lens

epicapsular stars

congenital melanocytes located in anterior lens capsule (represents minor embryological remnant of the tunica vasculosa lentis

tunica vasculosa lentis

temporary network of capillaries that nourish crystalline lens during fetal development

mittendorf dot

congenital circular opacity localized to posterior lens capsule (represents remnant of the anterior tunica vasculosa lentis where former hyaloid artery attached)

Bergmeister’s Papilla

congenital fibrovascular tissue located anteriorly to optic nerve head (represents remnant of the posterior tunica vasculosa lentis where former hyaloid artery attached)

lenticonus

focal teardrop bulge presenting secondary to defects in lens capsule formation (bilateral, males, Alport syndrome)

alport syndrome

genetically inherited condition w/ resultant compromise of basement membrane collagen biosynthesis (Xlinked)

posteriro lenticonus

unilateral, Lowe Syndrome

Lowe Syndrome (oculocerebrorenal syndrome)

• genetically inherited multisystem condition which results in 120 mutations

• males

• congenital cataracts, intellectual disability, renal tubular dysfunction

Lentiglobus

• generalized hemispherical deformity of the lens

• rare condition

• unilateral

• may be associated w/ posterior polar opacity

microspherophakia

small & spherical lens

Microphakia (spherophakia)

lens w/ smaller than normal diameter; may be found in isolation or w/ Lowe Syndrome

Ectopia lentis

• displacement of lens from normal position

• acquired/ hereditary

• luxated/ subluxated

• most common cause of heritable lens dislocation = Marfan Syndrome

Marfan Syndrome

• inherited connective tissue disorder most commonly caused by mutations in the FBN1 gene (negatively impacts fibrillin-1)

• autosomal dominant

• tall, thin stature, long limbs

• bilateral ectopia lentis (superotemporal subluxation)

homocystinuria syndrome

• inherited metabolic disorder where decreased enzymatic metabolism of amino acid methionine results in systemic accumulation of homocysteine & methionine

• autosomal recessive inheritance (variable expressivity)\

• bilateral ectopia lentis (inferonasal subluxation)

• presents by 25 years old

signs of ectopia lentis

• VA normal/decreased

• SLE: subluxated/ luxated, iridodenesis, phacodenesis, AC cells & flares

• increased IOP

phacodonesis

• vibration/ trembling of lens during eye movement

• indicative of weakness in zonular apparatus

treatment/ management of ectopia lentis

• primarily aimed at correcting induced RE

  • EG: mild subluxation

  • CL: aphakic cases, severe subluxation/ luxation

• surgical removal indicated when intractable ametropia, meridional amblyopia, cataract, lens-induced glaucoma, lens-induced uveitis, endothelial touch

• management of associated systemic conditions