Chapter 6 Pediatric Ophthalmology and Strabismus
6.1 Questions
Easy | Medium | Hard |
1. (Medium) A 2-year-old girl presents for strabismus evaluation. The child has prominent epicanthal folds and is diagnosed with pseudostrabismus. On dilated fundoscopic examination, the optic nerves are found to have a cup-to-disc ratio of 0.6 in both eyes with otherwise normal appearance. The intraocular pressures are normal, and corneas are clear with normal corneal diameters. What component of the patient’s medical history is most consistent with her examination findings?
The child was born at 42 weeks and required resuscitation for less than 1 minute at birth due to meconium aspiration and was subsequently discharged after 48 hours.
There is a family history of glaucoma, and this child has infantile glaucoma.
The child was born at 28 weeks and had a history of intraventricular hemorrhage.
The child was born full term and was hospitalized at 3 weeks of age for fever when sepsis was ruled out.
2. (Easy) A 5-year-old girl is referred for an eye examination due to a failed vision screening at school. The school form from the nurse’s office states that the child has failed stereopsis testing. Why the testing of fusion and stereoacuity should be performed first during the eye examination?
Testing of visual acuity and cover testing for strabismus may dissociate a patient with tenuous stereoacuity and underestimate the patient’s fusion.
Stereoacuity testing is the only part of the examination that needs to be assessed with accuracy because of the reason for the referral.
Stereoacuity testing is the most engaging part of the examination for a child.
Strabismus measurements will be inaccurate if done prior to stereoacuity testing.
3. (Medium) When evaluating the visual acuity of a preverbal infant, CSM (central, steady, and maintained) is one method of characterizing a patient’s fixation behavior. Which section of this method is tested under binocular conditions?
Steady
Central and maintained
Maintained
All three sections
4. (Medium) A 12-year-old healthy girl with nystagmus is reported by the technician to have decreased visual acuity of each eye compared to last year’s examination. You recheck the visual acuity and find that it is actually unchanged. The remainder of the examination is stable, and there have been no new concerns voiced by her or her family. What is a potential explanation for this discrepancy in visual acuity at the same visit?
Technician checked vision only under binocular conditions.
You used a high plus power lens to blur the fellow eye during testing.
Technician used preverbal optotypes (i.e., Teller cards, matching optotypes).
You used optotypes with crowding bars.
5. (Medium) A 10-year-old boy being treated for attention deficit disorder is referred to the eye clinic for concerns about difficulty with reading. He tells you that he needs glasses. Cycloplegic retinoscopy reveals +0.75 D of hyperopia in both eyes. What diagnostic testing can be done to determine if glasses may be helpful for the patient?
Assess visual acuity at near before dilation.
Refer the patient for comprehensive neuropsychological testing.
Dynamic retinoscopy.
No further testing is needed as he does not need glasses.
6. (Easy) A 4-year-old girl with anisometropic amblyopia is not tolerating treatment of amblyopia with patching therapy. Atropine 1.0% as an eyedrop is suggested as an alternative treatment option for amblyopia. The child’s father inquires about possible side effects of the drop. Which one of the following would you inform him?
There are no adverse effects of atropine if given as an eyedrop.
Possible side effects include fever, dry mouth, flushing of the face, tachycardia, delirium, and dizziness.
If severe adverse effects occur, accidental ingestion should be considered, and the child should be taken to the emergency room for evaluation and possible treatment with physostigmine.
Both B and C.
7. (Easy) A 20-year-old woman is referred for a complete eye examination due to a history of strabismus. She states that she has a history of “infantile esotropia” and underwent strabismus surgery at 2 years of age. What was the likely age of onset of this patient’s strabismus?
Two years old.
Less than 12 months of age.
Less than 6 months of age.
The age of onset cannot be determined.
8. (Hard) A 2-year-old girl is referred to your office from the emergency room for vomiting and development of a new left head turn. She is esotropic in primary gaze. An MRI of the brain done in the emergency room shows a mass in the left pons. What other examination findings may be present?
Incomitant esotropia that is larger in right gaze
A large secondary esotropia with fixation of the left eye
Limitation of adduction in the right eye
Limitation of abduction in the right eye
9. (Medium) A 56-year-old woman presents with a longstanding alternating exotropia. She states that she had eye surgery in childhood to straighten the eyes but does not recall the type of strabismus she had. She has no history of diplopia. She also had pterygium excision surgery in both eyes 5 years ago. External and slit lamp examinations show conjunctival scarring both nasally and temporally in both eyes. What intraoperative finding would confirm the diagnosis of consecutive exotropia?
Lateral rectus muscles found to be inserted 14.5 mm posterior to the limbus
Medial rectus muscles found to be inserted 5.5 mm posterior to the limbus
Medial rectus muscles found to be inserted 12 mm posterior to the limbus
Lateral rectus muscles found to be inserted 7 mm posterior to the limbus
10. (Easy) The insertions of which muscles comprise the spiral of Tillaux in order of proximity to the limbus (closest to farthest)?
Medial rectus, inferior rectus, superior rectus, lateral rectus
Inferior rectus, medial rectus, superior rectus, lateral rectus
Medial rectus, inferior rectus, lateral rectus, superior rectus
Inferior rectus, medial rectus, lateral rectus, superior rectus
11. (Medium) Which of the following are correct statements about the anatomy of the extraocular muscles?
The superior rectus forms a 51-degree angle with the visual axis; the tertiary action of the superior oblique is abduction; the superior oblique passes inferior to the superior rectus muscle.
The superior rectus forms a 23-degree angle with the visual axis; the tertiary action of the superior oblique is abduction; the superior oblique passes inferior to the superior rectus muscle.
