1. Pediatric Ophthalmology
Julie M. Lange, MD
Assistant Professor
Division of Pediatric Ophthalmology

2. Primary Learning Objective
Recognize important preventable and treatable causes of blindness or vision loss in infants and children

3. Secondary Learning Objectives
define strabismus
define amblyopia
identify causes of amblyopia
define esotropia and exotropia
define suppression
understand the basis for treatment of amblyopia, strabismus and refractive error in childhood

4. Secondary Learning Objectives (continued)
interpret tests of ocular alignment including corneal light reflex and cover-uncover testing
recognize pseudostrabismus
interpret abnormalities of the red reflex
describe retinopathy of prematurity
recognize ophthalmologic features of child abuse
list common causes of leukocoria
identify abnormal ocular motility disturbances presenting in childhood

5. Red Reflex testing
This is a basic test easily done by any practitioner.
A direct ophthalmoscope is used to assess the red reflex in each eye. The examiner is looking for any opacities, irregularities or asymmetries in the red reflex. ANY abnormality needs to be referred to an ophthalmologist.

6. Examples of abnormal red reflexes
Retinoblastoma
Cataract dislocated lens
Upper left: leukocoria left eye upper right: central cataract in left photo, dislocated lens in right photo
Lower left: central opacity or cataract in lens lower right: asymmetric light reflexes, this child may have hyperopia in 1 eye and myopia in the other
this can lead to amblyopia
Congenital polar cataract
Anisometropia: unequal refractions

7. Vision Screening Terminology
“Blinks to Light”
“Fix and Follow”
Children’s Figures
HOTV chart
Snellen chart

8. Vision Screening Terminology
Blinks to Light
This term is used mostly to describe vision in newborn eye exams.
An infant is expected to blink to light at birth, but does not usually show consistent fixation behavior until 2-3 months of age.
Fix and Follow
By 4 months of age, most infants will fixate on an object and be able to follow it.
When checking fixation, have the patient fixate on an object with 1 eye at a time. It is best to use an object, rather than a light, to test vision because even a person with low vision can sometimes see lights well.

9. Vision Screening Terminology
Children’s Figures
A chart consisting of pictures such as a hand and a car which are familiar to most children.
Because the objects have very different shapes and are easily differentiated, this chart is not ideal.
HOTV Chart
A chart consisting of only 4 letters (H, O, T, V). It is often used in conjunction with a hand-held chart which the child can point to in order to indicate the letter on the wall chart. This is useful in preverbal children and in those who do not yet know the alphabet.

10. Vision Screening Terminology
Snellen Chart
Snellen refers to the traditional eye chart consisting of a variety of letters which is read aloud. This is the most accurate chart to use, but its use is obviously limited by the age and literacy of the child.

11. Refractive Error
Every child has a refractive error. If the refractive error is high, glasses are warranted.
Children have the ability to accommodate much more than adults, therefore small amounts of symmetric hyperopia are not corrected.
Also, small amounts of myopia are not usually corrected due to the fact that the majority of a child’s daily activities are near tasks.
Glasses can be given as early a 6 months old and can prevent and/or treat amblyopia.

12. Amblyopia= less than normal best-corrected vision in 1 or both eyes.
Causes include:
Large refractive error in both eyes
Difference in refractive error between the 2 eyes is called anisometropia. The more blurred eye will actually lose vision because the child starts to prefer the better seeing eye and only continues to develop vision in that eye.
Strabismus or misalignment of the eyes. The child will prefer 1 eye while the more frequently deviated eye will lose vision. This is done to avoid diplopia which is inevitably the result if the eyes are misaligned.
Structural abnormality of the eye can prevent a clear image and therefore proper visual development (cataract, corneal opacity, vitreous opacity, etc.)

13. Amblyopia Treatments
The first step is to put the child in the proper glasses if a significant refractive error exists
Patching of the better seeing eye several hours per day in order to force the use of the weaker eye
Cycloplegic eye drops can be used to blur the better seeing eye temporarily
In cases of a structural abnormality, it is sometimes possible to address the problem directly (cataract surgery, corneal transplant, etc.) These patients often need patching after their surgery as well.

14. Amblyopia Treatments
Amblyopia treatments work best when implemented early. Around the age of 9 years old, a child’s best-corrected vision is cemented.
If not treated, amblyopia can cause blindness.
If significant strabismus exists once equal vision is obtained, eye muscle surgery is warranted.

