ICD10 code of Medullary Sponge Kidney and ICD9 code

Medullary Sponge Kidney (MSK), also known as Cacchi-Ricci disease, is coded as Q61.5 in the ICD-10-CM classification system. In the older ICD-9-CM system, this condition is identified by the code 753.16.

What is the clinical nature of Medullary Sponge Kidney?

Medullary Sponge Kidney is a congenital renal disorder characterized by the cystic dilation of the collecting ducts in the medullary pyramids of the kidneys. Because the urine flow is often stagnant within these "spongy" dilations, individuals with Medullary Sponge Kidney are at a significantly higher risk for recurrent nephrolithiasis (kidney stones) and urinary tract infections. While many people remain asymptomatic, those who do experience symptoms often face chronic pain and metabolic complications that require long-term management by nephrologists.

How is Medullary Sponge Kidney diagnosed?

Diagnosis typically involves imaging to visualize the characteristic "bouquet of flowers" appearance of the kidneys. The following diagnostic tools are most commonly utilized:

• Intravenous Pyelogram (IVP): Historically the gold standard for visualizing contrast medium pooling in the dilated ducts.


• Computed Tomography (CT) Urography: Currently the preferred non-invasive imaging method to identify Medullary Sponge Kidney.


• Renal Ultrasound: Often used for initial screening, though it may be less sensitive than CT imaging for detecting early-stage Medullary Sponge Kidney.

Is Medullary Sponge Kidney hereditary?

The genetics of Medullary Sponge Kidney are complex. While most cases are considered sporadic, there is evidence suggesting a potential autosomal dominant pattern of inheritance in some families. Research into the specific genetic mutations associated with Medullary Sponge Kidney is ongoing, and genetic counseling is recommended for families with a history of the condition.

Community Insights and Support

Navigating a rare diagnosis can be isolating. Within the DiseaseMaps.org community, 347 people with Medullary Sponge Kidney have shared their experiences. Connecting with others living with Medullary Sponge Kidney can provide valuable practical advice on managing chronic stone formation and coping with the emotional impact of the condition.

Next steps

• Consult a nephrologist to establish a metabolic workup for kidney stone prevention.


• Maintain high fluid intake to reduce the risk of stone formation associated with Medullary Sponge Kidney.


• Join the DiseaseMaps.org community to connect with other patients and share resources.


• Request a referral to a genetic counselor if you have a family history of renal cystic diseases.

Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment from a qualified healthcare provider.

References

• NIH Genetic and Rare Diseases Information Center (GARD): Medullary Sponge Kidney


• Orphanet: Cacchi-Ricci disease (ORPHA:2476)


• OMIM (Online Mendelian Inheritance in Man): Medullary Sponge Kidney (Entry #174000)


• National Kidney Foundation: Information on Rare Kidney Diseases