Corneal Pigmentation Quiz
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Questions and Answers

What causes the overproduction of melanin in epithelial melanosis?

  • Environmental factors
  • Congenital conditions (correct)
  • Genetic mutations
  • Infectious diseases

Which systemic metabolic disease is associated with increased melanin production due to excess homogentisic acid?

  • Diabetes Mellitus
  • Wilson's Disease
  • Cystic Fibrosis
  • Ochronosis in Alkaptonuria (correct)

Which medication is known to cause the formation of melanin deposits in the cornea due to chronic use?

  • Antibiotics
  • Epinephrine (correct)
  • Corticosteroids
  • Beta-blockers

In which ocular condition might melanin deposits occur in the endothelium?

<p>Pigmentary glaucoma (D)</p> Signup and view all the answers

What differentiates benign primary epithelial melanocytic migration (BEPM) from malignant melanoma?

<p>BEPM is benign and flat (C)</p> Signup and view all the answers

Which age group is most likely to exhibit endothelial melanosis due to senile degeneration?

<p>Older patients (D)</p> Signup and view all the answers

What is a potential source of melanin deposition in the stroma?

<p>Ochronosis associated with alkaptonuria (C)</p> Signup and view all the answers

What condition is associated with a vertical line of pigment in the endothelium?

<p>Krukenberg's spindle (B)</p> Signup and view all the answers

What is one potential source of too much pigment in the anterior chamber?

<p>Friction between the iris and zonules (C)</p> Signup and view all the answers

What condition leads to endothelial melanosis from trauma?

<p>Traumatic hyphema (B)</p> Signup and view all the answers

What type of pigmentation occurs when iron accumulates in the cornea?

<p>Metallic pigmentation (B)</p> Signup and view all the answers

Which line is characterized by an accumulation of tears due to corneal dryness in older individuals?

<p>Hudson-Stahi line (D)</p> Signup and view all the answers

What can cause traumatic blood staining in the endothelium?

<p>Subconjunctival hemorrhage (A)</p> Signup and view all the answers

What does the term 'Stocker’s line' refer to?

<p>A line appearing due to pterygium on the cornea (B)</p> Signup and view all the answers

Which pigmentation occurs as a result of a corneal defect and the presence of foreign bodies?

<p>FB iron Coat’s ring (A)</p> Signup and view all the answers

What is the physiological consequence of a corneal defect causing iron accumulation?

<p>Staining of corneal tissue (C)</p> Signup and view all the answers

What is the primary characteristic of Haab’s striae?

<p>They consist of horizontal or oblique lines. (B)</p> Signup and view all the answers

Which conditions are associated with Haab’s striae?

<p>Acute hydros and congenital glaucoma. (A)</p> Signup and view all the answers

What is the treatment protocol for iridocorneal dysgenesis?

<p>Observation is typically sufficient. (B)</p> Signup and view all the answers

Which feature is characteristic of Rieger Anomaly?

<p>Iris hypoplasia and corectopia. (D)</p> Signup and view all the answers

What differentiates Haab’s striae from Posterior Polymorphous Corneal Dystrophy (PPCD)?

<p>Haab’s striae do not have cystic formations. (C)</p> Signup and view all the answers

Which disease is characterized by a copper deposition leading to a Kayser-Fleischer ring?

<p>Wilson’s disease (C)</p> Signup and view all the answers

What is the primary source of vortex epitheliopathy/corneal verticillata?

<p>Antimalarial drugs (B)</p> Signup and view all the answers

Which of the following is NOT a characteristic of microcornea?

<p>Common in myopia (A)</p> Signup and view all the answers

Which substance is indicated by the presence of brown deposits in the cornea due to chlorpromazine?

<p>Antipsychotic medications (B)</p> Signup and view all the answers

What enzyme is affected by Wilson's disease, leading to copper metabolism issues?