15. Patching is the most common treatment for amblyopia

16. Checking eye movements
Oculomotor exam
-Fixation with each eye individually
-Range of movement of each eye
-Steady? Look for nystagmus
-Straight? Look for strabismus
-Don’t forget to look at the pupils and eyelids

17. Ocular motility disorders that may present in childhood
Nystagmus
Cranial Nerve 6 palsy
Convergence Insufficiency
Strabismus
Esotropia
Exotropia
Hyper/Hypotropia

18. Nystagmus
Nystagmus is a term to describe fast, uncontrollable movements of the eyes.
1. Infantile: present at or soon after birth, can be associated with eye disease. Visual acuity in these patients is often nor correctable to 20/20.
2. Acquired:
Secondary to medication
Head injury
Inner ear disorder
Stroke
B12 deficiency
Multiple sclerosis
Brain tumors

19. Cranial Nerve 6 Palsy
CN 6 controls the lateral rectus extraocular muscle
In a CN6 palsy, there will be limitation to abduction in 1 or both eyes and a misalignment of the eyes in certain gazes
This can be congenital or acquired
It can be secondary to increased intracranial pressure or recent head trauma
This warrants urgent referral to an ophthalmologist and/or ER

20. Look for abnormal head positions
This can indicate
A null point for nystagmus
An avoidance of diplopia in CN 6 palsy
Eye preference in amblyopia
Hypertropia in head tilts

21. Strabismus Orthophoric: describes eyes that are aligned.
Strabismus is a misalignment of the eyes. It can be constant or intermittent. Strabismus is not normal and should be taken seriously.

22. Strabismus Exotropia (XT) eye drifts inward eye drifts outward
Esotropia (ET)
eye drifts
inward
Exotropia (XT)
eye drifts outward

23. Corneal light reflex testing
Assess alignment of eyes by having patient look at a penlight. The light reflex should fall within the pupil bilaterally.
Also called Hirshberg Testing

24. If the light reflex is asymmetric in 1 of the eyes, a misalignment may be present.
The light reflex on the child’s left eye shows the eye is turned inward (esotropia)

25. Orthophoric: eyes aligned Esotropia: L eye turned in
Exotropia: L eye turned out
Hypertropia: L eye drifted
upward

26. Alternate Cover Testing: 3 Steps to Detect Eye Deviation
Step 1: The “Cover Test"
Cover one eye while the patient fixates on an object (ie, toy, parent) During and after placement of the cover, the uncovered eye is observed for any movements that indicate the need to refocus.

27. Alternate Cover Testing: 3 Steps to Detect Eye Deviation
Step 2: The “Uncover Test"
Cover one eye, but focus the examination on the covered eye once the cover is removed. If the just-uncovered eye moves to regain focus, a strabismus is present.

28. Alternate Cover Testing: 3 Steps to Detect Eye Deviation
Step 3: The “Alternate Cover Test"
Occlude each eye in an alternating manner. If the affected eye moves in an attempt to regain fixation, a strabismus is present.

29. Cover Testing http://www.richmondeye.com/eyemotil.asp
This is an interactive webpage where the user can cover and uncover the eyes on the screen to see fixation/refixation movements indicating strabismus.

30. Pseudostrabismus
A false appearance of strabismus. It is most commonly seen in infants and toddlers who have a wide nasal bridge or epicanthal folds. These facial features can partially cover the medial part of each eye giving the appearance that the eyes are crossed inward when actually upon testing, the eyes are orthophoric.

31. Epicanthal folds causing pseudostrabismus
Epicanthal folds causing pseudostrabismus

32. look at the light reflexes
Pseudoesotropia
look at the light reflexes
Corneal light reflexes are centered and so this child is orthophoric. Her wide nasal bridge and epicanthal folds make her appear esotropic.

33. Pseudoesotropia
Again, wide nasal bridge and epicanthal folds give the appearance of esotropia. However, if you look at the light reflexes, you will see the child is orthophoric.

34. When testing a child’s fixation
When testing a child’s fixation. One should always be looking for eye preference. Children should be able to use either eye and not have a strong preference. If a preference is noted; this is a sign of amblyopia.
When strabismus is present, preference is easier to assess. The examiner should make certain the child can fixate and maintain fixation with either eye.
When strabismus is not present, the examiner must occlude each eye separately and test the fixation and ability to maintain fixation. Children who prefer one eye will strongly object to cover of that eye.

