Developmental and Congenital Glaucomas — MCQs
In primary open-angle glaucoma (POAG), which of the following findings is NOT typically seen?
Which of the following is NOT a feature of Primary Infantile (Congenital) glaucoma?
Which of the following is not a feature of open-angle glaucoma?
What is the cause of glaucoma in retinoblastoma?
All of the following can be used to decrease intraocular pressure in glaucoma except?
Statement 1 - A 59-year-old patient presents with flaccid bullae. Histopathology shows a suprabasal acantholytic split. Statement 2 - The row of tombstones appearance is diagnostic of Pemphigus vulgaris.
In which of the following conditions is the intraocular pressure very high, and inflammation is minimal?
Which of the following statements about congenital glaucoma is incorrect?
A one-month-old baby presents with excessive tearing (watering) and an increased corneal size. What is the most likely diagnosis?
Which of the following ocular findings is not associated with diabetes?
Developmental and Congenital Glaucomas Indian Medical PG Practice Questions and MCQs
Question 1: In primary open-angle glaucoma (POAG), which of the following findings is NOT typically seen?
- A. Bayoneting sign (arteriovenous crossing changes)
- B. Peripapillary atrophy
- C. Horizontal cupping of the optic disc
- D. Macular edema (Correct Answer)
Explanation: ***Macular edema*** - **Macular edema** is characterized by fluid accumulation in the fovea or retina, causing blurry vision and metamorphopsia. - While it can occur in conditions like **diabetic retinopathy** or **uveitis**, it is **not a typical finding in POAG**, which primarily affects the optic nerve. *Horizontal cupping of the optic disc* - **Horizontal cupping** (or vertical elongation of the optic cup) is a common sign of **glaucomatous damage**, reflecting the loss of retinal ganglion cells. - This morphological change suggests the progression of optic nerve atrophy. *Bayoneting sign (arteriovenous crossing changes)* - The **bayoneting sign**, where blood vessels appear to dip below the optic disc margin and then sharply bend, is a feature of **advanced glaucomatous cupping**. - It indicates significant loss of optic nerve tissue and is often associated with deeply excavated optic discs. *Peripapillary atrophy* - **Peripapillary atrophy** (PPA) refers to areas of RPE and choroidal atrophy surrounding the optic disc, commonly seen in glaucoma. - While its presence and extent may correlate with **glaucoma severity**, it is a recognized clinical feature of the disease.
Question 2: Which of the following is NOT a feature of Primary Infantile (Congenital) glaucoma?
- A. Aniridia may be associated (Correct Answer)
- B. Treatment includes trabeculotomy
- C. Buphthalmos can occur
- D. Cornea is typically enlarged and cloudy.
Explanation: ***Aniridia may be associated*** - **Aniridia** is a congenital absence of the iris that causes **secondary glaucoma**, not primary infantile glaucoma. - Aniridia-associated glaucoma is a distinct entity from primary congenital glaucoma (PCG), which occurs due to isolated developmental abnormalities of the anterior chamber angle. - This is **NOT a feature** of primary infantile glaucoma, making it the correct answer to this negation question. *Treatment includes trabeculotomy* - **Trabeculotomy** or **goniotomy** are the primary surgical treatments for primary infantile glaucoma. - These procedures aim to improve aqueous outflow by incising or opening the trabecular meshwork. - This is a **true feature** of the management of primary infantile glaucoma. *Buphthalmos can occur* - **Buphthalmos** (\"ox eye\") refers to the enlargement of the globe due to elevated intraocular pressure in infants when the sclera is still distensible. - It is a **classic clinical sign** of primary infantile glaucoma, typically occurring before age 3 years. - This is a **characteristic feature** of the condition. *Cornea is typically enlarged and cloudy* - The **cornea becomes enlarged** (increased horizontal corneal diameter >12 mm in newborns) due to stretching from elevated IOP. - **Corneal cloudiness** results from corneal edema and Haab's striae (breaks in Descemet's membrane). - These are **pathognomonic findings** in primary infantile glaucoma.
Question 3: Which of the following is not a feature of open-angle glaucoma?