The superior rectus forms a 23-degree angle with the visual axis; the tertiary action of the superior oblique is depression; the superior oblique passes inferior to the superior rectus muscle.
The superior rectus forms a 23-degree angle with the visual axis; the tertiary action of the superior oblique is depression; the superior oblique passes superior to the superior rectus muscle.
12. (Hard) How many bellies can the inferior oblique muscle have?
One
Two
Three
All of the above
13. (Medium) A 62-year-old man presents with a large right exotropia following endoscopic ethmoid sinus surgery. He has −5 limitation of adduction of the right eye and is diplopic. What is the primary limiting factor to surgically improving the eye alignment?
The medial rectus muscle cannot be identified on imaging due to the presence of blood and scar tissue.
The medial rectus muscle has no direct anatomic relationships with other extraocular muscles.
Only the lateral rectus has a direct anatomic relationship with the inferior oblique muscle.
The superior oblique muscle is composed of tendon anteriorly.
14. (Medium) Which arterial branches give rise to the anterior ciliary arteries?
The lateral and medial muscular branches
The lacrimal artery
The infraorbital artery
The vortex artery
15. (Medium) A 63-year-old woman undergoes strabismus surgery, including left inferior rectus muscle recession, for thyroid ophthalmopathy, which is causing diplopia. A large recession is required to resolve the diplopia in primary gaze. The patient should be made aware of what potential postoperative finding?
A persistent but comitant strabismus
Severe inflammation of the anterior segment
Left lower lid retraction and change in the palpebral fissure
Left lower lid elevation and change in the palpebral fissure
16. (Hard) In the first year of life, the dimensions of the eye undergo rapid changes including which of the following?
Decrease in axial length
Flattening of keratometry
Thickening of the cornea
Increase in crystalline lens power
17. (Hard) Based on the natural history of the refractive state in early childhood, how can you counsel the family on a 6-year-old boy with a refractive error of +0.25 D?
The refractive error will not change as the child gets older.
The child may become significantly more hyperopic as he gets older.
The child may become myopic as he gets older.
The future refractive state of the child cannot be predicted.
18. (Medium) Which eye movement findings are normal in a 3-month-old infant?
Constant strabismus
Elevation deficit
Adduction deficit
Nystagmus
19. (Hard) By what age is visual acuity estimated to reach 20/20?
By 6 to 7 months of age based on visually evoked potential (VEP) studies
By 3 to 5 years of age based on method illustrated in the figure
By 6 to 7 years of age based on optotype testing
Both A and B
20. (Hard) A 4-year-old girl presents with an anomalous head position and history of strabismus surgery for a right superior oblique paresis. You confirm the diagnosis on your examination. Her mother states that the head tilt initially improved after surgery but has recurred and worsened. She does all activities, including running, with a large left head tilt. Intraoperatively, forced ductions reveal 4+ tightness of the right inferior oblique and 1+ laxity of the right superior oblique. You identify the right inferior oblique inserted 2 mm posterior to the right inferior rectus insertion. What surgical procedure should be performed for this patient at this time to improve the head position?
Further recess the right inferior oblique muscle.
Advance the right inferior oblique to the original insertion.
Recess the right inferior rectus muscle to the level of the current inferior oblique insertion.
Perform denervation and extirpation of the right inferior oblique muscle.
21. (Hard) A 23-year-old woman with a history of infantile esotropia and strabismus surgery in early childhood presents to discuss treatment options for her eyes, which frequently drift up and out. Visual acuity is 20/25 in both eyes. On examination, you find a large left dissociated vertical deviation (DVD) that manifests without occlusion, as well as a small right DVD that is only evident with occlusion. There is 2+ inferior oblique overaction of both eyes. What surgical treatment would you recommend?
Left superior rectus muscle recession
Left inferior oblique muscle recession and anteriorization
Bilateral inferior oblique muscle anteriorization
Right inferior oblique muscle recession and anteriorization
22. (Medium) A 9-year-old boy presents with high accommodative convergence-to-accommodation (AC/A) ratio esotropia (diagnosed when he was 2 years old) as mother would like a second opinion. She is concerned about whether he still needs his glasses. He appears to be orthotropic in his glasses. How would you counsel the mother?
Proceed with strabismus surgery with posterior fixation sutures or bilateral medial rectus muscle recessions.
If the child is orthotropic in bifocals and tolerates spectacle wear, no change in management recommended.
Promptly discontinue his glasses as he is older now.
Convergence exercises.
23. (Hard) A 79-year-old woman presents with oblique diplopia at distance and is found to have a 30∆ esotropia and 14∆ right hypertropia. Motility is full in both eyes. Torsion is detected neither on the fundus examination nor with double Maddox rod. Neuroimaging is normal. She strongly prefers that surgery only be done on one eye and that she can continue to wear glasses for reading only. What is the best surgical option for this patient?
Right medial rectus muscle recession, right lateral rectus muscle resection, right superior rectus muscle recession.
Inform the patient that bilateral medial rectus muscle recession is the only treatment option, and she will need prism glasses after surgery.
Right medial rectus muscle recession, right lateral rectus muscle resection with infraplacement of the muscles.
Right medial rectus muscle recession, right lateral rectus muscle plication, right superior rectus muscle recession.
24. (Medium) A 20-year-old man presents with exotropia and undergoes strabismus surgery with bilateral lateral rectus muscle recessions. At postoperative week 1, the patient has a small esophoria in primary gaze. He complains of intermittent diplopia and would like to know what can be done about the diplopia. What do you tell him?
One of the risks of strabismus surgery is diplopia, of which he was informed preoperatively and nothing can be done to improve the diplopia at this time.
