Diseases of the Sclera Practice Questions

Q: Ciliary staphyloma occurs due to all of the following except:

Episcleritis. ***Episcleritis*** - **Episcleritis** is a benign, self-limiting inflammation of the episclera, which is a superficial layer of connective tissue, and does not lead to **scleral thinning** or ectasia. - Therefore, it does not cause **ciliary staphyloma**, which is a bulging of the sclera in the ciliary body region. *Scleritis* - **Scleritis** is a severe, chronic inflammatory disease affecting the sclera, often leading to **scleral thinning** and weakening. - This thinning can predispose to the formation of a **ciliary staphyloma**, especially if the inflammation is localized in the ciliary region. *Absolute glaucoma* - **Absolute glaucoma** is a severe form of glaucoma characterized by persistently high **intraocular pressure (IOP)**, leading to total vision loss and often significant **scleral thinning** due to chronic pressure. - The elevated IOP can cause stretching and thinning of the sclera, particularly in weakened areas like the **ciliary body**, resulting in a **ciliary staphyloma**. *Perforating injury* - A **perforating injury** to the globe can directly weaken the **scleral wall**, especially if it occurs near the **ciliary body**. - Subsequent healing with scar tissue, often under intraocular pressure, can lead to ectasia and the formation of a **ciliary staphyloma**.

Q: Not true about blue sclera

Seen in Marfan's syndrome. ***Seen in Marfan's syndrome*** - **Blue sclera** is not a characteristic feature of **Marfan's syndrome**. - Marfan's syndrome is primarily associated with **connective tissue abnormalities** affecting the heart, blood vessels, eyes (lens dislocation), and skeleton, but not typically blue sclera. *Blue colour is produced by underlying uveal pigment* - The blue appearance of the sclera is due to the **thinness of the scleral collagen**, allowing the underlying **dark choroidal pigment** (part of the uvea) to be more visible. - This transparency effect is sometimes enhanced by the presence of **sub-scleral fat**, which reflects blue light. *Seen in osteogenesis imperfecta* - **Osteogenesis imperfecta** is a genetic disorder characterized by **brittle bones** and **blue sclera**. - The blue color is due to the **thinning of the sclera**, allowing the underlying choroidal veins to show through. *Not seen in Ehlers-Danlos syndrome* - **Blue sclera** can indeed be a feature of **Ehlers-Danlos syndrome**, particularly in types associated with **collagen defects** [1]. - Like osteogenesis imperfecta, the thinned sclera allows the underlying **choroid** to become visible, creating the blue hue [1].

Q: Most common cause of posterior staphyloma

Myopia. ***Myopia*** - **Pathological myopia**, characterized by excessive axial elongation of the eyeball, is the most common cause of posterior staphyloma. - The stretching and thinning of the sclera in high myopia leads to localized outward bulging, clinically known as a posterior staphyloma. *Glaucoma* - While glaucoma can cause **optic nerve damage** and visual field loss, it is not directly associated with the formation of a posterior staphyloma. - Its primary effect is on intraocular pressure and its consequences on the **optic nerve head**. *Scleritis* - **Scleritis** is an inflammatory condition affecting the sclera, which can lead to scleral thinning and even perforation. - However, it typically causes localized inflammation and thinning rather than the characteristic outward bulging seen in posterior staphyloma. *Trauma* - Severe ocular **trauma** can lead to globe rupture or other structural changes. - While trauma could theoretically cause localized scleral weakness, it is not the most frequent cause of posterior staphyloma compared to the chronic, progressive changes seen in high myopia.

Q: Most common type of scleritis among the following is

Non-necrotizing. ***Non-necrotizing*** - This category, particularly **diffuse non-necrotizing scleritis**, is the most frequently encountered type. - It is often characterized by widespread inflammation of the sclera without tissue loss. *Necrotizing* - This is a severe form of scleritis associated with significant **tissue destruction** and a high risk of vision loss. - While serious, it is thankfully much rarer than the non-necrotizing forms. *Granulomatous* - This term describes a **histopathological feature** of inflammation rather than a distinct clinical type of scleritis. - Granulomatous inflammation can be seen in various forms of scleritis but is not a primary classification of its most common presentation. *Posterior* - **Posterior scleritis** specifically involves inflammation of the sclera behind the equator of the globe. - It is less common than anterior scleritis and can present with different symptoms such as vision loss or pain with eye movement.

