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Eye and Orbit

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Conjunctiva:
 External infections: Trachoma (Chlamydia trachomatis) remains the leading cause of
preventable blindness in the world: some 85 mio people have active trachoma (infective stage)
and 7.8 mio people have blinding stage. In AA conjunctivitis caused by staph. aureus, str.
pneumoniae, coagulase-negative staphylococci is prevalent.

 Squamous cell carcinoma: most arise from actinic keratosis in the interpalpebral fissure of
the limbal conjunctiva and grow slowly in an exophytic, sometimes papillary manner. Neglected
tumors invade into the eye and posteriorly into the orbit.

Retina:
 Retinoblastoma:
- the most common eye tumor in childhood
- molecular genetics: the result of a single gene mutation [Rb gene, chromosome 13, tumor
suppressor gene]
- hereditary form: germline mutation
- non-hereditary form: initial mutation in a somatic retinal cell
 in both: the loss of both allelic wild-type copies of the Rb gene is the key step in malignant
transformation
- he retina is a derivative of the brain  retinoblastoma is an example of a poorly differentiated
(embryonal) neoplasia
- dissemination through choroid vessels or/and through optic nerve
- advanced: even through the sclera  orbit  preauaricular lymph nodes
- metastasis: CNS, skull, other bones, lymph nodes

Uvea:
 Uvea includes: iris, ciliary body, choroid

 Uveitis:
o in HIV/AIDS: pneumocystis carinii, toxoplasma Gondii, CMV
o systemic: sarcoidosis
o autoimmune: sympathetic ophthalmia

 Uveal melanoma:
- rare, but the most common primary intraocular maligant tumor in adults
- 8-fold risk for white race compared with blacks
- the tumor arises from melanocytes located in these structures (not from the pigment epithel of
retina, iris and ciliary body)
- spread: hematogenous and preferentially to the liver

Orbit:
- in general inflammatory diseases are more common than primary tumors (infections spreading
from the surrounding sinuses [mucor, diabetes])
- most lesions increase the volume of the orbital content  exophthalmus, proptosis

 Thyroid ophthalmia (Graves orbitopathy)


- is the most common cause of exophthalmus (more than 50% in adults)
- autoimmune-based inflammation (inflammatory infiltrate restricted to the muscle bellies of extracellular
muscle consisting of lymphocytes, plasma cells, scattered mast cells)

 Rhabdomyosarcoma
- the most common primary malignant orbital tumor in childhood (mean age: 7 years)

 Lymphoid tumors: non-Hodgkin lymphoma


- most: low-grade B-cell lymphomas
- older patients (rare in children)

 Optic nerve glioma


- nearly always low-grade astrocytoma
- first decades, 50% have neurofibromatosis type I

 Optic nerve meningioma

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