One of the risks of strabismus surgery is diplopia, but in his case, the diplopia will likely improve over time, and monitoring is recommended.
Prism glasses or a temporary prism will negatively affect the long-term outcome of the surgery and is not recommended.
Repeat surgery is recommended at this time.
25. (Medium) A 12-year-old boy undergoes strabismus surgery using a fornix incision for intermittent exotropia. At postoperative week 1, the patient is brought to the eye clinic for redness, tearing, irritation, and mild lid edema of both eyes. He does not complain of pain. Visual acuity is unchanged, slit lamp examination demonstrates clear corneas, quiet anterior chamber, and fundus examination is normal. What is the likely etiology of the findings?
Allergy to the sutures
Dellen formation
Endophthalmitis
Severe conjunctival scarring
26. (Medium) An 8-year-old boy undergoes strabismus surgery for esotropia. Intraoperatively, the patient’s cardiopulmonary monitor shows the following. What should be done next?
Cancel the surgery for the patient to undergo a full cardiac work-up.
Ask the anesthesiologist to administer an antiemetic medication.
Pause the surgery until the heart rate normalizes.
Proceed with the surgery uninterrupted.
27. (Easy) A 34-year-old woman presents with intermittent esotropia of 12∆ associated with diplopia. She demonstrates excellent stereopsis at near. Motility is full in both eyes with good visual acuity and normal dilated fundus examination. She strongly prefers not to undergo incisional strabismus surgery and does not want to wear glasses. She asks if botulinum toxin can be used as a treatment alternative. What do you tell the patient about this treatment option?
Risks of the procedure include ptosis, overcorrection, development of vertical strabismus after injection of a horizontal muscle, all of which are permanent.
Scleral perforation is not a risk of this procedure, as it is with incisional surgery.
Repeated injections may be needed, but the injected muscle may lengthen while it is paralyzed and provide long-term improvement of alignment.
Only incisional surgery is recommended at this time.
28. (Medium) A 4-year old girl presents with esotropia of 40 prism diopters at near and 20 prism diopters at distance with visual acuity of 20/60 in the right eye and 20/30 in the left eye. Motility is full. Cycloplegic refraction is +4.00 +3.00 × 90 in the right eye and +3 in the left eye. Eye examination is otherwise unremarkable including dilated fundus examination. Which one would be the most important recommended next step?
Brain MRI
Plano sphere +3.00 add bifocals
Patching
Full cycloplegic refraction
29. (Medium) A 6-year-old boy presents with a diagnosis of strabismic amblyopia and 40 prism diopters of esotropia at distance and 40 prism diopters of esotropia at near. Visual acuity is 20/20 in the right eye and 20/200 in the left eye with no improvement in spite of 6 h/d patching for 2 years. Cycloplegic refraction is +1.00 in each eye. You obtain an optic nerve optical coherence tomography (OCT) of the left eye which reveals an optic nerve diameter of 400 µm. Which of the following would be appropriate next step?
Full cycloplegic refraction
Bifocals
Endocrine consult
Resume patching 6 h/d
30. (Easy) In a child with septo-optic dysplasia, a brain MRI is likely to show which of the following findings?
Abnormal decussation of the optic nerve fibers
Absent septum pellucidum
Optic nerve glioma
Basal encephalocele
31. (Medium) A child with severe optic nerve hypoplasia of the left eye presents with 40 prism diopters of esotropia at distance and near and visual acuity of 20/20 in the right eye and 20/200 in the left eye. What surgical intervention would be recommended to address the strabismus?
Left medial rectus recession, left lateral rectus resection, because the left eye is the amblyopic eye and would therefore minimize risk.
Right medial rectus recession, right lateral rectus resection, because the right eye is the functioning eye therefore it is most likely to benefit from surgery.
Bilateral lateral rectus recession.
Strabismus surgery is unlikely to be beneficial in a case of unilateral optic nerve hypoplasia.
32. (Hard) A 2-year-old girl presents with esotropia since birth with a small left face turn. Her nine cardinal positions of gaze are shown in the figure. Which of the following is true about this child’s condition?
She likely has a small exotropia in right gaze.
She likely has poor fusion.
Her right cranial nerve VI nucleus is likely absent.
Her left cranial nerve III nucleus is firing during left gaze.
33. (Hard) An 11-year-old otherwise healthy boy presents with 2 years of progressive ptosis of the left upper eyelid with a left hypotropia of 30 prism diopters and now ptosis of the right upper eyelid. He has a −3 supraduction deficit of the left eye and −1 supraduction deficit of the right eye. Pupils are equal. His findings do not fluctuate, his myasthenia panel is negative, and a rest test is negative. An MRI of the brain and orbit is unremarkable and a trial of high-dose corticosteroids does not improve his signs or symptoms. What do you consider the most reasonable next step?
Cerebral angiogram
Plasmapheresis
Electrocardiogram
Thyroid function tests
34. (Medium) An 18-month old boy presents to your clinic with the following strabismus examination and another pertinent clinical examination finding. He has symmetric mild proptosis consistent with shallow orbits. What is likely true about this patient?
He has Crouzon’s syndrome with associated craniosynostosis and syndactyly.
He has significant risk of optic nerve coloboma.
Elevated intracranial pressure is unlikely to result in papilledema.
He has a V-pattern strabismus due to rotated configuration of his extraocular muscles within his orbits.
35. (Hard) A 6-year-old boy presented with 1 month of the left eye turning inward along with a face turn to the right. He had an upper respiratory infection prior to onset of the ocular symptoms. On his sensorimotor examination, he had 20 to 25 prism diopters of esotropia in primary gaze at near with 30 prism diopters of esotropia at distance. He had a −2 to −3 abduction deficit of the right eye. His optic nerves had cupping 0.55 on the right and 0.7 on the left with no pallor or edema. Intraocular pressures were normal. His brain MRI is shown in the figure. What is likely to be true about this patient?
