DISORDERS OF THE

URINARY SYSTEM

MEDULLARY SPONGE

KIDNEY

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 P A 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 T A N

DR. ASMATULLAH SAPAND

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……………

Medullary Sponge Kidney

Medullary Sponge Kidney

Medullary Sponge Kidney

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.

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.

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.

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.

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.

Diagnosis

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.

Complications of MSK

Renal stones UTIs Hematuria Distalrenal tubular acidosis (Type 1 RTA) Renal insufficiency (rarely) Marked chronic pain CRF