<p>Ceruloplasmin (B)</p> Signup and view all the answers

Which condition is potentially fatal and is associated with ocular signs along with severe kidney and heart disease?

<p>Fabry's disease (A)</p> Signup and view all the answers

What type of glaucoma is specifically associated with microcornea?

<p>Closed-angle glaucoma (C)</p> Signup and view all the answers

Which of the following is NOT a consequence of excessive copper deposition?

<p>Vortex epitheliopathy (D)</p> Signup and view all the answers

Which medication is likely to cause vortex epitheliopathy?

<p>Hydroxychloroquine (C)</p> Signup and view all the answers

Which disease is characterized by brown whorl-like opacities that may cause halos of light?

<p>Vortex epitheliopathy (B)</p> Signup and view all the answers

What is a characteristic feature of megalocornea?

<p>Corneas are symmetrically enlarged to a diameter of at least 13 mm (B)</p> Signup and view all the answers

Megalocornea is classified under which type of inheritance?

<p>X-linked recessive (B)</p> Signup and view all the answers

What distinguishes corneal plana from other corneal conditions?

<p>Flat corneas with a shallow anterior chamber (C)</p> Signup and view all the answers

Which of the following symptoms is associated with optic nerve head hypoplasia?

<p>Normal optic nerve head size of about 1.45 mm (A)</p> Signup and view all the answers

Which type of corneal scleral condition always leads to blindness?

<p>Type 2 scleral condition (A)</p> Signup and view all the answers

What is the primary management strategy for corneal scleral conditions?

<p>Corneal transplant as the only option (A)</p> Signup and view all the answers

Which condition can lead to the development of open-angle glaucoma?

<p>Steep anterior chamber (C)</p> Signup and view all the answers

What is a typical refractive error associated with corneal plana?

<p>High hyperopia (C)</p> Signup and view all the answers

Which condition is primarily caused by congenital glaucoma?

<p>Buphthalmos (B)</p> Signup and view all the answers

Flashcards

Epithelial Melanosis

Melanin deposits in the cornea's epithelium, sometimes congenital (present at birth).

BEPM/BEMM

Benign condition in dark-skinned individuals where melanin migrates from the conjunctiva to the cornea's epithelium.

Stromal Melanosis

Melanin deposition in the cornea's stroma, often linked to systemic diseases like Alkaptonuria.

Alkaptonuria

A genetic disorder causing excess homogentisic acid, leading to melanin deposits (ochronosis) in tissues including the cornea.

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Endothelial Melanosis

Rare, melanin deposits in the cornea's endothelium, possibly related to aging, or inflammatory or degenerative diseases.

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Pigmentary Glaucoma (KS)

Condition where melanin deposits can lead to increased eye pressure.

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Adrenochrome deposition

Dark melanin deposits in the cornea due to chronic epinephrine use.

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Pigmentary glaucoma

A type of glaucoma caused by excessive pigment in the anterior chamber.

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Krukenberg's spindle

A vertical line of pigment in the eye's endothelium, seen in pigmentary glaucoma.

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Hematogenous pigmentation

Pigmentation in the eye from blood components (e.g., anemia).

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Metallic pigmentation

Eye pigmentation from metals like iron and silver.

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Siderosis

Iron pigmentation in the cornea, often due to a corneal defect.

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Coat's ring

A ring of iron oxide, foreign object in cornea.

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Hudson's/Stahl's line

Greenish/brown line below cornea due to inferior corneal dryness.

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Fleischer ring

Iron ring in keratoconus (weakening of cornea).

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What is megalocornea?

A condition where the cornea is larger than normal from birth (congenital). It's a non-progressive anomaly, meaning it doesn't get worse over time.

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What is buphthalmos?

A condition characterized by enlarged corneas caused by congenital glaucoma. It can lead to vision loss if untreated.

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What is the difference between megalocornea and buphthalmos?

Megalocornea is a harmless, birth defect with a larger cornea. Buphthalmos is a serious condition caused by glaucoma, where the cornea bulges due to increased eye pressure.