He likely had a left cranial nerve VI palsy due to a brain tumor.
He likely had an acute acquired comitant esotropia.
He likely had a viral cranial nerve VI palsy of the right eye.
He likely had a right cranial nerve VI palsy due to a brain tumor.
36. (Hard) A 24-year-old woman with long-standing esotropia presents with crossed diplopia. You perform cover testing and identify 30 prism diopters of esotropia. The afterimage test results are shown in the figure part A. Which of the following is true about this patient?
This patient has harmonious anomalous retinal correspondence.
This patient has paradoxical diplopia.
This patient has a dragged macula.
This patient has a pseudofovea located temporal to the actual fovea.
37. (Hard) Among images shown in figure parts A to C, what likely represents what a healthy 3-month-old infant sees?
Image A.
Image B.
Image C.
The estimated vision of a 3-month-old infant is not known.
38. (Medium) A 6-month-old ex-27-week gestational age male infant presents with exotropia. He had been screened for retinopathy of prematurity while in the neonatal intensive care unit, however, failed communication between the neonatal intensive care unit staff and the ophthalmology office led to his being lost to follow-up. Though no movement is seen on alternate cover testing with excellent fixation, he has 35 prism diopters of exotropia based on the Krimsky testing. Dilated fundus examination of the left eye is shown in the figure. (courtesy of Francine Baran, MD). Which of the following is most accurate about this patient?
This patient has anomalous retinal correspondence.
This patient has eccentric fixation.
This patient has a positive angle kappa.
This patient has exotropia.
39. (Hard) A 10-year-old boy underwent bilateral medial rectus recession when he was 1-year-old. He now presents with 5 prism diopters of constant esotropia. Visual acuity is 20/20 in the right eye and 20/25 in the left eye, and worth four dot testing at near is shown in part A of the figure and worth four dot testing at distance is shown in part B of the figure. What is most likely to be true about this patient?
This patient has absent stereopsis.
This patient would benefit from strabismus surgery.
This patient has a suppression scotoma in monocular viewing conditions.
This patient has partial stereopsis.
40. (Medium) A 9-month-old male infant with trisomy 21 presents with torticollis is shown in the figure. What would be an appropriate test to perform on this patient to confirm a congenital cranial nerve IV palsy?
Double Maddox rod
4 prism diopter base-out test
Hirschberg’s test in right head tilt
Single Maddox rod in left gaze
41. (Hard) A 7-year-old boy presents to your clinic with intermittent exotropia of 35 prism diopters at distance and orthophoria at near. You perform a 60-minute patch test to maximally dissociate his eyes. Immediately after removing the patch, you begin to perform alternate cover testing at near. What result would most closely correspond with tenacious proximal fusion?
35 prism diopters of exophoria.
Orthophoria.
15 prism diopters of esotropia.
This test cannot diagnose tenacious proximal fusion.
42. (Easy) A 2-year-old girl is poorly cooperative in the clinic and will not tolerate cover testing; however, her parents are convinced that she crosses her eyes. Looking at her picture in the figure, what would you say is most likely true about this patient?
Pseudoesotropia only
Esotropia
Esophoria
Exotropia
43. (Hard) A 4-year-old girl is undergoing evaluation for strabismus surgery. Her nine cardinal positions of gaze are shown in the figure. What surgical options would be most appropriate?
Left lateral rectus recession and left medial rectus resection with bilateral superior oblique tenotomies
Bilateral lateral rectus recession with inferior transposition
Bilateral medial rectus recession with inferior transposition
Right lateral rectus recession and right medial rectus resection with bilateral inferior oblique myectomies
44. (Hard) A 10-year-old girl presents to your clinic with poor vision since birth and a previous diagnosis of optic nerve hypoplasia. She underwent bilateral medial rectus muscle recessions at 1 year of age for infantile esotropia. On examination, her visual acuity is 2/400 in each eye with a large angle jerk nystagmus. The optic nerve appears normally sized, and the fundus examination is unremarkable. The electroretinogram is shown in the figure. Which of the following is likely associated with this child’s symptoms?
Optic nerve telangiectasias
Hypopituitarism
Autosomal recessive inheritance
Progressive external ophthalmoplegia
45. (Hard) A 3-year-old ex-25-week premature infant has been diagnosed with retinopathy of prematurity, infantile exotropia, and blindness. He has never undergone retinal surgery or laser. Parents state that he has never been able to see since birth. Patient is alert and interactive. His visual acuity appears to be light perception only with either eye. Fundus examination is unremarkable in both eyes with normally sized optic nerves, and nystagmus is absent. His optical coherence tomography (OCT) is shown in the figure. Which of the following is the most likely diagnosis?
Cortical visual impairment
Optic nerve hypoplasia
Stage V retinopathy of prematurity
Leber’s congenital amaurosis
46. (Easy) A 3-year-old boy with no significant family history of blindness has been diagnosed with Usher’s syndrome based on sensorineural deafness and vestibular abnormalities. His visual acuity is 20/40 binocularly using Allen optotypes, but he does not tolerate monocular testing. He has an intermittent exotropia of 30 prism diopters, and his fundus examination is unremarkable. His cycloplegic refraction is +1.00 sphere in each eye. He has no nystagmus. Which of the following would be a reasonable next step for diagnostic testing?