- A. Elevated intraocular pressure
- B. Increased cup-to-disc ratio
- C. Gradual peripheral vision loss
- D. Pain and redness (Correct Answer)
Explanation: ***Pain and redness*** - **Open-angle glaucoma** is typically a **painless** condition, often referred to as the "silent thief of sight", because central vision is preserved until late stages. - **Pain** and **redness** are more characteristic of **acute angle-closure glaucoma** or other inflammatory eye conditions, not the chronic, progressive nature of open-angle glaucoma. *Elevated intraocular pressure* - **Elevated intraocular pressure** (IOP) is a primary risk factor and a hallmark feature of **open-angle glaucoma**, although normal-tension glaucoma exists. - Sustained high IOP can lead to **optic nerve damage** over time. *Increased cup-to-disc ratio* - An **increased cup-to-disc ratio** on examination of the optic disc is a key diagnostic feature of **open-angle glaucoma**, indicating **optic nerve damage** and atrophy. - This enlargement of the optic cup is due to the loss of nerve fibers. *Gradual peripheral vision loss* - **Gradual peripheral vision loss** is the characteristic pattern of vision loss in **open-angle glaucoma**, often unnoticed by the patient until advanced stages. - The central vision remains relatively intact until late in the disease, making early detection challenging.
Question 4: What is the cause of glaucoma in retinoblastoma?
- A. Neovascularisation (Correct Answer)
- B. Mass effect of the tumour
- C. Blockage of trabecular network
- D. Lysis of the lens
Explanation: ***Neovascularisation*** - Retinoblastoma leads to **neovascularization of the iris (NVI)** and **angle structures** due to tumor necrosis, ischemia, and release of **angiogenic factors (VEGF)**. - The **neovascular membrane** grows over and obstructs the **trabecular meshwork** and anterior chamber angle, causing **secondary neovascular glaucoma**. - This is the **most characteristic and common mechanism** of glaucoma in advanced retinoblastoma. - Neovascular glaucoma in retinoblastoma is typically **refractory to medical treatment** and indicates poor prognosis. *Blockage of trabecular network* - While tumor cells can directly seed into the anterior chamber and block the trabecular meshwork, this mechanism is **less common** than neovascularization. - This option is also **less specific** as it doesn't identify the underlying pathophysiological process (neovascularization) that is characteristic of retinoblastoma-associated glaucoma. - Direct trabecular blockage by tumor cells typically occurs in advanced disease and often coexists with neovascularization. *Mass effect of the tumour* - The tumor mass itself rarely causes glaucoma through direct mechanical compression of outflow pathways. - Retinoblastoma causes glaucoma primarily through **secondary mechanisms** such as inflammation, neovascularization, or cellular seeding, not simple bulk effect. - Massive tumors may cause angle closure, but this is uncommon compared to neovascular mechanisms. *Lysis of the lens* - **Phacolytic glaucoma** from lens protein leakage is rare in retinoblastoma. - While lens damage can occur with advanced tumors, it is **not a typical or characteristic cause** of glaucoma in retinoblastoma. - The primary mechanisms involve the **tumor-angle-trabecular meshwork axis**, not lens pathology.
Question 5: All of the following can be used to decrease intraocular pressure in glaucoma except?
- A. Mannitol
- B. Clonidine
- C. Dexamethasone (Correct Answer)
- D. Methazolamide
Explanation: ***Dexamethasone*** - **Corticosteroids** like dexamethasone are known to **increase intraocular pressure** by reducing the outflow of aqueous humor, thus exacerbating glaucoma. - This effect is often dose-dependent and can lead to **steroid-induced glaucoma** in susceptible individuals. *Mannitol* - **Mannitol** is an osmotic diuretic used to **rapidly decrease intraocular pressure** by drawing fluid out of the vitreous humor. - It is often used in acute angle-closure glaucoma or before ocular surgery. *Clonidine* - **Clonidine** is an alpha-2 adrenergic agonist that can **decrease aqueous humor production**, thereby reducing intraocular pressure. - While it has been studied for glaucoma, its systemic side effects often limit its use. *Methazolamide* - **Methazolamide** is a **carbonic anhydrase inhibitor** that reduces the production of aqueous humor. - This reduction in fluid production directly leads to a decrease in intraocular pressure, making it a common glaucoma treatment.