Q: The most common variety of scleritis is:

Non-necrotizing anterior diffuse. ***Non-necrotizing anterior diffuse*** - This is the **most common type of scleritis**, characterized by widespread inflammation of the anterior sclera without tissue loss. - It presents with **redness**, **pain**, and **tenderness** over a large area of the sclera. *Non-necrotizing anterior nodular* - This type features **localized, tender nodules** on the sclera, which are less common than the diffuse form. - While also non-necrotizing and anterior, the nodular presentation is not the most frequent variety. *Anterior necrotizing without inflammation* - This severe form, known as **scleromalacia perforans**, is rare and typically seen in patients with long-standing rheumatoid arthritis. - It is characterized by **scleral thinning and necrosis** without significant inflammatory signs. *Anterior necrotizing with inflammation* - This is a **severe and destructive form** of scleritis associated with significant pain and potential vision loss. - While serious, it is rare compared to diffuse non-necrotizing anterior scleritis.

Q: Blue sclera is seen in all of the following conditions except:

Keratoconus. ***Keratoconus*** - Keratoconus is a progressive eye disease in which the normally round cornea thins and begins to bulge into a cone-like shape, leading to **vision distortion**. - Blue sclera is **not a feature** of keratoconus. This is a **corneal condition** that does not affect the sclera. - Blue sclera, seen in the other conditions listed, occurs due to thinning of the sclera, making the underlying choroidal pigment visible. *Marfan's syndrome* - Patients with Marfan's syndrome can have blue sclera due to the **thinning of collagen** in the scleral tissue, allowing the underlying choroid to show through. - This connective tissue disorder affects multiple body systems, including the skeletal, cardiovascular, and ocular systems, with features like **arachnodactyly** and **aortic root dilation**. *Osteogenesis imperfecta* - Often referred to as **brittle bone disease**, osteogenesis imperfecta is characterized by defective **type I collagen synthesis**, which also affects the sclera. - The sclera becomes thin and translucent, revealing the underlying choroidal pigment, thus appearing **blue**. *Rheumatoid arthritis* - In rheumatoid arthritis, particularly with severe or long-standing disease, the sclera can become thinned due to **scleritis** or **scleromalacia perforans**. - This thinning can lead to a **blue discoloration** of the sclera, making the underlying choroid visible.

Q: Most common type of scleritis is

Anterior. ***Anterior*** - **Anterior scleritis** accounts for approximately **98%** of all scleritis cases, making it the most common type. - It involves inflammation of the sclera anterior to the **equator of the globe**, visible on external examination. - Anterior scleritis is further subdivided into **diffuse, nodular,** and **necrotizing** forms based on clinical presentation and severity. *Diffuse anterior* - **Diffuse anterior scleritis** is the most common subtype of anterior scleritis, characterized by widespread inflammation. - While common among anterior types, it represents a subset rather than the overall most common anatomical category. *Nodular anterior* - **Nodular anterior scleritis** presents with discrete nodules of inflamed scleral tissue. - It is less common than diffuse anterior scleritis but more common than necrotizing forms. *Posterior* - **Posterior scleritis** is rare, accounting for only about **2%** of all scleritis cases. - It involves inflammation posterior to the **equator of the globe** and can be difficult to diagnose due to its hidden location, often presenting with pain, proptosis, and vision loss.

Q: Most common cause of posterior staphyloma?

Myopia. ***Myopia*** - **Posterior staphyloma** is a characteristic degenerative change in **high myopia**, where the sclera thins and bulges posteriorly. - The rapid and excessive axial elongation of the eyeball in myopia leads to stretching and weakening of the posterior sclera. *Hypermetropia* - **Hypermetropia** (farsightedness) is characterized by an eyeball that is too short, leading to light focusing behind the retina. - It is not associated with the pathological thinning and bulging of the posterior sclera seen in staphyloma. *conjunctivitis* - **Conjunctivitis** is an inflammation of the conjunctiva, the membrane lining the inside of the eyelids and covering the sclera. - It does not involve structural changes to the sclera or retina that would lead to posterior staphyloma. *Glaucoma* - **Glaucoma** is a group of diseases that damage the optic nerve, often due to high intraocular pressure, leading to vision loss. - While it can cause optic disc cupping, it is not directly associated with the development of posterior staphyloma.

