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Cystic Diseases of the Kidney

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Histopathological examination confirmed a renal cell carcinoma in a background of acquired cystic disease of the kidney. Back to: ... – PowerPoint PPT presentation

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Title: Cystic Diseases of the Kidney


1
Cystic Diseases of the Kidney
  • Dr. Raid Jastania

2
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

3
Case Presentation
4
  • 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.

8
  • 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
10
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

18
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

19
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.

27
  • 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

30
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

31
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

32
Renal stones
  • Predisposing factors for Mg Ammonium phospate
    stones
  • Alkaline urine
  • Infections, specially proteus, staph

33
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

34
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
38
Case Presentation
39
  • 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
48
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

49
Hydronephrosis
  • Congenital
  • Atresia of urethra or ureter
  • Valve of urethra or ureter
  • Renal torsion
  • Kinking of ureter

50
Hydronephrosis
  • Acquired
  • Foreign body stone
  • Tumors prostate, bladder
  • Inflammation prostatitis, urethritis,
    ureteritis, retroperitoneal fibrosis
  • Neurogenic spinal cord damage
  • Pregnancy mild, reversible

51
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

52
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

56
Hydronephrosis
  • Micro
  • Tubular dilatation, interstitial edema, mild
    inflammation
  • Atrophy, fibrosis
  • Late glomerular damage
  • When sudden and complete papillary necrosis
  • Infection is common

57
Hydronephrosis
  • Clinical
  • Bilateral, complete Anuria
  • Bilateral, incomplete polyuria
  • Unilateral detected late when both kidneys are
    affected

58
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

59
More Pictures
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