Corneal Pigmentations (PDF)

DDX pigmentations by the type of pigmentation ○ the material: is it melanin? Is it iron? DDX pigmentations by the location on the cornea ○ is it in the epithelium? Descemet’s? Stroma? Always know the use of meds by the patient that may cause pigmented deposits...

DDX pigmentations by the type of pigmentation ○ the material: is it melanin? Is it iron? DDX pigmentations by the location on the cornea ○ is it in the epithelium? Descemet’s? Stroma? Always know the use of meds by the patient that may cause pigmented deposits ○ Certain medications that have a tendency to cause corneal pigmentation R/O systemic metabolic diseases that’s cause corneal deposits ○ Systemic metabolic disease that creates some form of pigmentation in any layer Look for other signs of deposits in different ocular structures Melanin Pigmentation Can be deposited in anteriorly, stromal, or endothelium melanosis; basically anywhere A. Epithelial melanosis a. Congenital (overproduction of melanin and could be deposited anywhere) b. Benign primary epithelial melanocytic migration (BEPM or BEMM) i. Dark skinned people tend to have more melanin and in the conjunctiva and crosses it in the cornea and migrates to the cornea and is called BEPM or BEMM; invades the conjunctiva and is benign and flat (differentiate from melanoma when its not benign and elevated) ii. c. Melanomas i. d. Adrenochrome deposition i. Epinephrine (an adrenergic agonist that affects both alpha 1 and alpha 2 receptors - dilates the eye) ii. Chronic use of epinephrine oxidizes and leads to the formation of melanin iii. Very black melanin  iv. v. vi. B. Stromal melanosis a. Ochronosis in Alkaptonuria (Increased homogentisic acid- acid that is responsible for synthesis of melanin (too much acid = too much melanin) i. Systemic disease ^ and too much melanin production can go into tendons, skin and in the eye ii. Their skin looks like this  iii. Alkaptonuria in eye b. The accumulation of melanin production in the stroma (Ochronosis) C. Endothelial melanosis a. Congenital b. Senile i. older patients (late 70s-80s-90s) c. Degenerative and inflammatory conditions i. Which condition in the endothelial ? ii. Diabetes, HSV, HZV, Corneal guttata, Chronic glaucoma- all these conditions will create deposit of melanin in endothelium d. PDS/Pigmentary glaucoma (KS) i. leads to pigmentary glaucoma ii. too much pigment in the anterior chamber. Where does it come from? The iris is cóncave and touches the zonules - friction and the pigment from the iris gets released iii. Krukenberg's spindle- vertical line of pigment in the endothelium (specific formation of endothelial melanin that is present in pigmentary glaucoma) 1. e. Trauma i. Endothelial melanosis from trauma Hematogenous (BLOOD) pigmentation A. Epithelial a. Anemia (hemochromatosis) B. Stromal a. Interstitial keratitis i. formation in the abnormal blood vessels in stroma secondary to systemic diseases such as syphilis b. Traumatic hyphema (blood in anterior chamber) i. Endothelial blood staining becomes of blood in anterior chamber C. Endothelial a. Traumatic hyphema b. Subconjunctival hemorrhage that has stayed there for a long period of time Metallic pigmentation A. Iron pigment / Siderosis (NEED to have a corneal defect; can be compromised by dryness or FB) - accumulation of compromised corneal defect and the tears accumulate there (iron is an ingredient in the tears) and leaves the iron there and stains it B. a. FB iron Coat’s ring i. Foreign body falls in cornea and will oxide ii. 2 reasons: 1. ?  