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Sclero-cornea

A condition where the cornea becomes opaque (cloudy) and hard.

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Type 1 Sclero-cornea

A milder form of sclero-cornea where only the peripheral cornea becomes opaque. It's an autosomal dominant condition, meaning it can be passed down from a parent.

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Type 2 Sclero-cornea

A severe form of sclero-cornea affecting the entire cornea, rendering it opaque and hard. This form is autosomal recessive, meaning both parents need to carry the gene for the child to be affected.

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Corneal Plana

A condition where the cornea is unusually flat.

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Type 1 Corneal Plana

A milder form of corneal plana with steeper corneal curvature (K readings) - ranging from 36-37 to 40. It's an autosomal dominant condition.

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Type 2 Corneal Plana

A more severe form of corneal plana with very flat corneal curvature (K readings) - around 23. This form is autosomal recessive.

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What is the difference between corneal plana Type 1 and Type 2?

Type 1 is milder, with higher corneal curvature and an autosomal dominant inheritance pattern. Type 2 is more severe, with very flat corneal curvature and an autosomal recessive inheritance pattern.

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Haab's striae

Horizontal or oblique breaks in Descemet's membrane, often associated with congenital glaucoma, birth trauma, or acute hydrops.

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Vogt striae

Vertical folds in the posterior stroma and Descemet's membrane, typically seen in keratoconus, and can be stress lines in diabetes.

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Posterior Embryotoxon

A prominent Schwalbe's line (termination of Descemet's membrane) at the limbus, seen in 10% of the population and associated with some syndromes.

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Axenfeld Anomaly

A finding in Axenfeld-Rieger syndrome characterized by posterior embryotoxon and unconnected iris strands towards the cornea, without synechia (adhesion).

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Rieger Anomaly

A less severe form of Axenfeld-Rieger syndrome affecting only the eye, with findings such as iris hypoplasia, posterior embryotoxon, and abnormal pupil shape.

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Chrysiasis

Gold deposition in the cornea, often due to occupational exposure or rheumatoid arthritis treatment. Affects Descemet's membrane, endothelium, and even stroma.

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Chalcosis

Copper deposition in the cornea, primarily in Descemet's membrane. Often develops due to occupational exposure.

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Kayser-Fleischer Ring

A copper ring found in Descemet's membrane, a hallmark of Wilson's disease. This liver disease affects ceruloplasmin, the enzyme responsible for copper metabolism.

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Vortex Epitheliopathy

Brown, whorl-like opacities in the cornea, often caused by antimalarial drugs like hydroxychloroquine, chloroquine, or amiodarone. Can also be a sign of Fabry's disease.

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Chlorpromazine Deposits

Brown deposits in Descemet's membrane and endothelium, caused by the antipsychotic medication chlorpromazine.

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Microcornea

A smaller than normal cornea, less than 10mm in diameter. Inherited as an autosomal dominant trait. Patients are typically hyperopic and prone to shallow anterior chambers, increasing the risk of closed-angle glaucoma.

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Fabry's Disease

A rare genetic disorder that can cause vortex epitheliopathy in the cornea, along with severe kidney and heart disease.

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Corneal Congenital Anomalies

Birth defects of the cornea, such as microcornea, which is a smaller than normal cornea.

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Inheritance of Microcornea

Microcornea is inherited as an autosomal dominant trait. This means that if one parent has microcornea, there is a higher chance that their children will also have it.

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Hyperopia and Microcornea

Patients with microcornea are typically hyperopic, meaning they have difficulty seeing near objects. This is because their cornea is too flat and light rays focus behind the retina.

