertension
• Infection
• Association berry aneurysm of brain, cysts in
  liver, pancreas, others. Diverticular disease.
• Slow progression
• End stage kidney at age of 50y

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Childhood polycystic kideny

• Autosomal recessive, chromosome 6
• Perinatal, neonatal, infantile, juvenile
• Pulmonary and renal failure
• Multiple cysts, uniform lining (collecting
  tubules)
• Association
• Multiple cysts in the liver
• Congenital hepatic fibrosis

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Dysplastic Kidney
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Case Presentation
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• A 52 year old man presented for evaluation of
  left flank pain of 1 year duration. He was
  nonsmoker with a history of left nephrolithotomy
  for staghorn calculus 20 years back. He had
  hypertension and ischemic heart disease as
  co-morbid conditions.
• On clinical examination patient had a scar of
  left flank incision with a palpable hard kidney.
  His urine analysis showed microscopic hematuria
  and culture was sterile. His renal biochemistry
  was normal.

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• On renal ultrasound a staghorn calculus was
  detected without associated hydronephrosis.
  Patient underwent transperitoneal laparoscopic
  nephrectomy. During surgery there were dense
  perirenal adhesions especially near the hilum.
  The hilar lymph nodes were also enlarged and
  densely adherent to the renal vessels. The
  procedure was electively converted to open
  surgery via flank incision. The total operating
  time was 5 hours and 20 minutes with estimated
  blood loss of 200 ml.

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• The patient had a smooth post-operative recovery.
  
• The histopathological examination of the specimen
  revealed presence of keratinizing type of
  squamous cell carcinoma of renal pelvis with
  metastasis to hilar lymph nodes. Patient was
  advised postoperative palliative
  chemo-radiotherapy. After six weeks of surgery
  patient developed fulminant herpes zoster and
  expired due to same.

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Back to Kidney Stones
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Urinary outflow obstruction

• Occur anywhere in the urinary system
• Renal stones common

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Urinary outflow obstruction

• Occur anywhere in the urinary system
• Renal stones common
• Pathogenesis
• 1. 75 Ca oxalate, or Ca phosphate
• 2. 15 Mg Ammonium Phosphate
• 3. 10 Uric acid
• 4. Rare cystine stones

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Renal stones

• Predisposing factors for Ca stones
• 1. High urine concentration
• 2. Hypercalcemia (hyperparathyroidism, vit. D
  intoxication, sarcoidosis)
• 3. Hypercalciuria
• 4. High uric acid in urine
• 5. hyperoxaluria

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Renal stones

• Predisposing factors for Mg Ammonium phospate
  stones
• Alkaline urine
• Infections, specially proteus, staph

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Renal stones

• Predisposing factors for uric acid stones
• 1. Gout
• 2. Hematologic malignancies eg. Leukemia
• Predisposing factor for cystine stone
• 1. Genetic defect in renal transport system for
  aminoacids

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Renal stones

• Morphology
• Unilateral 80
• Occur in renal pelvis, calyces, bladder
• Staghorn stone Mg ammonium phosphate
• Clinical
• Asymptomatic, massive hydronephrosis
• Renal colic flank pain, radiate to groin
• Gross hematuria
• infections

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Primary Oxalosis
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Case Presentation
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• A 78-year-old cachectic woman presented with an
  enormous abdominal tumour, which, according to
  the patient, had slowly increased in diameter.
  Medical history was unremarkable except for a
  hysterectomy gt30 years before. A CT scan revealed
  a giant cystic tumour filling almost the entire
  abdominal cavity. It was analysed by two
  independent radiologists who suspected a tumour
  originating from the right kidney and
  additionally a cystic ovarian neoplasm.

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• A diagnostic and therapeutic laparotomy was
  performed the tumour presented as a cystic, 35
  30 25 cm expansive structure adhesive to
  adjacent organs without definite signs of
  invasive growth. The right renal hilar vessels
  could finally be identified at its basis.
• Another tumourous structure emerged in the pelvis
  originating from the ovary was also resected.

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• The histopathological examination revealed a gt15
  kg hydronephrotic right kidney, lacking hardly
  any residual renal cortex parenchyma.
• The second specimen was identified as an ovary
  with regressive changes and a large partially
  calcified cyst. There was no evidence of
  malignant growth.

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Back toHydronephrosis
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Hydronephrosis

• Dilatation of renal pelvis and calyces
• Atrophy of the renal parenchyma
• Caused by obstruction partial, complete,
  unilateral or bilateral
• Causes
• Congenital (hereditary, developmental)
• Acquired

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Hydronephrosis

• Congenital
• Atresia of urethra or ureter
• Valve of urethra or ureter
• Renal torsion
• Kinking of ureter

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Hydronephrosis

• Acquired
• Foreign body stone
• Tumors prostate, bladder
• Inflammation prostatitis, urethritis,
  ureteritis, retroperitoneal fibrosis
• Neurogenic spinal cord damage
• Pregnancy mild, reversible

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Hydronephrosis

• Pathogenesis
• Obstruction high pressure leak of fluid in
  the interstitium fluid back to lymphtics
• High pressure dilated collecting system
  defect in the function of the tubules poor
  concentration of urine polyuria
• Higher pressure vascular stasis, congestion
  decrease blood flow decrease glomerular
  filtrate renal insufficiency

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Hydronephrosis

• Pathogenesis
• Irreversible damage occurs in 3 weeks of complete
  obstruction
• Or 3 months of partial obstruction

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Hydronephrosis

• Morphology
• Unilateral large kidney (20cm)
• Distended pelvis and calyces
• Atrophy of the parenchyma
• Hydronephrosis, hydroureters
• If sudden and complete
• Mild dilation
• Loss of renal function

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Hydronephrosis

• Micro
• Tubular dilatation, interstitial edema, mild
  inflammation
• Atrophy, fibrosis
• Late glomerular damage
• When sudden and complete papillary necrosis
• Infection is common

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Hydronephrosis

• Clinical
• Bilateral, complete Anuria
• Bilateral, incomplete polyuria
• Unilateral detected late when both kidneys are
  affected

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Objectives

• By the end of this session the students should be
  able to
• List the common causes of renal cysts
• List the types of polycystic kidney disease
• List the types of renal stones and their
  implications
• Understand the pathogenesis of hydronephrosis

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More Pictures
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