Ophthalmology: Cataracts and Systemic Diseases

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Questions and Answers

A patient presents with green "sunflower" cataracts. Which systemic disease is MOST likely associated with this finding?

  • Wilson's disease (correct)
  • Neurofibromatosis
  • Down's syndrome
  • Myotonic dystrophy

Which systemic condition is frequently linked to the development of "Christmas tree cataracts"?

  • Neurofibromatosis
  • Myotonic dystrophy (correct)
  • Down's syndrome
  • Atopic dermatitis

Shield-like, dense anterior subcapsular plaques are MOST indicative of cataracts related to which systemic condition?

  • Wilson's disease
  • Atopic dermatitis (correct)
  • Neurofibromatosis
  • Down's syndrome

A patient is diagnosed with posterior subcapsular cataracts. Which systemic disease should be MOST strongly considered as a potential underlying cause?

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

What is the MOST prevalent cause of congenital cataracts?

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

Congenital cataracts are found bilaterally in approximately what proportion of cases?

<p>2/3 (D)</p> Signup and view all the answers

Cataract progression typically induces a refractive shift toward which type of astigmatism?

<p>Against-the-rule astigmatism (C)</p> Signup and view all the answers

If a patient reports that a beam of light appears bent or distorted, but maintains uniform thickness, which macular condition is MOST likely present?

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

What is the underlying cause of ocular complications in Pseudoxanthoma Elasticum (PXE)?

<p>Calcification of elastic fibers. (C)</p> Signup and view all the answers

A patient is diagnosed with Gronblad-Strandberg syndrome. Which of the following clinical findings would be MOST indicative of this diagnosis?

<p>Angioid streaks and loose skin folds (B)</p> Signup and view all the answers

Why might Ehlers-Danlos syndrome increase a patient's risk of retinal detachment?

<p>Because it affects collagen, impacting the structural integrity of the eye. (D)</p> Signup and view all the answers

In late stages of a certain condition, significant vision loss can occur due to optic nerve compression. Which lab findings are MOST likely associated with this?

<p>Increased serum alkaline phosphatase and urine calcium levels. (A)</p> Signup and view all the answers

Which of the following is the MOST likely systemic manifestation in a patient presenting with angioid streaks who is also diagnosed with Paget's disease?

<p>Enlarged skull (D)</p> Signup and view all the answers

A patient with angioid streaks is being evaluated for associated systemic conditions. What is the MOST critical cardiovascular risk to assess due to the potential association with Pseudoxanthoma Elasticum (PXE)?

<p>Accelerated atherosclerosis (A)</p> Signup and view all the answers

A 49-year-old patient recently started taking oral tamoxifen. Besides an annual comprehensive dilated eye exam, what additional test should be performed at each visit to monitor for potential ocular side effects?

<p>Macular optical coherence tomography (OCT) (D)</p> Signup and view all the answers

A patient taking tamoxifen exhibits decreased visual acuity. Where are white crystalline retinal deposits MOST likely concentrated?

<p>Confined to the plexiform and nerve fiber layers, densest around the paramacular region. (B)</p> Signup and view all the answers

A 35-year-old patient presents with angioid streaks and a family history of cardiovascular issues. Which genetic testing strategy would be MOST appropriate to initially investigate the underlying cause?

<p>Targeted sequencing for ABCC6 gene mutations. (C)</p> Signup and view all the answers

A 60-year-old patient with known Paget's disease presents with new-onset angioid streaks. Which of the following management strategies should be prioritized in addition to ophthalmological care?

<p>Initiation of bisphosphonate therapy and audiometry (B)</p> Signup and view all the answers

What is the MOST common triad of clinical findings in retinitis pigmentosa (RP)?

<p>Retinal bone-spicule pigmentation, arteriolar attenuation , and optic disc pallor. (D)</p> Signup and view all the answers

What ocular side effect is MOST likely to occur in a patient taking high doses (exceeding 120mg daily) of tamoxifen?

<p>Development of full-thickness macular holes. (C)</p> Signup and view all the answers

Besides bone-spicule pigmentation, which retinal vascular change is MOST commonly observed in patients with retinitis pigmentosa?

<p>Retinal arteriolar attenuation (B)</p> Signup and view all the answers

Which systemic condition is LEAST likely to be associated with the formation of angioid streaks?

<p>Type 1 Diabetes (C)</p> Signup and view all the answers

A patient with retinitis pigmentosa is being evaluated. While bone-spicule pigmentation and arteriolar attenuation are noted, which additional optic nerve finding completes the classic triad of this condition?

<p>Optic disc pallor (B)</p> Signup and view all the answers

Which characteristic is indicative of dry age-related macular degeneration (ARMD) rather than wet ARMD?

<p>Drusenoid pigment epithelial detachment (A)</p> Signup and view all the answers

Besides glial cell processes, which of the following cellular components contributes to the formation of the internal limiting membrane of the retina?

<p>Muller cells (A)</p> Signup and view all the answers

At what distance from the foveal pit is the peak density of rod photoreceptors located in the retina?

