Eye

Orbital Cavernous malformation

• Basically hemangioma of the eye - but no true epithelial capsule and technically not a neoplasm

• >80% are intraconal, typically laterally located

• Presents with proptosis and can cause local compression - eye issues

• Surgical resection is curative

• Rarely hemorrhage (note venolymphatic malformations in kids do have hemorrhage)

Coats Disease (Retinal telangiectasias)

• Typically age 3-9

• M>F

• Affected eye is smaller than normal eye

• May see retinal detachment (V-shape)

• Unilateral

• Basically looks like a single messed up eye

Retinoblastoma

• 95% of cases diagnosed before age 5 yo

• Presents with leukocoria

• Calcified retinal lesions with increased vascularity on doppler

• Unilateral or bilateral (auto dominant inheritance typically for bilateral disease)

• Trilateral Rb

  • Bilateral Rb (each eye) + Pineoblastoma

• Quadrilateral Rb

  • Trilateral + suprasellar embryonal CNS tumor + osteosarcoma

• Mets

  • via Optic nerve cuasing leptomeningeal enhancement

  • also to bone

Tolosa Hunt Syndrome

Angiolymphatic malformation

• Multiloculated cystic and solid mass

• Possible adjacent osseous remodeling secondary to persistent compression on bone

• Really just looks like some fuck shit behind the eye

• Fluid-fluid levels (Rb does not have)

• Less commonly have calcs (Rb has calcs)

• Extra and intraconal

• Proptosis and swelling (Rb may have some of this but leukocoria and vision loss is key)

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