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Renal Cysts in Children

Renal Cysts in Children

Introduction

Renal cysts are fluid-filled sacs that form in the kidneys. In children, they can be isolated findings or part of genetic syndromes. Understanding their classification and clinical significance is crucial for appropriate management.

Key Points

  • Prevalence: Simple renal cysts are found in approximately 0.1-0.45% of children
  • Age of presentation varies based on underlying etiology
  • Can be unilateral or bilateral
  • May be asymptomatic or present with complications

Types of Renal Cysts

1. Simple Renal Cysts

  • Solitary fluid-filled cavities
  • Well-circumscribed
  • No internal echoes on ultrasound
  • Usually asymptomatic

2. Complex Renal Cysts

  • Internal septations
  • Wall thickening
  • Calcifications
  • Solid components

3. Genetic Cystic Diseases

  • Autosomal Recessive Polycystic Kidney Disease (ARPKD)
    • Presents in infancy or early childhood
    • Bilateral kidney involvement
    • Associated liver disease
  • Autosomal Dominant Polycystic Kidney Disease (ADPKD)
    • May present in childhood
    • Progressive cyst formation
    • Family history often present

Clinical Presentation

Symptoms

  • Often asymptomatic and discovered incidentally
  • Flank pain or abdominal pain
  • Hematuria
  • Urinary tract infections
  • Hypertension
  • Mass effect symptoms

Physical Examination

  • Palpable abdominal mass in large cysts
  • Elevated blood pressure
  • Growth parameters (important in genetic conditions)
  • Associated anomalies in syndromic cases

Risk Factors

  • Family history of cystic kidney disease
  • History of kidney disease
  • Associated syndromes

Diagnostic Approach

Imaging Studies

  • Renal Ultrasound
    • Initial imaging modality of choice
    • Characterizes cyst features
    • Evaluates both kidneys
  • CT Scan
    • Better characterization of complex cysts
    • Bosniak classification
    • Limited use due to radiation exposure
  • MRI
    • Detailed anatomy without radiation
    • Useful for complex cases
    • Evaluation of associated conditions

Laboratory Studies

  • Renal function tests
  • Urinalysis
  • Genetic testing when indicated
  • Blood pressure monitoring

Treatment Approaches

Conservative Management

  • Regular monitoring
  • Blood pressure control
  • Treatment of complications
  • Periodic imaging

Surgical Intervention

  • Indications:
    • Large symptomatic cysts
    • Complicated cysts
    • Suspicion of malignancy
    • Progressive growth
  • Procedures:
    • Cyst aspiration
    • Sclerotherapy
    • Laparoscopic deroofing
    • Partial nephrectomy

Follow-up Care

  • Regular monitoring of:
    • Blood pressure
    • Renal function
    • Cyst size and characteristics
    • Growth and development
  • Genetic counseling when appropriate
Further Reading




Video Notes with Music



Renal Cysts: Objective QnA
  1. What is a renal cyst?
    A fluid-filled sac within the kidney
  2. What are the two main categories of renal cysts in children?
    Simple cysts and complex cysts
  3. Which imaging modality is most commonly used to diagnose renal cysts in children?
    Ultrasound
  4. What are the characteristics of a simple renal cyst on ultrasound?
    Anechoic, round or oval, thin-walled, with posterior acoustic enhancement
  5. How common are simple renal cysts in children?
    Relatively rare, occurring in less than 1% of children
  6. What is the most common presenting symptom of renal cysts in children?
    Most are asymptomatic and found incidentally
  7. What is autosomal dominant polycystic kidney disease (ADPKD)?
    A genetic disorder characterized by multiple bilateral renal cysts
  8. What genes are associated with ADPKD?
    PKD1 and PKD2
  9. At what age do renal cysts typically become detectable in children with ADPKD?
    Variable, but often in late childhood or adolescence
  10. What is autosomal recessive polycystic kidney disease (ARPKD)?
    A genetic disorder causing cystic kidney disease and congenital hepatic fibrosis
  11. What gene is associated with ARPKD?
    PKHD1
  12. How does ARPKD typically present in the neonatal period?
    Enlarged, echogenic kidneys, often with oligohydramnios and pulmonary hypoplasia
  13. What is multicystic dysplastic kidney (MCDK)?
    A congenital anomaly characterized by multiple non-communicating cysts replacing normal renal tissue
  14. What is the typical management approach for unilateral MCDK?
    Conservative management with serial ultrasound monitoring
  15. What percentage of MCDKs show complete involution by adolescence?
    Approximately 50%
  16. What is the association between MCDK and contralateral vesicoureteral reflux?
    Up to 25% of patients with MCDK have contralateral VUR
  17. What is a multilocular cystic nephroma?
    A rare, benign renal tumor composed of multiple cysts
  18. How is multilocular cystic nephroma typically managed?
    Surgical excision (partial or total nephrectomy)
  19. What is the difference between cystic partially differentiated nephroblastoma and Wilms tumor?
    Cystic partially differentiated nephroblastoma is a cystic variant of Wilms tumor with a better prognosis
  20. What is the role of genetic counseling in families with polycystic kidney disease?
    To provide information about inheritance patterns, prognosis, and family planning
  21. What extra-renal manifestations are associated with ADPKD?
    Liver cysts, intracranial aneurysms, mitral valve prolapse, and colonic diverticulosis
  22. What is the main differential diagnosis for ARPKD in the prenatal period?
    Posterior urethral valves in male fetuses
  23. What is the role of MRI in evaluating renal cysts?
    To further characterize complex cysts and differentiate cystic tumors
  24. What is a peripelvic cyst?
    A cyst located in the renal hilum, often mimicking hydronephrosis on ultrasound
  25. What is the Bosniak classification system used for?
    To categorize renal cysts based on their complexity and risk of malignancy
  26. In which condition are renal cysts associated with cysts in other organs?
    Tuberous sclerosis complex
  27. What is the management approach for asymptomatic simple renal cysts in children?
    Typically observation with periodic ultrasound monitoring
  28. What complications can arise from large renal cysts?
    Pain, infection, hemorrhage, or obstruction of the collecting system
  29. What is the role of cyst aspiration in pediatric renal cysts?
    It may be used for symptomatic relief or diagnostic purposes in complex cysts
  30. How does hypertension relate to renal cysts in children?
    It can be a complication of polycystic kidney diseases or large obstructing cysts
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