Natural History of CVST
I believe you may be asking about CVST (Cerebral Venous Sinus Thrombosis), but the evidence provided relates to cystic kidney disease and ADPKD (Autosomal Dominant Polycystic Kidney Disease). I will address the natural history of cystic kidney disease based on the available evidence, as this appears to be what the evidence supports.
Natural History of Autosomal Dominant Polycystic Kidney Disease (ADPKD)
ADPKD follows a progressive course with bilateral kidney cyst formation leading to kidney enlargement, declining renal function, and eventual kidney failure, with disease severity stratified by the Mayo Imaging Classification predicting timing of kidney failure. 1
Disease Progression and Kidney Function
- Progressive cyst formation and renal enlargement lead to renal insufficiency, ultimately requiring lifelong dialysis or renal transplantation 2
- The Mayo Imaging Classification (MIC) divides height-adjusted total kidney volume (htTKV)/age into 5 different classes (1A through 1E) that predict future kidney function decline and timing of kidney failure 1, 3
- Only typical ADPKD (subclass 1) has validated prognostic value for predicting renal survival from birth 1
- Longitudinal eGFR slope aids in monitoring disease progression and identifying complications such as pain from cyst enlargement 1
Common Complications and Their Natural History
Hypertension
- High blood pressure develops commonly in ADPKD and requires regular monitoring with standardized office BP measurement 1
- Out-of-office BP measurements with home or ambulatory monitoring should complement office readings 1
Hematuria and Cyst Hemorrhage
- Macroscopic hematuria occurs in 5-15% of children with ADPKD 1
- Macroscopic hematuria is associated with enlarged total kidney volume in adults 1
- Gross hematuria before age 30-35 years is associated with worse renal survival in adults with ADPKD 1
Urinary Tract and Cyst Infections
- Cohort studies suggest an increased incidence of UTIs in children with ADPKD (up to 15-25%) 1
- Cyst infection is a very rare complication of childhood ADPKD 1
- Treatment of cyst infection has a high failure rate and requires long-term antibiotics, with cyst drainage reserved only for refractory infections 1
Nephrolithiasis
- Nephrolithiasis is an exceedingly rare complication in children with ADPKD 1
Extrarenal Manifestations
Liver Cysts:
- The prevalence of hepatic cysts in children with ADPKD is <5%, with no reports of severe cases 1
- The size and number of ADPKD-related liver cysts increase in pregnancy 1
- Regular screening for liver cysts is not recommended in children with confirmed ADPKD 1
Cardiac Manifestations:
- Mitral valve prolapse occurs in 12% of children with ADPKD, compared to 3% of healthy siblings 1
- Screening for mitral valve prolapse is not recommended in children with ADPKD without a heart murmur 1
Intracranial Aneurysms:
- Screening for intracranial aneurysms is not recommended for children with ADPKD 1
- Rupture of intracranial aneurysm is an exceedingly rare complication in childhood 1
Disease Variants and Atypical Presentations
- ADPKD can present as typical (bilateral and diffuse) or atypical forms including unilateral, segmental, asymmetric, or lopsided distributions 1
- Bilateral presentation with acquired unilateral or bilateral kidney atrophy represents another variant 1
- Early referral to a specialized centre is recommended for children with very-early-onset ADPKD or autosomal recessive polycystic kidney disease-like presentations 1
Systemic Nature of Disease
- ADPKD is a systemic disorder with cysts appearing with decreasing frequency in kidneys, liver, pancreas, brain, spleen, ovaries, and testis 4
- Cardiac valvular disorders, abdominal and inguinal hernias, and aneurysms of cerebral and coronary arteries and aorta are also associated with ADPKD 4
If you were asking about Cerebral Venous Sinus Thrombosis (CVST) rather than cystic kidney disease, please clarify and I can provide information on that condition instead.