Pediatric Ophthalmology and Strabismus — MCQs

Pediatric Ophthalmology and Strabismus Indian Medical PG Practice Questions and MCQs

Question 11: Fusion of the eyelids by thin tags is called:

A. Ankyloblepharon filiforme adnatum (Correct Answer)
B. Euryblepharon
C. Ablepharon
D. Epiblepharon

Explanation: ### Explanation **Correct Option: A. Ankyloblepharon filiforme adnatum** Ankyloblepharon refers to the partial or complete fusion of the upper and lower eyelid margins. The specific variant **Ankyloblepharon filiforme adnatum (AFA)** is a rare congenital anomaly where the lids are joined by fine, extensible **proteinaceous bands (tags)**. These bands consist of a central core of vascularized connective tissue surrounded by epithelium. It is usually an isolated finding but can be associated with multisystem syndromes like AEC (Ankyloblepharon-Ectodermal defects-Cleft lip/palate) syndrome. **Analysis of Incorrect Options:** * **B. Euryblepharon:** This is a congenital horizontal enlargement of the palpebral fissure, leading to a "large eye" appearance and often resulting in lateral ectropion. * **C. Ablepharon:** This refers to the complete absence of eyelids. It is often seen in "Ablepharon-Macrostomia Syndrome." * **D. Epiblepharon:** A condition where an extra horizontal fold of skin and orbicularis muscle pushes the lashes vertically or against the globe (pseudotrichiasis), most commonly seen in Asian children and usually involving the lower lid. **High-Yield Clinical Pearls for NEET-PG:** * **Treatment of AFA:** Simple excision or "snip" of the bands is required urgently to prevent **stimulus-deprivation amblyopia**. * **Cryptophthalmos:** A more severe condition than ankyloblepharon where the eyelid skin is continuous over the eyeball (no palpebral fissure), often associated with **Fraser Syndrome**. * **Distichiasis:** Presence of an extra row of lashes originating from the **Meibomian gland openings**.

Question 12: The Reese-Ellsworth classification categorizes which of the following tumors with respect to numbers, size, and location?

A. Rhabdomyosarcoma
B. Retinoblastoma (Correct Answer)
C. Optic nerve sheath tumor
D. Meningioma

Explanation: **Explanation:** The **Reese-Ellsworth (R-E) classification** was historically the primary system used to predict the prognosis for **eye salvage** (vision preservation) in patients with **Retinoblastoma** treated with external beam radiotherapy (EBRT). 1. **Why Retinoblastoma is correct:** The R-E classification divides Retinoblastoma into five groups (Group I to V) based on the **number** of tumors, their **size** (measured in disc diameters), and their **location** (equatorial vs. vitreous seeding). Group I (Very Favorable) typically involves small, solitary tumors, while Group V (Very Unfavorable) involves massive tumors involving over half the retina or vitreous seeding. * *Note:* In modern practice, the **International Classification of Retinoblastoma (ICRB)** has largely replaced R-E as it better predicts success with systemic chemotherapy. 2. **Why other options are incorrect:** * **Rhabdomyosarcoma:** This is the most common primary orbital malignancy in children. It is classified using the TNM system and clinical grouping (IRSG), not R-E. * **Optic Nerve Sheath Tumor/Meningioma:** These are orbital/neurological tumors. Meningiomas (e.g., optic nerve sheath meningioma) are staged based on imaging and extent of intracranial spread, often associated with Neurofibromatosis type 2. **High-Yield Clinical Pearls for NEET-PG:** * **Most common intraocular tumor of childhood:** Retinoblastoma. * **Most common presenting sign:** Leukocoria (white pupillary reflex), followed by strabismus. * **Genetics:** Mutation in the **RB1 gene** on chromosome **13q14**. * **Pathology:** Look for **Flexner-Wintersteiner rosettes** (highly specific) and Homer-Wright rosettes. * **Calcification:** Dystrophic calcification is a hallmark feature seen on B-scan USG or CT.

Question 13: All of the following can cause a white pupillary reflex except?