Double Maddox rod
Genetic testing
Brain CT
Fluorescein angiogram
47. (Hard) A 2-month-old infant presents to your clinic with absent fix and follow response as well as intermittent, variable exotropia. His examination is unremarkable except for variable exotropia with a normal dilated fundus examination and absence of nystagmus. The infant fixes and follow your face but no toys or other objects. At what age would you consider obtaining additional work-up if vision does not improve?
Now
3 to 5 months old
6 to 9 months old
3 to 4 years old
48. (Hard) A 2-year-old girl presents with “abnormal movements” of her eyes since birth. Which of the following features distinguishes congenital motor nystagmus from fusion maldevelopment nystagmus syndrome (aka latent nystagmus)?
Nystagmus reverses direction depending on which eye is occluded.
There is a null point with corresponding head posture.
Nystagmus increases with an optokinetic nystagmus (OKN) drum moving in the opposite direction to the fast phase.
The velocity of the slow phase exponentially increases with distance from fixation.
49. (Hard) A 6-month-old infant presents with bilateral, horizontal nystagmus and normal-appearing anterior and posterior segment examination. Pupillary examination revealed the findings shown in the figure. What other testing may aid in the diagnosis?
Fluorescein angiography (FA)
Electroretinogram (ERG)
Magnetic resonance imaging (MRI)
Urine test for homovanillic acid (HVA) and vanillylmandelic acid (VMA)
50. (Medium) A 15-month-old child presents to you with a small amplitude, high-frequency binocular nystagmus present for the past 6 months, right head tilt, and head nodding when attempting to follow a target. What would be an appropriate next step in management?
Baclofen
Eye muscle surgery
Urine test for homovanillic acid (HVA) and vanillylmandelic acid (VMA)
MRI neuroimaging
51. (Easy) Which of the following is uncommon in patients with infantile esotropia with cross-fixation?
Amblyopia
Inferior oblique overaction
Dissociative vertical deviation
Latent nystagmus
52. (Medium) A rambunctious 3-year-old boy presents to your clinic with a 25∆ comitant esotropia. Cycloplegic refraction was limited but showed roughly +3.00 diopters of hyperopic error in both eyes. The full hyperopic error was prescribed, and the patient returned for follow-up with good compliance. However, he continues to demonstrate 12∆ of intermittent esotropia. What would be the next step in his management?
Bilateral medial rectus recessions
Bilateral lateral rectus recessions
Repeat cycloplegic refraction
Alternate patching with close follow-up
53. (Medium) A 4-year-old girl with accommodative esotropia presents for follow-up. With her +4.50 D lenses on, she has a 20∆ alternating esotropia. When she takes her glasses off, the deviation increases to a 45∆ esotropia. Repeat cycloplegic refraction reveals that she is wearing her full hyperopic correction. What would be the next step in her management?
Bilateral medial rectus recession 3.5 mm
Bilateral medial rectus recession 5.5 mm
Bilateral lateral rectus recession 5 mm
Bilateral lateral rectus resection 7 mm
54. (Easy) Two weeks after bilateral medial rectus muscle recession surgery, a patient’s parent urgently sends you the following picture of his eyes. On examination, there is significantly limited adduction of the right eye. Forced ductions were intact. Which of the following is the most likely diagnosis?
Anterior segment ischemia
Slipped muscle
Adherence syndrome
Consecutive exotropia
55. (Hard) A 7-year-old boy comes to your clinic with his parents who report that occasionally his eye “drifts out” when he is tired. On your sensorimotor examination, he has the following findings: X(T) 45∆ and X(T)’ 15∆. The near deviation remains the same with a +3.00 lens but increases to X(T)’ 37∆ after 1 hour of monocular occlusion. What is the diagnosis?
Intermittent exotropia with convergence insufficiency
Intermittent exotropia with true divergence excess
Intermittent exotropia with tenacious proximal fusion
Intermittent exotropia with a high accommodative convergence to accommodation (AC/A) ratio
56. (Medium) Which of the following is not typically utilized in the management of intermittent exotropia?
Orthoptic exercises
Overminus glasses
Alternate patching
Base-out prisms ground into spectacles
57. (Medium) Which of the following is the most common pattern of strabismus in patients with craniosynostosis?
A-pattern exotropia
V-pattern exotropia
A-pattern esotropia
V-pattern esotropia
58. (Medium) A 9-year-old boy presents with the following examination. Forced duction testing in the office was noted to be negative in both eyes. Which of the following would be the best initial surgical option?
Left superior rectus recession
Left inferior rectus recession
Left frontalis sling surgery
Left medial rectus and lateral rectus superior transposition
59. (Hard) A 4-year-old girl presents with the following examination. Which of the following is the most likely diagnosis?
Brown’s syndrome
Superior oblique palsy
Monocular elevation deficiency
Superior rectus palsy
60. (Hard) You have been following a 5-year-old male patient who presents with the following motility examination since infancy. He has excellent stereo-acuity with a compensatory head posture. Which of the following is his most likely head posture?
Right head tilt
Left head tilt
Right face turn
Chin down
61. (Easy) A 65-year-old woman presents with left hypotropia and the following CT findings. Which of the following tests would most likely be abnormal in this patient?
Complete blood count
Liver function tests
Thyroid function tests
Bone marrow biopsy
62. (Medium) Which of the following is the most appropriate management for a patient with type I Duane’s syndrome who is orthotropic in primary gaze but esotropic in left gaze?
Observation
Left medial rectus recession and lateral rectus resection
Left superior rectus lateral transposition and medial rectus recession
Right superior rectus lateral transposition and medial rectus recession
63. (Medium) Which of the following is not a characteristic of Moebius’ syndrome?