35. Congenital Esotropia Congenital Esotropia
Arises before 6 months of age
Typical characteristics include:
Large and constant
alternating/cross fixation
not significantly hyperopic, usually normal refractive error
full abduction
possible nystagmus, often the latent type

36. Cross-fixation in congenital ET
Babies with congenital esotropia will often cross fixate meaning instead of trying to use the eyes together, they will use the left eye for right gaze (upper photo) and the right eye for left gaze (lower photo). They can also change which eye they are using anytime and it can be hard to assess them. They usually can abduct both eyes, but it can be difficult to elicit this.
Cross-fixation in congenital ET

37. Esotropia
Esotropia arising after 6 months of age is usually classified as accomodative or non accomodative.
Accomodative: patient has a high hyperopic prescription and the eyes are aligned in glasses
Non-Accomodative: patient has low refractive error and glasses do not significantly change the alignment.

38. Esotropia
Esotropia is often associated with amblyopia and needs be treated right away.

39. ESOTROPIA Patient with left esotropia.
Photo courtesy of Gary Rogers, MD
ESOTROPIA

40. Esotropia much improved in glasses
Esotropia much improved in glasses. Therefore, this is Accomodative Esotropia.

41. Bifocals are sometimes used to add more plus lenses to the bottom of the existing prescription to help with more esotropia at near.

42. Exotropia: temporal deviation of the eyesXT
Less risk of amblyopia
Often begins as intermittent with some control
Can progress and become more constant

43. EXOTROPIA
Patient with right exotropia. Look at the light reflexes.

44. Exotropia
Exotropia is sometimes helped my myopic (nearsighted) glasses, but large deviations usually require strabismus surgery to correct.

45. Suppression
If strabismus arises in a child with binocular vision, the child experiences diplopia. Therefore, the child will usually suppress one eye as an adaptation to avoid diplopia. This often leads to Amblyopia.

46. Misalignment Mngt Quiz

47. Strabismus surgery
Basic concept is to weaken or strengthen an eye muscle
Methods to weaken:
recess (move the insertion of a muscle posteriorly)
insert a spacer into the muscle therefore effectively making the muscle longer
Botox
disinsertion
Methods to strengthen:
resect, tuck, advance

48. Methods to weaken muscle:
Recess: move the insertion of a muscle posteriorly
insert a spacer into the muscle therefore effectively making the muscle longer
botox
disinsertion
Methods to strengthen:
resect: excise part of the muscle, thereby making it shorter
Tuck: essentially folding a segment of muscle on itself and securing it in place, thereby tightening it
Advance: moving the insertion anteriorly

49. A recession (weakening) of the medial rectus is the surgery most often performed for the correction of non-refractive esotropia.
It is done by disinserting the muscle from the globe and then reattaching it to the globe posterior to its original insertion.

50. 1. An incision is made through conjunctiva and tenons fascia
1. An incision is made through conjunctiva and tenons fascia. The desired eye muscle is then located with a muscle hook.

51. Here the medial rectus muscle is isolated with a
muscle hook and then cleared of connective tissue.

52. The muscle is then secured with a double armed suture just anterior
to its insertion onto the globe.
4. The muscle is then excised from the globe with the sutures preventing loss of the muscle.

53. Calipers are then used to measure and mark a predetermined
distance from the original insertion of the muscle.

54. The muscle is then reattached to the globe using the double-armed suture already in place to make partial-thickness scleral passes at the marks made by the calipers.
7. Suture are then tied and cut. The conjunctiva is re-approximated and sometimes sutured.
8. The same procedure is often performed on the other eye.

55. Strabismus Surgery Video
This is a video of strabismus surgery. It shows medial rectus recession which is done for esotropia and is probably the most common eye muscle surgery done.

56. Vision Exam Quiz

57. Retinopathy of Prematurity
Retinopathy of prematurity (ROP) is a potentially blinding eye disorder that primarily affects premature infants weighing 1500 grams or less or with a gestation of less than 30 weeks.

58. ROP
Retinal blood vessels begin to develop approximately 3 months after conception and usually complete their development before the birth of a full term infant.
If an infant is born very prematurely, eye development is often not complete and the growth of the retinal vessels may be disrupted. The vessels may stop growing or grow abnormally from the retina into vitreous. These abnormal vessels are very fragile and bleed easily.

59. Retinopathy of Prematurity
The diagram shows abnormal blood vessel development in the superior retina. The white wavy line indicates where the retinal vessels stopped growing normally and abnormal growth begins. Between the white line and the ora serrata (the anterior edge of the retina) exists an avascular zone that is ischemic.