Question 6: Statement 1 - A 59-year-old patient presents with flaccid bullae. Histopathology shows a suprabasal acantholytic split. Statement 2 - The row of tombstones appearance is diagnostic of Pemphigus vulgaris.
- A. Statements 1 & 2 are correct, 2 is not explaining 1 (Correct Answer)
- B. Statements 1 and 2 are correct and 2 is the correct explanation for 1
- C. Statements 1 and 2 are incorrect
- D. Statement 1 is incorrect
Explanation: ***Correct: Statements 1 & 2 are correct, 2 is not explaining 1*** **Analysis of Statement 1:** - A 59-year-old patient with **flaccid bullae** and **suprabasal acantholytic split** on histopathology is the classic presentation of **Pemphigus vulgaris** - The flaccid (easily ruptured) nature of bullae distinguishes it from tense bullae seen in bullous pemphigoid - The suprabasal location of the split (just above the basal layer) with acantholysis (loss of cell-to-cell adhesion) is pathognomonic - **Statement 1 is CORRECT** ✓ **Analysis of Statement 2:** - The **"row of tombstones" or "tombstone appearance"** is indeed a diagnostic histopathological feature of Pemphigus vulgaris - This appearance results from basal keratinocytes remaining attached to the basement membrane while suprabasal cells separate due to acantholysis - The intact basal cells standing upright resemble a row of tombstones - **Statement 2 is CORRECT** ✓ **Does Statement 2 explain Statement 1?** - Statement 2 describes a **histopathological appearance** (tombstone pattern) that is a **consequence** of the suprabasal split - However, it does NOT explain the **underlying cause** of the flaccid bullae or the suprabasal split - The true explanation involves **IgG autoantibodies against desmoglein 3 (and desmoglein 1)**, which attack intercellular adhesion structures (desmosomes), causing **acantholysis** - Therefore, **Statement 2 does NOT explain Statement 1** ✗ *Incorrect: Statement 2 is the correct explanation for Statement 1* - While both statements describe features of Pemphigus vulgaris, the tombstone appearance is a descriptive finding, not an explanatory mechanism *Incorrect: Statements 1 and 2 are incorrect* - Both statements are medically accurate descriptions of Pemphigus vulgaris features *Incorrect: Statement 1 is incorrect* - Statement 1 correctly describes the cardinal clinical and histopathological features of Pemphigus vulgaris
Question 7: In which of the following conditions is the intraocular pressure very high, and inflammation is minimal?
- A. Glaucomatocyclic crises (Correct Answer)
- B. Angle closure glaucoma
- C. Acute iridocyclitis
- D. Hypertensive uveitis
Explanation: ***Glaucomatocyclic crises*** - This condition is characterized by recurrent, self-limiting episodes of markedly **elevated intraocular pressure (IOP)** with minimal or no overt signs of inflammation in the anterior chamber. - The elevated IOP is thought to result from **altered humor outflow** due to subtle inflammation of the trabecular meshwork. *Acute iridocyclitis* - Presents with significant signs of **intraocular inflammation**, including **cells and flare** in the anterior chamber, typically with pain and photophobia. - While IOP can be elevated, it's a direct result of inflammation reducing outflow, and the inflammation itself is prominent. *Angle closure glaucoma* - This condition involves a sudden and severe rise in **IOP** due to blockage of the aqueous humor outflow pathway by the peripheral iris, but it's not primarily an inflammatory process. - While the eye can appear red and painful, this is due to ischemia and corneal edema, not marked **intraocular inflammation** like that seen in uveitis. *Hypertensive uveitis* - Refers to any **uveitis** that causes a rise in **intraocular pressure**, meaning significant inflammation is present. - The high IOP is secondary to the inflammation, which can obstruct the trabecular meshwork or stimulate prostaglandin release, both causing reduced outflow.
Question 8: Which of the following statements about congenital glaucoma is incorrect?