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Study Notes

Corneal Pigmentation

  • Pigmentation can be found in different layers of the cornea (epithelium, Descemet's, stroma)
  • Melanin and iron are common causes of corneal pigmentation
  • Certain medications can cause deposits
  • Systemic metabolic diseases can cause pigmented deposits
  • Always consider the patient's medication history when evaluating corneal pigmentation

Melanin Pigmentation

  • Melanin can deposit in anterior, stromal, or endothelial layers; any corneal location
  • Epithelial Melanosis:
    • Congenital: excessive melanin production, potentially anywhere
    • Benign Primary Epithelial Melanocytic Migration (BEPM/BEMM): common in dark-skinned individuals, benign, migratory melanin cells
    • Differentiation from melanoma involves looking for elevated lesions
  • Melanomas: cancerous
  • Adrenochrome:
    • Epinephrine can oxidize and form melanin
    • Chronic epinephrine use can lead to melanin formation
    • Very darkly colored melanin

Stromal Melanosis

  • Ochronosis (Alkaptonuria):
    • Increased homogentisic acid leads to melanin production
    • Systemic disease causing excessive melanin deposition in tendons, skin, and eyes
    • Characterized by a brownish-purple discoloration of the skin

Endothelial Melanosis

  • Congenital: present at birth
  • Senile: common in older individuals (late 70s-80s)
  • Development or inflammation can contribute to melanin build-up
    • Systemic diseases (diabetes, herpes, etc.)
    • Pigmentary glaucoma
    • Trauma

Hematogenous (Blood) Pigmentation

  • Epithelial: anemia (hemochromatosis)
  • Stromal: interstitial keratitis (abnormal blood vessels secondary to systemic diseases like syphilis), Traumatic hyphema (blood in the anterior chamber)
  • Endothelial: traumatic hyphema, subconjunctival hemorrhages (blood beneath the conjunctiva)
  • Metallic pigment/Siderosis: corneal defect/dryness allows iron accumulation (iron in tears stains the cornea)

Other Pigmentation Considerations

  • Foreign body (FB) iron coat's ring: oxidized iron deposits
  • Hudson Stahi line: greenish-brownish line below cornea (inferior parts of cornea exposed to air leads to dryness)
  • Ferry line: limbal elevation increasing corneal tears and accumulation
  • Stocker's line: pterygium-associated indentation in the cornea
  • Fleischer ring or line: thinning at the base of the keratoconus, tears accumulate
  • RK iron line: silver deposits (e.g., surgical tools), associated with surgery
  • Gold (Chrysiasis): gold deposits, associated with rheumatoid arthritis

Drug-Induced Pigmentations

  • Vortex epitheliopathy/corneal verticillata: whorl-like brown opacities, associated with some medications (anti-malarials, etc.) and systemic diseases

  • Copper (Chalcosis), Kayser-Fleischer ring: Wilson's disease (liver disease)

  • Chlorpromazine: brown deposits in Descemet's and endothelium layer

Corneal Anomalies

  • Microcornea: cornea diameter less than 10mm
  • Megalocornea: cornea diameter greater than 13mm, usually in males (X-linked)
  • Sclero(hard)-cornea: opacified cornea, mild to severe forms.
    • Type 1 is autosomal dominant, just peripheral
    • Type 2 is autosomal recessive, affects the whole cornea, causes blindness
  • Corneal plana: corneas are always flat
  • Haab's striae: vertical or horizontal lines in Descemet's membrane, commonly seen in congenital glaucoma and rupture of Descemet's because of trauma or keratoconus
  • Vogt striae: vertical folds in the posteriors stroma and Descemet's, associated with keratoconus
  • Iridocorneal dysgenesis (ICD):
    • Posterior embryotoxon: prominent Schwalbe's line
    • Axenfeld-Rieger syndrome: iris hypoplasia, Open-angle glaucoma

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Corneal Pigmentations PDF

Description

Test your knowledge on corneal pigmentation, including melanin deposits and their implications. Learn about the various layers of the cornea, the role of medications, and systemic diseases that can lead to pigmentation. This quiz is essential for understanding corneal health and diagnostics.

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