Medullary Sponge Kidney, Clinical Features, Investigations and Treatment

Medullary Sponge Kidney:

Medullary Sponge Kidney a benign disorder is presented at birth and is not diagnosed until fourth or fifth decade. Kidneys have a marked irregular enlargement of the medullary and interpapillary collecting ducts. This is associated with medullary cysts that are diffuse giving a “Swiss cheese” appearance in these regions.

Clinical features of Medullary Sponge Kidney

Medullary Sponge Kidney presents in 50s or 60s with following symptoms.

Hematuria: gross or microscopic (red/dark colored urine)
Recurrent UTI,(increase urinary frequency, burning micturation, pain during urination, flank or abdominal pain)
Renal stone formation and nephrocalcinosis. (flank pain, blood in urine, pus in urine, urinary retention, supra-pubic pain)
Distal renal tubular acidosis
Decreased urinary concentrating ability. (large amount of colorless colored urine)

Investigations of Medullary Sponge kidney

Intravenous pyelography (IVP) (now called intravenous Urography IVU): it shows striations in the papillary portions of the kidney produced by accumulation of contrast in dilated collecting ducts,

medullary sponge kidney IVU

Treatment of Medullary Sponge Kidney

No medical curative treatment.
Adequate fluid intake to prevent stone formation.
if hypercalciuria presents then give thiazide diuretics that decrease calcium excretion.
Alkali therapy is advised if there is renal tubular acidosis.

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