iii. b. Hudson Stahi line i. Horizontal greenish/brownish line that is below the cornea ii. Older ages; blinking process is decreased so that means the inferior cornea is being exposed to the air and that leads to dryness = inferior part of cornea is compromised = tears accumulate there iii. Inferior ⅓ cornea is exposed iv. c. Ferry line i. elevation in limbus; compromises the cornea; tears accumulate and same story d. Stocker’s line i. Ptergium = tip of pterygium indents the cornea and that area will compromise the area and same concept ii. e. Fleischer ring or line i. happens in keratoconus (base of the conus is thin and compromised so the tears accumulate there and blah blah blah)  ii. iii. f. RK iron line i. Silver (Argyrosis) 1. People who work with silver (silver surgical tools); deposits of silver in the eye 2. Even if you had surgery 3. Deposited Descemet’s, endothelium, even stroma 4. ii. Gold (Chrysiasis) 1. By occupation but also a substance that is being used for rheumatoid arthritis 2. Deposited Descemet’s, endothelium, even stroma 3.  4. iii. Copper (Chalcosis) 1. Developed by occupation also 2. In Descemet’s g. Kayser-Fleischer ring i. Wilson’s disease 1. A liver disease; also affects an enzyme that is called Ceruloplasmin enzyme. The enzyme that metabolizes copper in body, copper important for immune system and for bones, protection in infections but cannot have too much so the enzyme metabolizes the extra copper. The extra deposition of copper and can have many areas of copper and one of the organs that it will deposited in is the cornea and the ring is found in Descemet’s membrane. Endothelium copper disease. 2. Drug-induced pigmentations A. Vortex epitheliopathy/corneal verticillata a. Brown whorl like Opacities b. May cause halos of light other than that, does not affect vision c. ******** what medication will you suspect to see vortex epithelial? Antimalarial drugs such as hydroxychloroquine, chloroquine (Plaquenil) used for RA, and lupus, even Amiodarone (controls arrhythmias and HTN) d. ALWAYS r/o Systemic disease that can cause the same type of vortex epitheliopathy e. If patient does not take any of those medications, suspect that the patient has Fabry’s disease (systemic and ocular signs) - severe kidney and heart disease that is fatal ??? i. Chlorpromazine deposits 1. Anti-psychotic (depressants) medications 2. Brown deposits 3. Descemet’s & endothelium 4. ii. Epinephrine iii. Gold and silver f. g. Skin in pt w/ Fibry’s (tingling sensation in feet)  h. i. Corneal Congenital Anomalies (normal 11-12 mm) 1. Microcornea (< 10 mm) a. Inheritiance: autosomal dominant disease b. HIGH hyperopia; never going to see a myopia with microcornea c. Cornea may be flat d. AC: shallow; may get glaucoma specifically closed-angle type e. Lens: small lens (microspherophakia) i. may develop cataracts early f. Optic nerve head hypoplasia i. Normal ONH: Close to 1.45 mm ii. Small nerve head (ranges from 13 mm) a. Inheritance: X-linked (will only see in males) b. Refractive error: High myopes c. Cornea: Steep, get corneal dystrophies, d. Iris: Transillumination e. AC: very steep, may get glaucoma but open-angled type f. Lens: Cataracts and moves from its place (subluxation) g. What is the difference between megalocornea and buphthalmos in kids? ****** i. Megalocornea is a nonprogres­sive X-linked recessive con­genital anomaly in which the corneas are symmetrically enlarged to a diameter of at least 13 mm 1. megalocornea is associated with early cataract formation, glaucoma, and crystalline lens subluxation in adults 2. Long axial length ii. The key differential diagnosis of the pediatric enlarged cornea. Whereas megalocornea is an anomalous finding of minimal acute concern, buphthal­mos is caused by congenital glaucoma. iii. h. Systemic associations i. Alpert Syndrome: affects the eye and kidney disease and deafness ii. MS iii. EDS iv. DS (Down Syndrome- chromosomal defect) v. OI (Osteogenesis Imperfecta- bones