Development of Choroid Plexus

  • Choroid plexus development starts between 6–7 weeks post-conception (p/c).
  • Initial formation occurs in the fourth ventricle.
  • Later becomes most prominent in the lateral ventricles.
  • Achieves adult anatomical configuration by ~20 weeks gestation.

Formation Process:

  • Begins as neuroepithelial projections into ventricular cavities.
  • These projections form choroidal villi.
  • Small cysts/tubules can develop within these villi due to neuroepithelial tissue folding and separation.

Choroid Plexus Cysts (CPC)

Overview:

  • Common fetal ultrasound finding, usually benign.
  • Typically identified in the second trimester.
  • Prevalence: Found in up to 1% of pregnancies.

Clinical Characteristics:

  • Usually diagnosed between 16–24 weeks gestation.
  • Size typically <10 mm.
  • Majority spontaneously resolve by 24 weeks gestation.

Clinical Significance:

  • Isolated CPCs (no associated anomalies):
    • No increased risk for developmental or neurological impairment.
    • No significant long-term clinical implications.
  • Association with chromosomal anomalies:
    • Frequently seen in trisomy 18 (up to 50% of cases).
    • Rarely associated with trisomy 21.
  • Congenital infections:
    • Bilateral CPCs may rarely indicate congenital infections (e.g., CMV), especially if associated with subependymal germinolysis.

Prognosis and Management:

  • Generally excellent if isolated; no specific intervention needed.
  • Follow-up ultrasound recommended to confirm resolution.
  • Counseling regarding chromosome screening if additional markers or abnormalities present.

Choroid Plexus Papillomas

Definition:

  • Rare benign tumors arising from choroid plexus epithelium.
  • Typically found within lateral ventricles of fetus.

Clinical Presentation:

  • Usually presents with fetal hydrocephalus:
    • Due to excessive cerebrospinal fluid (CSF) production.
    • Potential obstruction of ventricular outflow foramina.

Imaging and Diagnosis:

  • Identified on prenatal ultrasound or fetal MRI.
  • Requires postnatal imaging confirmation and neurosurgical evaluation.

Prognosis and Management:

  • Management usually involves surgical resection after birth.
  • Prognosis favorable if timely intervention provided; complications primarily due to hydrocephalus.

Key Differential Points:

FeatureChoroid Plexus Cyst (CPC)Choroid Plexus Papilloma
Prevalence Common (~1%) Rare
Typical Size <10 mm Variable, can be large
Resolution Spontaneously resolves by ~24 weeks Does not resolve spontaneously
Association with Anomaly Trisomy 18, rarely trisomy 21 or CMV Hydrocephalus due to CSF production
Prognosis Excellent if isolated Good post-surgery, dependent on hydrocephalus management

Clinical Recommendations:

  • Isolated CPC: reassure parents, limited/no follow-up required.
  • Associated CPC findings (e.g., additional ultrasound anomalies):
    • Consider chromosome analysis.
    • Evaluate maternal infection status (CMV testing if indicated).
  • Choroid Plexus Papilloma: Refer for multidisciplinary prenatal and postnatal neurosurgical planning.