benign and malignant cystic renal disease including hydronephrosis

1. CYSTIC DISEASES OF
KIDNEY
DR. ARPITA
SAHA

2. KIDNEY CYST
• A FLUID-FILLED SAC
• LINED BY AN EPITHELIUM
• ARISING FROM A DILATATION IN ANY
PART OF THE NEPHRON OR
COLLECTING DUCT

3. 1. MULTICYSTIC RENAL DYSPLASIA
2. POLYCYSTIC KIDNEY DISEASE
A. AUTOSOMAL-DOMINANT (ADULT) POLYCYSTIC DISEASE
B. AUTOSOMAL-RECESSIVE (CHILDHOOD) POLYCYSTIC DISEASE
3. MEDULLARY CYSTIC DISEASE
A. MEDULLARY SPONGE KIDNEY
B. NEPHRONOPHTHISIS
4. ACQUIRED CYSTIC DISEASE
A. DIALYSIS-ASSOCIATED
B. HYDATID CYST
C. TUBERCULOSIS
D. NEOPLASM ASSOCIATED
5. LOCALIZED (SIMPLE) RENAL CYSTS
6. RENAL CYSTS IN HEREDITARY MALFORMATION SYNDROMES (E.G., TUBEROUS SCLEROSIS,
VHL)
7. GLOMERULOCYSTIC DISEASE
8. EXTRAPARENCHYMAL RENAL CYSTS
A. PYELOCALYCEAL CYSTS
B. HILAR LYMPHANGITIC CYSTS

4. ADPKD
• HEREDITARY
• AGE OF PRESENTATION- USUALLY 4TH
TO 5TH
DECADE
• COMMON
• 1/400- 1000 LIVE BIRTH
• M/C MUTATION-PKD1 IN CHROMOSOME 16 (85% CASES, MORE SEVERE)
• POLYCYSTIN 1
• PKD 2 IN CHROMOSOME 4 (LESS SEVERE)
• POLYCYSTIN 2

6. ADPKD
• GROSS:
• USUALLY BILATERAL ENORMOUS
ENLARGEMENT
• SIZES; WEIGHTS
• EXTERNAL SURFACE: MASS OF CYSTS
WITHNOINTERVENING PARENCHYMA
• CYSTS: CLEAR/SEROUS FLUID/TURBID
REDTOBROWN/HEMORRHAGIC FLUID
• ENLARGEMENT PRESSURE EFFECTS
ON CALYCES ANDPELVIS . .+/-

7. MICROSCOPY
• CYSTS: LINEDBY
CUBOIDAL/FLATTENEDEPITHELIUM
• INTERVENENING AREA:
ISCHEMIC ATROPHY
FUNCTIONINGNEPHRONS
INTERSTITIAL SCARRING
TUBULARATROPHY
• PELVICALYCEAL SYSTEM: PRESSURE
EFFECT

8. CLINICAL FEATURES
• ASYMPTOMATIC / RENAL INSUFFICIENCY > 30 YRS
• PAIN: HAEMORRHAGE/PROGRESSIVE DILATION OF CYSTS
• RENAL COLIC: EXCRETION OF BLOOD CLOTS CAUSES.
• ABDOMINAL PALPATION: HUGELY ENLARGED KIDNEYS
• HEMATURIA
• FEATURES OF PROGRESSIVE CHRONIC KIDNEY DISEASE:
• PROTEINURIA , POLYURIA, AND HYPERTENSION.

9. CLINICAL FEATURES…..
• PATIENTS WITH PKD2 MUTATIONS:
>OLDER AGE AT ONSET
>LATER DEVELOPMENT OF RENAL FAILURE.
• PROGRESSION IS ACCELERATED IN:
BLACKS (LARGELY CORRELATED WITH SICKLE-CELL TRAIT)
MALES
IN THE PRESENCE OF HYPERTENSION

10. DIAGNOSIS USUALLY ESTABLISHED BY USG
• REVEALS DIFFUSE
HYPERECHOGENICITY, BILATERALLY
ENLARGED KIDNEYS WITH CYSTS
• CRITERIA FOR ADPKD: <30 YEARS: AT
LEAST 2 CYST IN ONE KIDNEY
• IF EITHER PARENT HAS ADPKD
FINDING OF ENLARGED ECHOGENIC
KIDNEYS IN FETUS CONFIRMS
PRENATAL DIAGNOSIS

11. EXTRARENAL CONGENITAL ANOMALIES
• POLYCYSTIC LIVERDISEASE
• SPLEEN
• PANCREAS
• LUNGS
• INTRACRANIAL BERRY ANEURYSMS
• PINEAL GLAND
• SEMINAL VESICLE
• MITRAL VALVE PRO LAPSE AND OTHER CARDIAC VALVULAR ANOMALIES
• COLONIC DIVERTICULA
• SKELETAL ABNORMALITY