Inability to smile
Absent pectoralis muscle
Deficiency of upgaze
Limited horizontal motility
64. (Easy) A 50-year-old woman presents with bilateral ptosis and limitation of movement of both eyes in all directions. She notes that she has had this problem since birth and it has been stable since then. Forced duction testing was positive for restriction. Which of the following is the most likely diagnosis?
Chronic progressive external ophthalmoplegia (CPEO)
Congenital fibrosis syndrome
Thyroid eye disease
Myasthenia gravis
65. (Easy) A 6-year-old boy presents with a swollen right eyelid for the past 2 days. His mother does not recall any inciting factors such as blunt trauma or insect bites. What finding on examination would make you concerned for orbital cellulitis?
Conjunctival swelling and injection
Warmth and erythema of the eyelid
Tense eyelid
Decreased ocular motility
66. (Medium) An 8-year-old boy who presents with mild erythema of the periorbital region with normal vision, intraocular pressure, color vision, pupils, and motility has been started on a course of oral Augmentin for presumed preseptal cellulitis.
His clinical appearance worsens over the next couple of days, and he now reports worsening pain with eye movement. What would be the next step?Switch to oral clindamycin.
Switch to oral cephalexin.
Add oral vancomycin for presumed methicillinresistant Staphylococcus aureus.
Send to ER for CT scan.
67. (Easy) A 6-month-old male infant presents urgently to your office because he has had sudden swelling and erythema of skin inferior to the medial canthus in his left eye. There is also tearing and some mucoid discharge in the affected eye. The child has also been running a low-grade fever at home. What do you suggest next for management of this patient?
Treatment with oral antibiotics
Hospital admission for IV antibiotics
Immediate nasolacrimal duct probing and irrigation
Dacryocystorhinostomy
68. (Medium) A 3-year-old girl was diagnosed with juvenile rheumatoid arthritis 3 months ago after having a couple of episodes of right knee pain and swelling. Blood testing reveals rheumatoid factor negative (RF−), antinuclear antibody positive (ANA+). No other joints have been affected. The pediatric rheumatologist has started her on Naprosyn. She has no ocular irritation or injection. How often should she undergo slit lamp examinations to rule out uveitis?
Every 3 months
Every 6 months
Every 6 months, sooner with ocular irritation or injection
Once a year
69. (Medium) A 5-year-old girl with antinuclear antibody positive (ANA+) and juvenile idiopathic arthritis (JIA) oligoarthritis presents for her 3-month follow-up for uveitis screening. She is asymptomatic. What is the most likely type of uveitis in her condition?
Vitritis
Retinitis
Nongranulomatous anterior uveitis
Mutton-fat keratic precipitates
70. (Medium) A 7-year-old girl is transferred to your clinic after moving from out of state. She is 5-foot 6 inches in height and has very long, slender extremities. She is wearing extremely thick glasses. Her slit lamp examination reveals bilateral lens subluxation superotemporally. Genetic testing will reveal an anomaly on what chromosome?
11
15
17
22
71. (Medium) You evaluate a patient for the first time who has superotemporal lens subluxation of both eyes. In addition to genetic testing, what is the most important diagnostic testing this patient should undergo next?
Complete blood count
Stress test
Echocardiogram
Spirometry
72. (Easy) A 10-year-old boy suffered blunt trauma to his right eye with a Nerf gun while playing at home. He presents to the emergency room, and slit lamp examination reveals a 1-mm layered hyphema with 4+ red blood cells in the anterior chamber and mild corneal edema of the affected eye. What should be part of the initial evaluation?
Dilated fundus examination
Intraocular pressure measurement
Fluorescein staining of the cornea
All of the above
73. (Easy) You have diagnosed an 8-year-old African American boy with a traumatic hyphema. His intraocular pressure in the affected eye is measured to be 28 mm Hg, and that in the unaffected eye is 16 mm Hg. In addition to treating the elevated intraocular pressure, which of the following laboratory tests is recommended in this patient?
Beta-thalassemia testing
Rapid plasma reagin
Antinuclear antibody titers
Sickle cell screen
74. (Hard) An 11-month-old male infant with a strong family history of a genetic condition presents for an evaluation because his mom has noticed drooping of his left upper eyelid for the past 3 months. He demonstrates normal and equal vision with each eye tested individually. He has a margin to reflex distance 1 of 4.5 mm on the right and 2.0 mm on the left. He has mild proptosis of the left eye with moderate resistance to retropulsion. There are no palpable masses of the periorbital region. Anterior segment and dilated fundus examination are unremarkable in either eye. Cycloplegic retinoscopy reveals moderate anisometropia. He has multiple flat, pigmented skin lesions on his trunk and extremities. An MRI of the orbits is ordered to further evaluate the proptosis, and the T1-weighted scan with fat suppression is depicted in the image. What is the next step in management?
Close observation
Orbital biopsy
Orbital debulking
Enucleation
75. (Easy) A 22-year-old man is diagnosed with vestibular schwannomas while being worked up for progressive hearing loss and tinnitus. A referral to genetics is placed. What chromosomal mutation is most likely to be found in his condition, and what is the most common mode of inheritance?
Chromosome 17, autosomal dominant
Chromosome 17, autosomal recessive
Chromosome 22, autosomal dominant
Chromosome 22, autosomal recessive
76. (Medium) What is the appropriate management of a child with mild tearing secondary to bilateral epiblepharon causing trichiasis but no corneal or conjunctival abrasions?
E. Close observation
F. Lubrication eyedrops every 2 hours
G. Quickert’s suture repair
H. Orbicularis muscle stripping surgery
77. (Hard) The pediatric inpatient team consults you for a 3-month-old female infant who was admitted after being found unresponsive at home. Her dilated fundus examination is illustrated in the figures. What is the mortality rate of a child with this diagnosis?
