lecture 5 Flashcards
(47 cards)
marfans syndrome
• Most common systemic disease
associated with lens dislocation
• Ocular, Cardiovascular and musculoskeletal abnormities
subluxated dislocated lenses
marfans
homocystinuria
- Rare, autosomal recessive
- Usually between 3-10 yrs old
- Lens subluxation usually symmetrical
homocystinuria
Result of incomplete closure of the embryonic fissure
Usually inferior nasal, forming a “keyhole” pupil
May be associated with coloboma of other parts of the eye (e.g. lid, optic nerve)
iris coloboma
Aniridia (see photos)
Albinism
Uveitis
– Traumatic anterior uveitis is most likely to cause photophobia
uvea
Keratitis
Corneal foreign body Corneal abrasion Corneal scarring Corneal dystrophies
cornea
– Aniridia
– Anterior Chamber Cleavage Syndrome
– Sturge-Weber
– Neurofibromatosis
associated congential conditions of glaucoma
treatment for congenital glaucoma
– Primarily Surgical
– REFER TO PEDIATRIC GLAUCOMA SPECIALIST!
– Optic neuritis
– Vitritis/endophthalmitis
– Congenital cone dystrophies (associated poor vision and nystagmus)
posterior segment
other causes of photophobia
posterior segment
Intermittent XT
Migraine
Fair skinned individuals
Nasolacrimal duct obstruction – most common in
causes of epiphora
- Tearing that occurs only when the patient salivates
- Lesion of the 7th cranial nerve, causing facial paralysis and profuse lacrimation during eating
- AssociatedwithDuane’ssyndrome
crocodile tears syndrome
Similar to capillary hemangioma, but lymphoid in nature
Usually involves the orbit, can affect lid and conjunctiva
No spontaneous regression
Surgery needed if vision threatened due to optic nerve compression, corneal exposure or amblyopia
lymphangioma
Usually before 20 yrs old, 75%
optic nerve glioma
Arise from adrenal medulla
Most common solid tumor of childhood, accounting for 10- 15% of pediatric cancer
Frequent orbital metastases
90%
neuroblastoma
Can effect any part of eye:
Orbital infiltration of bone or soft tissue
Conjunctiva, iris infiltration Cataracts after treatment Subchoroidal mass, RD
Retinal & Vitreous • Hyperviscousity
• Retinal hemorrhages • Roth spots
leukemia
other causes of bulging
Capillary Hemangioma Orbital Cellulitis Dermoid Cyst Rhabdomyosarcoma
• Mostfrequentorbitalmalignancy in children
Corneal diameter > 11.5-12 mm in infant or > 13 mm in fully grown eye
Three patterns:
• Simplemegalocornea: unassociated with other ocular anomalies, autosomal dominant
• X-linked megalocornea (Anterior Megalophthalmos): recessive, associated with iris and angle abnormalities, lens subluxation and early cataract
• Buphthalmos: infantile glaucoma
megalocornea
Micropthalmos – Associations • PHPV • Congenital cataracts • Toxoplasmosis • Rubella • Coloboma • High hyperopia Anophthalmos • Primary optic vesicle fail to grow out from the cerebral vesicle Megalophthalmos
orbital size abnormalities
The most common corneal degeneration in early adolescent Bilateral, non-inflammatory 10% patients have FHx Association with other diseases: – Down’s syndrome – Floppy eyelid syndrome – Leber’s congenital amaurosis – Retinitis pigmentosa – Atopic disease: rubbing eye excessively
keratoconus
Inherited disorder of copper metabolism, copper deposit in liver, brainstem and eye
Copper deposits on corneal Descemet’s membrane
Kayser-Fleischer ring Most prominent inferiorly
and superiorly
Rarely, sunflower cataracts – yellowish, star-like, anterior subcapsular discoloration
wilsons disease
Severe Vitamin A deficiency, causing severe dry eye
One of most common causes of blindness in the world – Corneal scarring
– More common in undernourished populations
Night blindness is earliest symptom
xerophthalmia
Bilateral chronic follicular conjunctivitis
Leading cause of blindness in the developing country Conjunctival and corneal scaring
“SAFE” strategy promoted by WHO
– Surgery to correct advanced stages of the disease;
– Antibiotics to treat active infection, using Zithromax (azithromycin) donated by Pfizer Inc;
– Facial cleanliness to reduce disease transmission;
– Environmental change to increase access to clean water and improved sanitation.
trachoma
Dermoid
Vernal keratoconjunctivitis Trauma/chemical burn Keratitis
Foreign body
Congenital glaucoma
other causes of corneal opacities