12. CLINICAL OUTCOME
• LONG RUN ESRD
• CAUSE OF DEATH
• 40% CORONARY/HYPERTENSIVE HEART DISEASE
• 25% INFECTION
• 15% RUPTURED BERRY ANEURYSM
• REST OTHER CAUSES

13. ARPKD
• RARE 1: 40,000 LIVE BIRTH
• PKHD1 GENE IN CHROMOSOME 6
• FIBROCYSTIN

14. GROSS
• Enlarged
• Smooth external appearance.
• RETAIN RENIFORM SHAPE
• C/S- numerous small cysts in the cortex
and medulla kidney spongelike
• Dilated elongated channels are present
at RIGHT ANGLES TO THE CORTICAL
SURFACE
• Complete REPLACEMENT OF the
medulla and cortex

15. MICROSCOPY
• CYLINDRICAL DILATION OF ALL
COLLECTING TUBULES.
• CYSTS - UNIFORM LINING BY
CUBOIDAL CELLS
LIVER
• CYSTS
• ASSOCIATED WITH PORTAL FIBROSIS
• PROLIFERATION OF PORTAL BILE
DUCTS.

16. CLINICAL FEATURE
• LARGE ABDOMINAL MASS AT BIRTH
• POTTER PHENOTYPE
• FACIES D/T OLIGOHYDROMNIOS
• JOINT DEFORMATION
• PULMONARY HYPOPLASIA
• SEVERE- NEWBORN DIE SHORTLY
AFTER BIRTH
• OLDER CHILDREN (4-8 YRS) HEPATIC
DISEASE
• APPROX 23% EXPERIENCE VARICEAL
BLEEDING

17. MULTICYSTIC RENAL DYSPLASIA
• M.C.CAUSE OF PALPABLE ABDOMINAL LUMP IN NEONATE
• M.C. CAUSE OF CYSTIC DISEASE IN CHILDREN
• SPORADIC DISEASE
• DUE TO ABNORMAL METANEPHRIC DIFFERENTIATION

18. MRD
• A/W
• URETEROPELVIC ANOMALY
• CARDIOVASCULAR ABNORMALITY
• GROSS
• ENLARGED
• EXREMELY IRREGULAR
• MULTICYSTIC KIDNEY

19. MRD
• CYST LINED BY FLATTTENED EPITHELIAL LINIG
• SURROUNDED BY UNDIFFERENTIATED MESENCHYME, CARTILAGE, IMMATURE
COLLECTING DUCT
• NORMAL NEPHRONS ARE THERE BUT MANY OF THEM HAVE IMMATURE CD

20. UROLITHIASIS

21. TYPES
• FOURMAIN TYPES
• (1) CALCIUMSTONES - CALCIUMOXALATE ORCALCIUMOXALATE MIXEDWITH
CALCIUMPHOSPHATE
• (2) TRIPLE STONES ORSTRUVITE STONES- MAGNESIUMAMMONIUM
PHOSPHATE
• (3) URIC ACIDSTONES
• (4) CYSTINE

22. CAUSES
• OBSCURED
• PREDISPOSING FACTORS
• CONC OF SOLUTE
• CHANGES IN pH
• BACTERIAL INFECTIONS
• LACK OF INHIBITORS OF CRYSTAL FORMATION IN URINE

23. CALCIUM OXALATE STONE
• MC CAUSE HYPERCALCIURIA
1. ABSORPTIVE
2. RENAL
• ALKALINE URINE PREDISPOSE

24. STRUVITE STONE
• ALKALINE URINE
• POST UTI (P. vulg aris )
• AVITAMINOSIS A

25. URIC ACID STONE
• GOUT
• DISEASES INVOLVING RAPID CELL TURNOVER EG, LEUKEMIA
• ACIDIC URINE

26. CYSTINE STONE
• ACIDIC URINE
• A/W IMPAIRED RENAL CYSTINE TRANSPORT

27. MORPHOLOGY
• USUALY UNILATERAL
• RENAL PELVIS & CALYCES > BLADDER >…….
• SMALL/ LARGE
• SMOOTH/ JAGGED
• STAGHORN CALCULI

29. CLINICALLY
• ASYMPTOMATIC/ SIGNIFICANT RENAL
DAMAGE
• COLIC
• OBSTRUCT URINE FLOW ULCERATION,
BLEEDING (GROSS HEMATURIA) 
BACTERIAL INFECTION