<p>4.5 mm from the foveal pit (D)</p> Signup and view all the answers

Which molecule functions as a second messenger in the retina and plays a crucial role in the phototransduction cascade?

<p>Cyclic GMP (cGMP) (D)</p> Signup and view all the answers

What is the primary role of retinal pigment epithelium (RPE) in maintaining photoreceptor health and function?

<p>Phagocytosing shed outer segments of photoreceptors (D)</p> Signup and view all the answers

A patient presents with constricted visual fields and a 'bone-spicule' pigmentation pattern on fundus examination. Which of the following is the most likely underlying cellular mechanism contributing to this condition?

<p>Progressive apoptosis of rod photoreceptors (C)</p> Signup and view all the answers

In the context of retinal neurotransmission, what distinguishes ionotropic glutamate receptors from metabotropic glutamate receptors in bipolar cells?

<p>Ionotropic receptors mediate fast, transient postsynaptic responses via ion channels, whereas metabotropic receptors trigger slower, sustained effects through second messenger cascades. (D)</p> Signup and view all the answers

Which of the following best describes the role of amacrine cells in retinal processing?

<p>Modulating the output of bipolar cells and influencing ganglion cell activity through lateral interactions (A)</p> Signup and view all the answers

In a severe, untreated case of Neisseria gonorrhoeae conjunctivitis, which of the following is the most likely progression leading to significant vision loss?

<p>Epithelial invasion leading to peripheral corneal ulceration, followed by central extension, corneal perforation, and potential endophthalmitis. (C)</p> Signup and view all the answers

A 30-year-old patient presents with acute, unilateral conjunctivitis and mucopurulent discharge. Gram staining reveals Gram-negative, kidney-shaped diplococci. While awaiting culture results, which of the following is the MOST appropriate initial treatment strategy?

<p>Initiate topical broad-spectrum antibiotics, such as fluoroquinolones, while emphasizing strict hygiene to prevent spread. (A)</p> Signup and view all the answers

A 4-year-old child presents with acute conjunctivitis. Knowing that Haemophilus influenzae is commonly implicated in this age group, what specific clinical feature would MOST strongly suggest this etiology over other bacterial causes?

<p>Concurrent upper respiratory infection symptoms, such as otitis media or sinusitis. (A)</p> Signup and view all the answers

In differentiating between bacterial and viral causes of acute conjunctivitis, which clinical sign is LEAST likely to be associated with a bacterial infection?

<p>Subepithelial infiltrates on the cornea. (A)</p> Signup and view all the answers

What is the underlying mechanism by which trachoma leads to blindness if left untreated?

<p>Progressive inflammation and scarring of the conjunctiva, leading to entropion, trichiasis, and subsequent corneal damage. (A)</p> Signup and view all the answers

A patient from a lesser-developed country presents with Arlt’s lines and Herbert’s pits. What is the MOST likely long-term complication if this condition is left untreated?

<p>Corneal blindness from trichiasis and subsequent ulceration. (A)</p> Signup and view all the answers

When a patient is diagnosed with trachoma, in addition to antibiotics, what other intervention is MOST critical in preventing further vision loss?

<p>Eyelid surgery to correct entropion and trichiasis. (C)</p> Signup and view all the answers

In a patient with suspected trachoma presenting with mucopurulent discharge and superior tarsal follicles, what is the MOST appropriate method to confirm the diagnosis in a resource-limited setting?

<p>Giemsa staining of conjunctival scrapings to visualize intracytoplasmic inclusions. (C)</p> Signup and view all the answers

A patient presents with keratometry values in the 62-66D range. Which of the following adjustments should be made to accurately measure their corneal curvature using a manual keratometer?

<p>Add a +2.25D trial lens and add 16D to the drum reading. (C)</p> Signup and view all the answers

A patient's keratometry readings are significantly flatter than the lower limit of your manual keratometer. To obtain an accurate measurement, which trial lens should be used and how should the reading be adjusted?

<p>A -1.00 lens should be added, and 6D should be subtracted from the drum reading. (C)</p> Signup and view all the answers

Which of the following is the MOST accurate description of the relationship between corneal thinning and protrusion in pellucid marginal degeneration (PMD)?

<p>The area of corneal protrusion is located superior to the area of corneal thinning. (C)</p> Signup and view all the answers

A patient is diagnosed with pellucid marginal degeneration (PMD). Which finding would be LEAST likely in this patient?

<p>Central corneal scarring (D)</p> Signup and view all the answers

What topographic pattern is MOST indicative of pellucid marginal degeneration (PMD)?

<p>A 'crab claw' or 'kissing doves' pattern. (C)</p> Signup and view all the answers

In the management of corneal disease, when would a bandage contact lens be LEAST appropriate?

<p>To address mild discomfort associated with Arcus senilis. (B)</p> Signup and view all the answers

Why are bandage contact lenses used in the management of recurrent corneal erosion?