5%
30%
50%
70%
78. (Hard) An 8-month-old female infant presents with a lesion on the right upper eyelid. Her mother has noticed the bump on the central right upper eyelid for the past several months. Warm compresses and lid hygiene are recommended. After 3 months, her mother returns reporting no change in the lesion. Tissue is sent for pathology at the time of incision and drainage of this presumed chalazion. The pathology slide is shown in the figure. What is the next step in management?
Steroid injection
Surgical debulking of lesion
Topical timolol ointment
Systemic propranolol
79. (Hard) An 11-year-old boy presents urgently to clinic with history of blunt trauma to his left eye while on the trampoline. There was no loss of consciousness. On examination, he has a normal external examination, visual acuities, intraocular pressures, and unremarkable anterior segment and fundus examinations. The motility examination is challenging, as he reports reproducible eye pain and nausea when trying to elevate the left eye and refuses to cooperate with the motility examination. What is the next best step in evaluation?
Observation
Orbital imaging
Concussion work-up
Empiric treatment of pain and nausea
80. (Medium) A 6-year-old girl is referred to your office for evaluation of discoloration of her left upper eyelid. On examination, she has mild edema of her eyelid with moderate ecchymosis and 2+ ptosis of the left upper eyelid. She has normal visual acuities, intraocular pressures, anterior segment examination, and fundus examination. Motility is full in both eyes, and she does not complain of any ocular pain or discomfort. Parents deny witnessing any recent trauma to the eye. There are no palpable masses in the periorbital region. She does not have any bruises on the remainder of her body, and she has not been more fatigued than usual. What is the next best step in her work-up?
Observation.
Orbital imaging.
Start oral antibiotics.
Obtain complete blood count.
81. (Medium) A 14-year-old adolescent boy presents to your office with history of seizures and developmental delay. On initial glance at the patient, you notice that he has multiple hypopigmented macules on his extremities and what appears to be severe facial acne. What is the most likely finding on his ocular examination?
Retinal coloboma
Astrocytic hamartoma
Myelinated nerve fiber layer
Lisch nodules
82. (Easy) What is the most common genetic mode of inheritance for tuberous sclerosis?
Autosomal dominant
Autosomal recessive
X-linked dominant
Sporadic
83. (Easy) A 5-year-old girl with history of chronic kidney disease is referred for significant light sensitivity. Her best corrected visual acuity is 20/25 with both eyes. Slit lamp examination reveals significant iridescent corneal crystals in both eyes. The posterior examination is limited by her inability to cooperate given significant photophobia, but brief views of the fundus reveal a grossly normal posterior pole in both eyes. Which of the following is the best potential treatment to offer her?
Oral cysteamine
Topical cysteamine every hour
Deep anterior lamellar keratoplasty
Serum copper and ceruloplasmin test
84. (Medium) A 10-year-old boy is referred to your clinic by an optometrist for irregular pupils. His past medical history is significant for developmental delay, short stature, and umbilical hernia repair. On examination, his is vision is 20/60 in the right and 20/40 in the left eye with his −6.00 +4.00 × 113 OU glasses. His eye pressure is 32- and 35 mm Hg in the right and left eye, respectively, by Icare. His anterior segment examination is shown in the figure. Which gene mutation is identified with this disorder?
PITX2
PAX6
ACTA2
IKBG (previously NEMO) gene
85. (Hard) A 5-year-old girl presents to your clinic with a “white bump” on her left eye, which has been present since birth. Her local optometrist has been treating her with glasses (+0.25 plano OD; −1.00 +4.00 × 65 OS) and patching. Her family is interested in surgical removal of the lesion. They also note that she has had a head tilt her entire life. On examination, her best corrected visual acuity is 20/20 and 20/25 with her right and left eye, respectively. Given the diagnosis of her corneal lesion and its most common associated type of strabismus, what would you expect on sensorimotor examination?
Chin-up position and small right head turn
Left head turn
Right head tilt
Left head tilt
86. (Medium) A 10-year-old healthy child with a past medical history of skin rash 2 years ago presents with anisocoria. Mom reports that the anisocoria is more obvious when the child is watching television and less noticeable when reading his tablet. On examination, vision is 20/20 OU. The anterior segment is normal. Under lighted conditions, the right eye pupil measures 5 mm in diameter and left pupil 3 mm; in dim light, the right pupil is 6 mm and the left pupil is 5.5 mm. Based on the history and examination, what would you expect the results of your testing to be?
No testing; physiologic.
After 0.01% pilocarpine instillation OU, OD 2 mm and OS 3 mm.
After apraclonidine instillation OU, OD 3 mm OS 4.5 mm.
MRIs of brain, neck, chest, and abdomen reveal neuroblastoma.
87. (Medium) A 4-week-old newborn is referred to you for port-wine stain involving the right face including the right upper and lower eyelids. Visual behavior is deemed appropriate with either eye occluded. Intraocular pressure is 30 mm Hg in the right eye and 12 mm Hg in the left eye. The anterior segment is notable for a corneal diameter on the right of 12 mm and 10 mm on the left. The posterior examination is shown in the figure. Her intraocular pressure of the right eye remains elevated on maximum glaucoma medications. The decision is made to provide glaucoma surgery. Because of her syndrome, what particular surgical complication is she at risk for?
Intraoperative or postoperative exudation or hemorrhage
Difficult intraoperative visibility due to shallow anterior chamber
Robust postoperative inflammation
Endophthalmitis
88. (Easy) Primary congenital glaucoma (PCG) is the most common nonacquired glaucoma of childhood. It most commonly presents in the first year of life. What constitutes the classic clinical triad of PCG?
