Infundibulopelvic Stenosis Multicystic Kidney Syndrome C1832949
A congenital kidney condition where the collecting system fails to develop properly, causing fluid-filled cysts. Often affects both kidneys and impacts kidney function. Treatment depends on severity and may involve monitoring or surgical intervention.
Key facts at a glance
- ICD-10
- N28.9
- ICD-11
- GA00.0
- Body system
- renal
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Changes in urination or swelling
(renal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Palpable flank or abdominal mass
(renal)Hypertension (high blood pressure)
(renal)Recurrent urinary tract infections
(renal)Hematuria (blood in urine)
(renal)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Pyeloplasty for obstruction relief
Surgical reconstruction of the infundibulopelvic junction to relieve stenosis and improve urinary drainage from affected kidney segments. May be performed open or laparoscopically.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Renal function and imaging surveillance
Regular assessment of kidney function through serum creatinine, eGFR, and imaging studies to monitor cystic changes, obstruction progression, and overall renal reserve.
Additional treatment options
Hypertension management
Blood pressure control using renoprotective agents to prevent further kidney damage and manage secondary hypertension that commonly accompanies renal structural abnormalities.
medicationPercutaneous cyst drainage
Image-guided aspiration or drainage of symptomatic cysts causing pain, infection, or compression of adjacent kidney tissue. May provide temporary relief.
procedureFluid intake optimization and activity modification
Adequate hydration to maintain urine flow and reduce infection risk, combined with activity restrictions if bleeding or cyst rupture is a concern.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Infundibulopelvic Stenosis Multicystic Kidney Syndrome C1832949?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy