Thinning and stretching of scleral-uveal layers of globe

Progressive myopia (nearsightedness) results in posterior staphyloma

Posterior staphyloma may also occur in glaucoma, connective tissue disorders, scleritis, necrotizing infection, or trauma

Anterior staphyloma may occur if infection or inflammation corneal thinning

Elongated globe, thinning of posterior wall, unilateral or bilateral

Congenital neurocutaneous syndrome

Mutation of gene on chromosome 17

Orbitofacial abnormalities in NF1 typically unilateral

Plexiform neurofibroma (PNF) diagnostic

Up to 50% with facial and eyelid involvement have ipsilateral glaucoma

Buphthalmos

Thickening of uveal and scleral layer; anterior rim enlargement

PNF orbit and skull base + sphenoid bony dysplasia enlargement of orbital foramina and middle cranial fossa herniation of intracranial contents into orbit pulsatile exophthalmos

± enlargement, tortuosity, and enhancement of optic nerve glioma

Leading cause of blindness in African-Americans and 2nd leading cause of blindness worldwide

> 4,000,000 Americans have glaucoma; only 1/2 know they have it

Risk factors: African-American, > 60 years, family member with glaucoma, Hispanic, Asian, high myopia, diabetes mellitus, hypertension, trauma

Primary open angle > > angle closure

Diagnosis by tonometry (measures intraocular pressure), ophthalmoscopy (may show evidence of optic nerve atrophy), visual field tests (lose peripheral vision 1st), or gonioscopy (measure angle between iris and cornea)

Less plasticity in globe of adults; therefore buphthalmos and deep anterior chamber uncommon

Incidence 1:5,000-1:10,000 live births; boys > > girls; bilateral in majority

Present at birth; usually diagnosed within 1st year of life

Clinical: Large eyes, excessive tearing, cloudy cornea, and light sensitivity

Obstruction to flow of aqueous humor from anterior chamber elevated intraocular pressure, enlargement of globe and deep anterior chamber

Normal mean anterior chamber depth: 3 mm; in congenital glaucoma, mean anterior chamber depth: 6.3 mm

Complications: Subluxated lens, optic nerve atrophy

Enlarged AP dimension of globe

Deep anterior chamber

Idiopathic globe enlargement (AP dimension) convergence of light anterior to retina (nearsightedness)