07.06.2016

1. A H M A D S H A H A B D A L I
I N S T U T U T E O F H I G H E R
E D U C AT I O N
D E PA R T M E N T O F
I N T E R N A L M E D I C I N E
K H O S T - A F G H A N I S TA N
DR. ASMATULLAH SAPAND

2. Medullary Sponge Kidney
 MSK also known as Cacchi-Ricci disease, is a
congenital disorder characterized by Marked irregular
cycstic dilatation of the medullary and interpapillary
collecting ducts in one or both kidneys.
 Associated with medullary cysts.
 Giving a Swiss-Cheese appearance in these regions.
Cont……………

3. Medullary Sponge Kidney

4. Medullary Sponge Kidney

5. Medullary Sponge Kidney

6. Medullary Sponge Kidney
 Patients with MSK are at increased risk of developing
kidney stones and Urinary Tract Infections.
 MSK is a benign disorder present at birth and not
diagnosed until fourht or fifth decade.
 10% of patients with MSK have an increased risk of
morbidity and associated with frequent stones (2-fold)
and UTIs.

7. Etiology
 Genetic Basis (Autosomal dominant inheritance).
 Occlusion and subsequent dilatation of the collecting
duct by uric acid during fetal life.
 Tubular obstruction due to calcium oxalate calculi
secondary to infantile hypercalciuria.

8. Epidemiology
 The frequency of MSK is 1 in 5000 to 1 in 20,000
births.
 The frequency of MSK is 12-21% in patients with
kidney stones(nephrolithiasis).
 The disease is bilateral in 70% of cases.

9. Presentation (Clinical Features)
 Most cases are asymptomatic and discovered during
investigation for hematuria.
 Symptomatic patients typically present as middle aged
adults with renal colic, nephrolithiasis, nephrocalcinosis
and/or recurrent UTIs.
 Some times the renal colic may be intractable.

10. Diagnosis
 Renal Ultrasonography (U/S) or Abdominal x-ray:
• it shows hyperdense papillae with clusters of small
stones.
• Intravenous urography (pyelography): it shows
irregular collecting ducts, sometimes having
“paintbrush-like” appearance. However, IV urography
has been largely replaced by contrast-enhanced CT.

11. Diagnosis

12. Treatment
 No medical curative treatment available.
 Adequate fluid intake to prevent stone formation.
 UTIs if occur should be treated.
 In patients with low levels of citrate or distal renal
tubular acidosis, treatment with potassium citrate
helps prevent the formation of new kidney stones.
 If hypercalciuria present then give thiazide diuretics.

13. Complications of MSK
 Renal stones
 UTIs
 Hematuria
 Distalrenal tubular acidosis (Type 1 RTA)
 Renal insufficiency (rarely)
 Marked chronic pain
 CRF