Epiphora, photophobia, blepharospasm
Epiphora, photophobia, buphthalmos
Blurry vision, photophobia, blepharospasm
Epiblepharon, blepharospasm, buphthalmos
89. (Medium) A 1-year-old boy with unilateral buphthalmos and elevated intraocular pressure (IOP) is scheduled for his examination under anesthesia and likely goniotomy in 1 week. You want to start him on topical drops to lower the IOP as much as possible to keep the cornea clear for surgery and prevent further glaucomatous damage. Which medication should be avoided in this child?
Betaxolol
Dorzolamide
Pilocarpine
Brimonidine
90. (Medium) You are called to the NICU to examine this 1-week old newborn baby. The nurses have not placed any dilating drops. Careful screening must be performed to rule out which of the following systemic diseases?
Retinoblastoma
Wilms’ tumor
Pheochromocytoma
Rhabdomyosarcoma
91. (Medium) A 12-year-old girl is referred for progressive myopia by her optometrist. She is 20/30 OU in −5.00 sphere glasses. Her intraocular pressure is 27 mm Hg OD and 26 mm Hg OS. Her anterior segment examination is normal. Her posterior examination is normal except for 0.7 cup to disc ratio of bilateral optic nerves. Her father had surgery as a teenager for “high eye pressures” and is doing reportedly well, although he admits he has not been evaluated by an eye care provider in 5 years. The genetics of this child’s eye condition is most likely which of the following?
Autosomal dominant, CLC1A/myocilin gene
Autosomal recessive, CYP1B1
X-linked, PITX2
Autosomal dominant, FOXC1
92. (Hard) What are the most common risk factors for developing glaucoma following cataract surgery?
Microcornea and early age of cataract surgery
Microcornea and pseudophakia
Microcornea and aphakia
Aphakia and family history of glaucoma
93. (Hard) A 4-year-old boy referred for failed vision screen presents to the clinic for his first eye examination. Uncorrected visual acuity is 20/400 and 20/30 in the right and left eye, respectively. He has a small anterior polar cataract in the right eye. His examination is otherwise structurally normal. His refraction is +4.00 +2.50 × 90 in the right eye and +1.00 in the left eye. What is the most appropriate next step in the management of his condition?
Cataract surgery with an intraocular lens placement in the right eye
Amblyopia therapy with glasses and patching
TORCH laboratory screening
MRI brain and orbit scan
94. (Hard) A 1-year-old boy had bilateral cataract surgery at age 3 months for dense nuclear cataracts. He has not tolerated contact lenses and parents elect for spectacles. His cycloplegic refraction is +18.00 +0.50 × 90 OD and +17.00 + 1.00 × 90 OS. What is the appropriate glasses Rx for him?
+20.00 × +0.50 × 90 OD and +19.00 +1.00 × 90 OS
+18.00 +0.50 × 90 OD and +17.00 + 1.00 × 90 OS
+16.00 +0.50 × 90 OD and +15.00 + 1.00 × 90 OS
+18.00 OD and +17.00 OS
95. (Medium) Which of the following is a risk factor for developing uveitis in juvenile idiopathic arthritis (JIA)?
Oligoarthritis
Old age of onset of arthritis
Antinuclear antibody (ANA) negative
Rheumatoid factor (RF) positive
96. (Hard) A neonate is born at 31 weeks 3 days weighing 1,453 g. The NICU would like to know when the next eye examination should be scheduled. What would be the most appropriate time frame?
Does not need follow-up; born after 30 weeks.
Needs follow-up; examination as soon as possible as baby is born after 30 weeks.
Needs follow-up; examination in 1 month.
Needs follow-up; examination in 2 weeks.
97. (Medium) A 13-year-old adolescent girl is referred by her pediatrician after a failed vision screen. She is a refugee and has not received any health care previously. On examination, her vision is 20/20 and 20/200 in her right and left eye, respectively. Her examination is notable for optic nerve findings in the left eye shown in the figure. What is the next appropriate step in management?
Brain MRI and MR angiogram
Referral back to pediatrician to evaluate for CHARGE syndrome
Goldman’s visual field
MRI and referral to endocrinology
98. (Medium) A 3-month-old male infant presents to your office for “eye shaking” since birth. He was born at 39 weeks and his birth was complicated by a prolonged NICU admission for neonatal jaundice and seizures. A brain MRI was performed in the NICU, which demonstrated absence of the septum pellucidum and agenesis of the corpus collosum. You are most concerned about which of the following eye conditions?
Morning glory disc anomaly (MGD)
Optic nerve hypoplasia
Foveal hypoplasia
X-linked congenital stationary night blindness (CSNB)
99. (Medium) An 11-year-old boy is undergoing bilateral strabismus surgery for poorly controlled intermittent exotropia. The procedure, indication, risks, benefits, and alternatives are discussed with her mother, and she provides verbal and written consent to proceed with the procedure. At this time, what should the surgeon do?
Should proceed with scheduling the surgery.
Consent the child.
Ask the child what his thoughts and concerns are as he should be able to assent to the procedure.
Should refer to child psychiatry.
100. (Medium) A 5-year-old girl presents with 7 months of redness of the left eye. She is otherwise healthy. On examination, visual acuity is 20/20 with both eyes, and the bulbar conjunctiva of the left eye is diffusely injected. There is 1+ follicular conjunctivitis of the inferior palpebral lid. On closer examination of her left eye, there is a 2-mm elevated round umbilicated lesion along her left lower lid. Her condition is most likely caused by which of the following?
DNA pox virus
Adenovirus
DNA herpes virus
Chlamydia bacteria
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