A. Retinoblastoma
B. Cataract
C. Retrolental fibroplasias
D. Glaucoma (Correct Answer)

Explanation: **Explanation:** The white pupillary reflex, clinically known as **Leukocoria**, occurs when light reflects off an abnormal intraocular mass or opacity located behind the lens or within the vitreous, rather than the normal red reflex from the vascular choroid. **Why Glaucoma is the correct answer:** In pediatric cases, **Congenital Glaucoma** typically presents with the "classic triad" of epiphora (tearing), photophobia, and blepharospasm. Clinically, it is characterized by a **cloudy or hazy cornea** (due to edema) and an enlarged eyeball (buphthalmos). It does not cause a white reflection from behind the pupil; therefore, it is not a cause of leukocoria. **Analysis of incorrect options (Causes of Leukocoria):** * **Retinoblastoma (Option A):** The most common primary intraocular malignancy of childhood and the most life-threatening cause of leukocoria. It must be ruled out first in any child presenting with a white reflex. * **Cataract (Option B):** Congenital cataract is the **most common cause** of leukocoria. The opacity of the crystalline lens blocks the red reflex. * **Retrolental Fibroplasia (Option C):** Now known as **Retinopathy of Prematurity (ROP)**. In advanced stages (Stage 5), total retinal detachment and fibrovascular scarring behind the lens create a dense white pupillary reflex. **Clinical Pearls for NEET-PG:** * **Differential Diagnosis of Leukocoria:** Includes Retinoblastoma, Congenital Cataract, ROP, Coats’ Disease, Persistent Fetal Vasculature (PFV), and Toxocariasis. * **Most common cause of Leukocoria:** Congenital Cataract. * **Most common intraocular tumor in children:** Retinoblastoma. * **Investigation of choice:** B-scan Ultrasonography (to look for calcification in Retinoblastoma).

Question 14: Unilateral water discharge from the eye of a newborn with no edema or chemosis is due to which of the following?

A. Chlamydia trachomatis
B. Neisseria gonorrhoeae
C. Mucoid discharge (sticky eye)
D. Chemical conjunctivitis (Correct Answer)

Explanation: **Explanation:** The correct answer is **Chemical conjunctivitis**. **1. Why Chemical Conjunctivitis is correct:** Chemical conjunctivitis is the most common cause of neonatal conjunctivitis (Ophthalmia Neonatorum) occurring within the **first 24 hours** of life. It is typically caused by the prophylactic use of silver nitrate (Credé's method) or antibiotic drops. The hallmark clinical presentation is **mild watery discharge** with minimal to no signs of inflammation (no significant edema or chemosis). It is a self-limiting condition that usually resolves within 48 hours without treatment. **2. Why other options are incorrect:** * **Chlamydia trachomatis:** This is the most common *infectious* cause of ophthalmia neonatorum, but it typically presents between **5 to 14 days** after birth. It is characterized by significant mucopurulent discharge, chemosis, and pseudomembrane formation. * **Neisseria gonorrhoeae:** This is the most *hyperacute and severe* form, appearing **2 to 5 days** after birth. It presents with profuse, thick purulent discharge, severe lid edema, and chemosis. It carries a high risk of corneal perforation. * **Mucoid discharge (Sticky eye):** While common in newborns due to Nasolacrimal Duct Obstruction (NLDO), it usually presents after the first week of life with persistent tearing and crusting, rather than an immediate onset of watery discharge. **3. Clinical Pearls for NEET-PG:** * **Timeline is Key:** * < 24 hours: Chemical * 2–5 days: Gonococcal (Most severe) * 5–14 days: Chlamydia (Most common infectious) * > 2 weeks: Herpes Simplex (presents with vesicles) * **Prophylaxis:** Erythromycin 0.5% ointment is now preferred over Silver Nitrate to prevent chemical irritation. * **Treatment:** Gonococcal requires systemic Ceftriaxone; Chlamydia requires systemic Erythromycin (to prevent associated pneumonia).

Question 15: What is the most common malignant orbital tumor in children?