<p>To provide a barrier against eyelid friction, promoting epithelial healing. (A)</p> Signup and view all the answers

Following photorefractive keratectomy (PRK) surgery, what is the PRIMARY reason for using a bandage contact lens?

<p>To provide comfort and facilitate corneal epithelial healing. (D)</p> Signup and view all the answers

Flashcards

Myotonic dystrophy

A genetic disorder often causing cataracts known as 'Christmas tree cataracts'.

Atopic dermatitis

A skin condition associated with shield-like anterior subcapsular cataracts.

Neurofibromatosis

A disorder linked to posterior subcapsular or cortical cataracts.

Wilson's disease

A genetic disorder linked with green 'sunflower' cataracts.

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Congenital cataracts

Cataracts present at birth, occurring in 1 in 3,000 live births.

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Most common cause of congenital cataracts

Genetic mutation is the most prevalent cause, accounting for ~25%.

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Against-the-rule astigmatism

A shift caused by cataracts, steepening the lens horizontally.

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Diabetic related ocular changes

Fluctuating vision due to poorly controlled diabetes leading to ocular issues.

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Wet ARMD Indicators

Signs associated with wet age-related macular degeneration include CNVM and hemorrhage.

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Dry ARMD Sign

Drusenoid pigment epithelial detachment is characteristic of dry ARMD.

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Internal Limiting Membrane

Composed of Muller cell processes and other glial cells.

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Muller Cells

These cells extend throughout the retina and maintain its architecture.

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Rod Photoreceptors Peak Density

Peak density of rod photoreceptors is found 4.5 mm from the foveal pit.

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Cone Photoreceptors

Only cones are present in the fovea, forming a hexagonal pattern.

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Phototransduction Cascade

A process involving second messengers in retinal signal transduction.

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Rod Ring

The area surrounding the fovea with the highest density of rod photoreceptors.

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Full thickness macular hole

A retinal condition where the macula is completely disrupted, causing distorted vision.

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Lamellar hole

A partial thickness retinal hole that does not affect the entire macula, leading to visual distortion.

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Pseudoxanthoma elasticum

A connective tissue disorder associated with angioid streaks in the retina, characterized by skin changes.

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Angioid streaks

Bends or breaks in the retina's blood vessels often seen in patients with systemic diseases.

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Gronblad-Strandberg syndrome

The combination of Pseudoxanthoma elasticum and ocular involvement with angioid streaks.

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Ehlers-Danlos syndrome

A genetic disorder affecting collagen, possibly leading to angioid streaks and ocular problems.

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Paget's disease

A chronic disease affecting bone remodeling, associated with angioid streaks in some patients.

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Ocular complications of PXE

Up to 85% of PXE patients develop eye issues, including angioid streaks.

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Gonorrhoeae invasion

Gonorrhoeae can affect healthy epithelium, leading to corneal ulceration.

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Corneal ulceration

Peripheral corneal ulceration can occur if conjunctivitis is ignored.

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Gram-negative organism appearance

Gram staining shows kidney-shaped diplococci for certain infections.

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Haemophilus influenza

Most common cause of bacterial conjunctivitis in warmer climates for adults.

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Streptococcus pneumonia

Common cause of bacterial conjunctivitis in cooler climates among adults.

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Trachoma

An infection from Chlamydia trachomatis causing blindness if untreated.

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Arlt's lines

Linear scarring from Trachoma on palpebral conjunctiva.

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Herbert's pits

Depressions at limbus caused by scarring from Trachoma.

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Asymptomatic Patients

Some patients may show no symptoms of vision loss despite underlying issues.

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Optic Nerve Compression

Enlarging bone may compress the optic nerve, causing vision loss.

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Tamoxifen Effects

Tamoxifen can cause visual acuity decrease and retinal deposits.

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OCT for Tamoxifen Users

Optical coherence tomography (OCT) is essential for monitoring maculopathy.

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Retinitis Pigmentosa Triad

Classic signs include arteriolar attenuation, bone-spicule pigmentation, and optic disc pallor.

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Optic Disc Changes in RP

Optic disc pallor is a common change in retinitis pigmentosa patients.

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Systemic Disorders Causing Angioid Streaks

Conditions like sickle-cell disease and acromegaly can lead to angioid streaks.

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Vision Tests for Tamoxifen Users

Annual dilated eye exams and SD-OCT should be performed for monitoring.

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+1.25 lens function

Extends keratometry range from 52D to about 60-61D.

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Adding a lens to keratometry

A +2.25 lens extends measurements from steeper values, adding 16D to readings.

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-1.00 lens function

Covers keratometry readings from 32-38D, subtracting 6D for true value.

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Pellucid marginal degeneration (PMD)

Characterized by irregular corneal thinning, typically 1-2 mm wide.

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Acute hydrops in PMD

Can lead to corneal scarring and neovascularization.

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Kissing doves pattern

A hallmark diagnostic sign in PMD observed on corneal topography.

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Uses for bandage contact lenses

Protects corneal surface from irritation; promotes healing.

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Conditions warranting bandage lenses

Include recurrent corneal erosion, corneal abrasion, and post-PRK.

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