- A. Thin and blue sclera seen
- B. Anterior chamber is shallow (Correct Answer)
- C. Photophobia is most common symptom
- D. Haab's Striae may be seen
Explanation: ***Anterior chamber is shallow*** - In congenital glaucoma, the **anterior chamber depth is typically normal or deep**, not shallow. - A shallow anterior chamber is more characteristic of **angle-closure glaucoma**, which is mechanistically different. - This makes the statement incorrect, as congenital glaucoma is associated with a **deep anterior chamber** due to globe enlargement. *Photophobia is most common symptom* - **Photophobia** (sensitivity to light) is indeed one of the classic presenting symptoms in congenital glaucoma. - It forms part of the classic triad: **photophobia, epiphora (tearing), and blepharospasm**. - This occurs due to **increased intraocular pressure** causing corneal edema and irritation. *Thin and blue sclera seen* - The **sclera** can appear thin and blue due to **buphthalmos** (enlargement of the eye) and stretching of the globe. - The stretching allows the underlying **uveal tissue** to show through, giving the characteristic blue appearance. - This is a direct consequence of elevated intraocular pressure in a developing eye. *Haab's Striae may be seen* - **Haab's striae** are **Descemet's membrane tears** that are pathognomonic of congenital glaucoma. - These horizontal or curvilinear breaks occur due to stretching of the cornea from **elevated intraocular pressure**. - They appear as visible linear opacities on corneal examination.
Question 9: A one-month-old baby presents with excessive tearing (watering) and an increased corneal size. What is the most likely diagnosis?
- A. Galactosemia
- B. Buphthalmos (Correct Answer)
- C. Cataract
- D. Hurler syndrome
Explanation: ***Buphthalmos*** - **Buphthalmos** refers to the enlargement of the eye in infants, typically caused by **congenital glaucoma**, which results in increased intraocular pressure. - The combination of **excessive tearing (epiphora)** and an **increased corneal size** (seen in the image as unusually large corneas for a one-month-old) are classic signs of buphthalmos due to elevated intraocular pressure stretching the infant's pliable sclera and cornea. *Galactosemia* - **Galactosemia** is a metabolic disorder that can cause cataracts and, in severe cases, liver damage and intellectual disability, but it does **not typically cause buphthalmos or enlarged corneas**. - While cataracts can lead to poor vision, they don't explain the excessive tearing or corneal enlargement. *Cataract* - A **cataract** is an opacity in the lens of the eye, which can cause blurry vision and a white pupil reflex (leukocoria), but **does not cause increased corneal size or excessive tearing** as primary symptoms. - While cataracts can occur in infants, they do not present with the specific combination of signs described. *Hurler syndrome* - **Hurler syndrome** is a lysosomal storage disorder (mucopolysaccharidosis type I) that can cause various ocular abnormalities, including **corneal clouding**, but typically **not corneal enlargement or buphthalmos**. - Other features include coarse facial features, skeletal abnormalities, and developmental delay, which are not mentioned in the presentation criteria.
Question 10: Which of the following ocular findings is not associated with diabetes?
- A. Retinopathy
- B. Early senile cataract
- C. Neovascular glaucoma
- D. Blepharophimosis (Correct Answer)
Explanation: ***Blepharophimosis*** - This is a **congenital disorder** characterized by small palpebral fissures, ptosis, and epicanthus inversus, which is **not associated with diabetes**. - It is a **developmental anomaly** of the eyelids, with no known link to metabolic conditions like diabetes. *Retinopathy* - **Diabetic retinopathy** is a common and serious complication of diabetes, caused by damage to the blood vessels in the retina. - It can lead to vision loss if not managed, and is directly linked to **poor glycemic control**. *Early senile cataract* - Diabetes is a significant risk factor for the **earlier development and progression of cataracts**, including senile cataracts. - High blood sugar levels can cause changes in the lens, leading to **opacification** and impaired vision. *Neovascular glaucoma* - This severe form of **secondary glaucoma** is often a complication of advanced **diabetic retinopathy**. - Ischemia in the retina triggers the growth of **new blood vessels** on the iris and in the angle of the eye, obstructing aqueous outflow and raising intraocular pressure.
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