A. Acute Myeloid Leukemia (AML)
B. Acute Lymphoblastic Leukemia (ALL)
C. Rhabdomyosarcoma (Correct Answer)
D. Cavernous hemangioma

Explanation: ### Explanation **Correct Answer: C. Rhabdomyosarcoma** **Why it is correct:** Rhabdomyosarcoma (RMS) is the **most common primary malignant orbital tumor in children**. It is a mesenchymal tumor arising from undifferentiated pluripotent cells (not necessarily from extraocular muscles). It typically presents as a **rapidly progressing, painless proptosis** in a child (average age 7–8 years). The most common histological subtype is the **Embryonal** type (best prognosis), while the Alveolar type is the most aggressive. **Why the other options are incorrect:** * **A & B (AML/ALL):** While leukemias (specifically AML/Chloroma) are a common cause of orbital involvement in children, they are considered **secondary** or metastatic infiltrations rather than primary orbital tumors. * **D. Cavernous Hemangioma:** This is the most common **benign** orbital tumor in **adults**. In children, the most common benign orbital tumor is the **Capillary Hemangioma**. **High-Yield Clinical Pearls for NEET-PG:** * **Most common primary malignant orbital tumor:** Rhabdomyosarcoma. * **Most common benign orbital tumor (Children):** Capillary Hemangioma. * **Most common benign orbital tumor (Adults):** Cavernous Hemangioma. * **Most common site of RMS:** Superonasal quadrant of the orbit. * **Histology Tip:** "Cross-striations" on light microscopy and "Rhabdomyoblasts" are characteristic. * **Management:** It is a medical emergency. Diagnosis is via biopsy, and treatment involves a combination of chemotherapy and radiotherapy (surgery is usually limited to biopsy or debulking).

Question 16: What is the most common calcified intraocular malignancy seen in children?

A. Rhabdomyosarcoma
B. Retinoblastoma (Correct Answer)
C. Metastasis
D. Leukaemia

Explanation: **Explanation:** **Retinoblastoma** is the most common primary intraocular malignancy of childhood. It originates from the neurosensory retina due to a mutation in the **RB1 gene** on chromosome 13q14. The hallmark feature of this tumor is **dystrophic calcification**, which occurs due to rapid tumor growth outstripping its blood supply, leading to necrosis and calcium deposition. On imaging (B-scan USG or CT), calcification is seen in over 90% of cases, making it a pathognomonic sign for diagnosis. **Analysis of Incorrect Options:** * **A. Rhabdomyosarcoma:** This is the most common primary **orbital** malignancy in children, but it is extraocular (not intraocular) and typically does not show intrinsic calcification. * **C. Metastasis:** While common in adults (usually from lung or breast), intraocular metastases are rare in children. When they occur, they are usually from neuroblastoma and rarely calcify. * **D. Leukaemia:** This is the most common systemic malignancy to involve the eye in children (often presenting as iris infiltration or hypopyon), but it is not a primary intraocular tumor and does not typically calcify. **High-Yield Clinical Pearls for NEET-PG:** * **Most common presentation:** Leukocoria (White pupillary reflex), followed by strabismus. * **Histopathology:** Look for **Flexner-Wintersteiner rosettes** (highly specific) and Homer-Wright rosettes. * **Imaging:** CT scan is the gold standard to detect calcification, though MRI is preferred to evaluate optic nerve involvement. * **Classification:** The International Classification for Intraocular Retinoblastoma (ICIR) is used for staging. * **Trilateral Retinoblastoma:** Bilateral retinoblastoma associated with a pineal gland tumor (Pinealoblastoma).

Question 17: In a child with phthisical eye of unknown etiology, which of the following possibilities should be considered?

