Fact-sheet: Medullary sponge kidney - Cacchi-Ricci disease
Updated on 09/30/2019 at 6:27 PM
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Definition
Also known as precalyceal canalicular ectasia.
A condition classified among the cystic diseases of the Kidney.
Etiology unknown.
Isolated dilatation of the distal portion of the collecting ducts (1 to 7 mm on histology) within several pyramids.
Uni- or bilateral disease.
Prevalence poorly defined, as the condition is asymptomatic apart from its complications.
Main complication: development of intratubular lithiasis (due to stasis within the dilated tubules).
Calcifications project in front of each papilla, resembling medullary Nephrocalcinosis.
Clinical features
Asymptomatic apart from complications.
Discovered during an episode of acute renal colic.
Infectious episodes secondary to urinary stasis.
Laboratory findings
Laboratory values unremarkable apart from complication episodes.
Possible associated metabolic abnormality favoring hypercalciuria.
Radiography
Possible visualization of stones on a supine plain abdominal film.
Ultrasound
Visualization of stones.
Cystic ectasias visible in advanced forms.
Infectious sequelae of the Kidney.
CT
Visualization of calculi.
Cystic ectasias visible in advanced forms.
Infectious sequelae of the kidney.
"Sponge" kidneys characteristic of the disease.
Vascular intervention
Intramedullary tubular dilatation is often visible only on intravenous urography (in the precalyceal phase).
Management
Management of calculi / renal colic, acute or chronic.
Management of infectious complications.
Control of metabolic factors if associated.
Differential diagnosis
Simple urinary lithiasis.
Nephrocalcinosis.