break easily)  i. Management i. treat all complication ii. deal w RE j. 3. Sclero(hard)-cornea (opacified cornea) a. Mild form i. Type 1 ii. Just gets opacification ring in the peripheral cornea iii. Only in the periphery iv. Autosomal dominant v. b. Severe form i. Type 2 ii. Whole cornea is opacified and hard; autosomal recessive ** iii. Completely blind iv. c. Always flat corneas (plana) d. Blood vessels (neovascularization) e. Management i. Have to do transplant ** no other option 4. Corneal plana a. Corneal K’s: i. Type 1: ~36-37 to 40 (autosomal dominant)  ii. Type 2: K’s are 23 (autosomal recessive) b. Refractive error: high association to high hyperopia c. Corneal: flat cornea d. AC: shallow - narrow/closed-angle glaucoma **** must know the differences e. Systemic associations i. Sclerodermal 1. tough thick skin in body f. Management i. Transplant ii. Prognosis for PK: difficult to treat g. 5. Haab’s striae a. Descemet’s breaks b. Orientation: horizontal or oblique but never vertical (differentiate the lines from keratoconus that gives us vertical line) c. 3 main associations i. CG (Congential glaucoma) ii. Birth trauma iii. Acute hydros 1. Severe stromal edema 2. From advanced keratoconus = get rupture of Descemet’s membrane d. VA and progression of striae i. Does not progress* e. DDX PPCD (Posterior Polymorphous Corneal Dystrophy) i. Lines in endothelium in any formation w/ cystic formations  ii. PPCD; cysts present where Haab’s striae do not have that f. 6. Vogt striae a. Vertical folds at the level of posterior stroma and Descemet’s b. Seen in keratoconus c. Stress lines in DM so they disappear with pressure on the globe Iridocorneal dysgensis 1. Posterior embryotoxon a. Prominent Schwalbe’s line (termination of the Descemet's membrane at the limbal area) i. From posterior and moves anteriorly and becomes more visible at limbus level = white line b. Normal or associated w/ ocular syndrome i. Normal 10% in population and always finding in some syndromes such as Axenfeld’s or Reiger’s c. Inheritance: AD d. Management i. Do nothing about it; we just see it  e. PE f. g. 2. Axenfeld-Rieger Syndrome a. b. Open-angle glaucoma and is due to bad development of Schelmm’s canal and TM c. Axenfeld Anomaly i. Findings: 1. PE (Posterior embryotoxin)  2. Unconnected iris strands (going toward the cornea; important here is that there is no connection- no synechia) a. 3. No glaucoma d. Axenfeld syndrome i. WITH glaucoma e. Rieger Anomaly— eye only i. Findings 1. Iris hypoplesia: thin 2. PE 3. Pupil may be oval or displaced (corectopia) a. b. Pseudopolycoria 4. PAS (Peripheral anterior synechia) a. 5. Iris ectropion (Ectropion uveae)  a. Pigmented epithelium moves anteriorly and becomes visible b. c. 6. Glaucoma association (Narrow/closed angle glaucoma due to synechiae) f. Rieger syndrome— (eye + systemic disease) i. Maxillary facial anomalies ii. Short teeth iii. Hearing problems and cardiac problems iv. RA + extraocular malformations ^ g. Management 3. Peter’s Anomaly [like Peter Pan :) ] a. Inheritance: AD or AR b. Chromosomal defects reported c. Corneal opacity: MAIN characteristic is its dense, white opacity i. It varies in size but below the opacity is white and dense and EXTREMEEEE thinning of the DM in the endothelium (like a thread) d. Peters 1 (mild form) i. Will see adhesion from opacity to iris  ii. iii. e. Peters 2 (severe form) i. Will see adhesion from opacity to lens ii. f. Association to cornea: open (poor TM development) or closed (synechiae) angle g. Systemic associations i. FAS (Fetal Alcohol Syndrome) ii. CNS (brain disorders) iii. Heart disease iv. Hearing v. Peters Plus syndrome (they did ask this on boards) 1. Systemically are dwarfs + Mental retardation h. OCT of Peter’s Anomaly i.