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Medullary Sponge Kidney, Clinical Features, Investigations and Treatment

medullary sponge kidney 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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