A. Bilateral trauma
B. Still's disease
C. Retinoblastoma (Correct Answer)
D. Untreated congenital glaucoma

Explanation: **Explanation:** In pediatric ophthalmology, a **phthisical eye** (a shrunken, non-functional, and atrophic globe) of unknown etiology is a critical clinical finding. While phthisis is usually the end-stage of trauma or chronic inflammation, in children, it can be a deceptive presentation of **Retinoblastoma (RB)**. **Why Retinoblastoma is the correct answer:** Retinoblastoma is the most common intraocular malignancy in children. Occasionally, a rapidly growing tumor can outgrow its blood supply, leading to massive necrosis. This necrosis triggers severe sterile endophthalmitis, which can result in the shrinkage of the globe (phthisis bulbi). This is often referred to as **"spontaneous regression"** or "pseudophthisis." The clinical danger is that the tumor cells may still be viable within the shrunken globe, and missing this diagnosis can be fatal. Therefore, any child with an unexplained phthisical eye must undergo imaging (B-scan or CT) to rule out intraocular calcification. **Analysis of Incorrect Options:** * **Bilateral trauma:** While trauma is a common cause of phthisis, it is usually unilateral and the history is typically known. It does not carry the same urgent diagnostic weight as ruling out a malignancy. * **Still’s disease (JIA):** Chronic iridocyclitis associated with Juvenile Idiopathic Arthritis can lead to phthisis due to ciliary body exhaustion. However, this is usually preceded by a long, documented history of uveitis and band-shaped keratopathy. * **Untreated congenital glaucoma:** This typically leads to **Buphthalmos** (an enlarged, "ox-like" eye) due to the distensibility of the young sclera under high pressure, rather than a shrunken eye. **High-Yield Clinical Pearls for NEET-PG:** * **Rule of Thumb:** Any child presenting with a phthisical eye without a clear history of trauma must be investigated for Retinoblastoma. * **Imaging:** Ultrasound (B-scan) or CT scan will show **intraocular calcification** (the hallmark of RB) even in a phthisical globe. * **Pathology:** Retinoblastoma is characterized by **Flexner-Wintersteiner rosettes**.

Question 18: What is the commonest intraocular tumor in children?

A. Retinoblastoma (Correct Answer)
B. Malignant melanoma
C. Teratoma
D. Neuroblastoma

Explanation: **Explanation:** **Retinoblastoma** is the most common primary intraocular malignancy of childhood. It originates from the neurosensory retina due to a mutation in the **RB1 gene** (located on chromosome 13q14). It typically presents within the first 3 years of life, most commonly manifesting as **leukocoria** (white pupillary reflex) or strabismus. Because it is a life-threatening condition that can spread via the optic nerve, early diagnosis is critical. **Analysis of Incorrect Options:** * **Malignant Melanoma:** This is the most common primary intraocular tumor in **adults**, not children. It typically arises from the uveal tract (choroid, ciliary body, or iris). * **Teratoma:** While orbital teratomas can occur in neonates causing extreme proptosis, they are rare and typically **extraocular** (located within the orbit but outside the globe). * **Neuroblastoma:** This is a common extracranial solid tumor in children, but it does not originate within the eye. It is, however, the most common tumor to **metastasize to the orbit** (causing "raccoon eyes" or ecchymosis), but it is not a primary intraocular tumor. **High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** 40% are heritable (often bilateral/multifocal); 60% are non-heritable (usually unilateral). * **Pathology:** Look for **Flexner-Wintersteiner rosettes** (highly specific) and Homer-Wright rosettes. * **Calcification:** Intraocular calcification in a child’s eye on CT/Ultrasound is a hallmark sign of Retinoblastoma. * **Trilateral Retinoblastoma:** Bilateral retinoblastoma associated with a pineal gland tumor (Pineoblastoma).

Question 19: What is the action of the superior oblique muscle?