Q: Blue sclera is associated with:

Osteogenesis imperfecta. ***Osteogenesis imperfecta*** - **Osteogenesis imperfecta** (brittle bone disease) is the **MOST CLASSIC** condition associated with blue sclera. - Caused by a defect in **Type I collagen synthesis**, which is a major structural component of the sclera. - The deficiency in functional collagen leads to an **abnormally thin and translucent sclera**, allowing the underlying **choroidal pigment** to show through, giving it a characteristic blue appearance. - Blue sclera is seen in **80-90% of patients** with Type I osteogenesis imperfecta. *Buphthalmos* - **Buphthalmos** (congenital glaucoma) can occasionally show bluish sclera due to scleral thinning from increased intraocular pressure. - However, the primary findings are **corneal enlargement, corneal edema**, and increased eye size, not blue sclera as a defining feature. *Marfan's syndrome* - **Marfan's syndrome** is a connective tissue disorder caused by **fibrillin-1** defects. - Classic ocular features include **ectopia lentis** (lens dislocation), high myopia, and retinal detachment risk. - Blue sclera is **NOT a recognized clinical feature** of Marfan's syndrome. *Ehlers-Danlos syndrome* - This connective tissue disorder can present with blue sclera due to collagen abnormalities. - However, it is **less common** than osteogenesis imperfecta as a cause of blue sclera.

Q: Blue sclera is seen in all except

keratoconus. ***keratoconus*** - **Keratoconus** is a condition where the cornea thins and bulges into a cone-like shape, leading to blurry vision and light sensitivity, but it does not cause blue sclera. - The sclera in keratoconus typically retains its normal white appearance. *Ehlers-Danlos syndrome* - **Ehlers-Danlos syndrome** is a group of genetic disorders affecting connective tissue, leading to overly flexible joints, stretchy skin, and fragile tissues. - The **thinness of the sclera** in these patients allows the underlying choroidal pigment to show through, making the sclera appear blue. *Marfan syndrome* - **Marfan syndrome** is a genetic disorder of connective tissue, causing issues in the heart, blood vessels, bones, joints, and eyes. - Similar to Ehlers-Danlos syndrome, the **sclera can be thinner** in Marfan syndrome, making it appear bluish due to the underlying choroidal vascularity. *Osteogenesis imperfecta* - **Osteogenesis imperfecta** is a genetic disorder characterized by fragile bones that break easily. It is caused by defects in type I collagen synthesis. - The sclera appears blue because the **underlying choroidal veins are visible** through the abnormally thin and translucent sclera.

Question 1

Ciliary staphyloma occurs due to all of the following except:

Accepted Answer

Episcleritis

Answer

Scleritis

Answer

Absolute glaucoma

Answer

Perforating injury

Question 2

Not true about blue sclera

Accepted Answer

Seen in Marfan's syndrome

Answer

Blue colour is produced by underlying uveal pigment

Answer

Seen in osteogenesis imperfecta

Answer

Not seen in Ehlers-Danlos syndrome

Question 3

Most common cause of posterior staphyloma

Accepted Answer

Myopia

Answer

Glaucoma

Answer

Scleritis

Answer

Trauma

Question 4

Most common type of scleritis among the following is

Accepted Answer

Non-necrotizing

Answer

Necrotizing

Answer

Granulomatous

Answer

Posterior

Question 5

The most common variety of scleritis is:

Accepted Answer

Non-necrotizing anterior diffuse

Answer

Non-necrotizing anterior nodular

Answer

Anterior necrotizing without inflammation

Answer

Anterior necrotizing with inflammation

Question 6

Blue sclera is seen in all of the following conditions except:

Accepted Answer

Keratoconus

Answer

Marfan's syndrome

Answer

Osteogenesis imperfecta

Answer

Rheumatoid arthritis

Question 7

Most common type of scleritis is

Accepted Answer

Answer

Diffuse anterior

Answer

Nodular anterior

Answer

Posterior

Question 8

Most common cause of posterior staphyloma?

Accepted Answer

Myopia

Answer

Hypermetropia

Answer

Conjunctivitis

Answer

Glaucoma

Question 9

Blue sclera is associated with:

Accepted Answer

Osteogenesis imperfecta

Answer

Ehlers-Danlos syndrome

Answer

Buphthalmos

Answer

Marfan's syndrome

Question 10

Blue sclera is seen in all except

Accepted Answer

keratoconus

Answer

Ehlers-Danlos syndrome

Answer

Marfan syndrome

Answer

Osteogenesis imperfecta

Diseases of the Sclera — MCQs

Diseases of the Sclera — MCQs

On this page

Practice by Chapter

Want unlimited practice?