A. Elevation, Intorsion, Adduction
B. Depression, Intorsion, Adduction
C. Elevation, Intorsion, Abduction
D. Depression, Intorsion, Abduction (Correct Answer)

Explanation: ### Explanation The actions of the extraocular muscles are determined by their origin, insertion, and the angle they make with the visual axis. The **Superior Oblique (SO)** originates from the apex of the orbit, passes through the trochlea (acting as its functional origin), and inserts onto the posterior-superior-lateral quadrant of the globe. 1. **Primary Action (Intorsion):** Because it inserts behind the equator on the superior aspect, its primary role is inward rotation. 2. **Secondary Action (Depression):** The SO pulls the posterior part of the globe upward, which causes the anterior part (the cornea) to move downward. 3. **Tertiary Action (Abduction):** Due to its 51° angle with the visual axis, it pulls the back of the eye medially, resulting in the front of the eye moving laterally (abduction). #### Analysis of Options: * **Option D (Correct):** Correctly identifies the triad of **Depression, Intorsion, and Abduction**. * **Option A & C:** Incorrect because the SO is a **depressor**, not an elevator. (The Superior Rectus and Inferior Oblique are elevators). * **Option B:** Incorrect because the SO is an **abductor**. Adduction is a tertiary action of the Superior and Inferior Recti. #### High-Yield Clinical Pearls for NEET-PG: * **RAD Rule:** **R**ecti are **AD**ductors (except Lateral Rectus). Therefore, Obliques are Abductors. * **SIN Rule:** **S**uperior muscles are **IN**torsionists (Superior Oblique and Superior Rectus). * **Testing Position:** To isolate the **depressing** action of the SO, the eye must be **adducted** (moved inward) to align the visual axis with the muscle's pull. * **Nerve Supply:** The SO is the only muscle supplied by the **4th Cranial Nerve (Trochlear)**. A palsy results in "Bielschowsky’s head tilt test" positivity (tilting to the opposite side to compensate for extorsion).

Question 20: Which of the following statements about latent nystagmus is FALSE?

A. Fast phase is directed towards the fixing eye.
B. Nystagmus increases with monocular occlusion.
C. It is often associated with intermittent exotropia. (Correct Answer)
D. A null point is present in adduction.

Explanation: ### Explanation **Latent Nystagmus (LN)** is a conjugate, horizontal jerk nystagmus that appears or worsens when one eye is occluded. **Why Option C is the Correct (False) Statement:** Latent nystagmus is classically associated with **Infantile Esotropia (Congenital Esotropia)**, not intermittent exotropia. It is part of the "Infantile Esotropia Syndrome," which also includes dissociated vertical deviation (DVD) and inferior oblique overaction. **Analysis of Other Options:** * **Option A (True):** In LN, the **fast phase** is always directed **towards the fixing eye**. If the left eye is covered, the right eye develops a jerk nystagmus with the fast phase to the right. * **Option B (True):** By definition, the nystagmus is "latent." It is either absent or minimal when both eyes are open but **increases significantly upon monocular occlusion**. * **Option C (True):** Patients with LN often find a **null point (zone of minimum intensity) in adduction**. This explains why children with infantile esotropia often adopt a compensatory head turn towards the side of the fixing eye to keep that eye in an adducted position to improve visual acuity. **High-Yield Clinical Pearls for NEET-PG:** * **Manifest Latent Nystagmus (MLN):** Occurs in patients with strabismus where only one eye is fixing (the other eye is functionally suppressed). It behaves like latent nystagmus but is present even with both eyes open. * **Alexander’s Law:** Nystagmus intensity increases when the patient looks in the direction of the fast phase. * **Key Association:** Always link **Latent Nystagmus + DVD + Inferior Oblique Overaction** with **Infantile Esotropia**. * **Management:** Usually requires no specific treatment for the nystagmus itself; management focuses on correcting the underlying strabismus and amblyopia.

Practice by Chapter

Amblyopia

Practice Questions

Esotropia

Practice Questions

Exotropia

Practice Questions

Vertical Deviations

Practice Questions

Special Forms of Strabismus

Practice Questions

Nystagmus in Children

Practice Questions

Pediatric Cataract

Practice Questions

Retinopathy of Prematurity

Practice Questions

Pediatric Glaucoma

Practice Questions

Pediatric Neuro-ophthalmology

Practice Questions

Genetic Eye Diseases in Children

Practice Questions

Pediatric Ocular Trauma

Practice Questions

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