60. ROP
These babies are at high risk of retinal detachment. ROP can cause blindness. ROP exams are done on all babies who fall within the risk guidelines. Because there are no external signs of this diagnosis, parents need to be reminded of the importance of these exams.

61. ROP
Progression of ROP can lead to a retinal detachment. The scar tissue associated with the fibrovascular ridge contracts, pulling the retina away from the wall of the eye. Eyes with retinal detachments due to ROP are very difficult to operate on and these babies often have poor visual outcomes.

62. Depending upon the stage and zone of the ROP, treatment may be indicated in an attempt to prevent vision loss. The gold standard treatment is laser. It is used to photocoagulate the avascular retina to eliminate the hypoxia which drives the abnormal vessel growth.
The inferior part of the photo shows laser spots covering the peripheral retina in the avascular zone. The retina shown superiorly in the photo is normal.
Laser spots are applied confluently to the avascular retina between the ridge and the ora serrata.

63. Over the last 5-6 years, physicians have begun injecting anti-VEGF molecules into the eyes of babies with severe ROP. Their retinal vascular abnormalities often significantly improve after the injection. This is an off-label use of intravitreal bevacizumab. This drug is most commonly used for exudative macular degeneration.
However, there are still many questions surrounding bevacizumab It has many potential side-effects and our understanding of the short- and long-term effects of VEGF inhibition in newborns is extremely limited.
The implications of VEGF blockade in an infant in whom physiologic VEGF is required for normal organogenesis are not known.
Avastin in ROP
The anti-VEGF drug has shown promise in the treatment of retinopathy of prematurity.
Vishak J. John, MD • Ditte Hess, CRA • Audina M. Berrocal, MD
Article Date: 10/1/2012

64. ROP Quiz

65. Leukocoria
Leukocoria, literally "white pupil”, occurs when the pupil is pale yellow or white rather than the usual black
Leukocoria of the left eye.

66. Common Causes of Leukocoria
cataract
retinal detachment
retinopathy of prematurity
retinal malformation
intraocular infection (endophthalmitis)
retinal vascular abnormality
intraocular tumor (retinoblastoma).

67. Retinoblastoma Most common primary intraocular malignancy of childhood
1/15,000 live births
3% of all childhood cancers
Leukocoria of the left eye.

68. RB
Retinoblastoma is a rare type of eye cancer that arises from the retina and develops in early childhood.
In most children with retinoblastoma, the disease affects only one eye. However, 1 out of 3 children with retinoblastoma develops cancer in both eyes.
The most common first sign is leukocoria. This white reflex is often noted in photographs. Other signs of retinoblastoma include strabismus, eye pain, and eye inflammation
If diagnosed early, retinoblastoma is often curable. However, if not treated promptly, it can spread beyond the eye to other parts of the body and be life-threatening

69. RB
When retinoblastoma is associated with a gene mutation that occurs in all of the body's cells, it is known as germinal retinoblastoma. People with this form of retinoblastoma have an increased risk of developing other life-threatening cancers such as pinealoma, osteosarcoma, and melanoma.

70. Endophytic RB grows from retina into vitreous causing vitreous seeds
Endophytic RB grows from retina into vitreous causing vitreous seeds

71. Exophytic RB grows outward from retina resulting in a mass beneath the retina

72. Presentation of Retinoblastoma
60% leukocoria
20% strabismus
Chronic uveitis

73. Retinoblastoma Quiz

74. Child Abuse
Suspicion should be aroused when apparently traumatic physical exam findings do not match with the history given by the caretaker (like multiple fractures on the face and head after a single short fall). Also, if the history does not match the developmental age of the child (like a 1 month old rolling off a bed or a 8 month old climbing out of a crib or high chair).
Any physician who suspects abuse may have occurred is required by law in every U.S. state to report it to the proper authorities.

75. Ocular Signs of Child Abuse
hyphema
Hyphema: common causes include severe direct trauma like a pellet gun, a car accident, a fist fight. Without a history which makes sense and is consistent, abuse should be suspected.
SCH: common causes include trauma or in some cases increased venous pressure related to severe vomiting or constipation.
Cataract: severe trauma can cause lens opacities.
Characteristic petal-shaped cataract from trauma
Subconjunctival hemorrhage

76. Shaken Baby Syndrome (Abusive Head Trauma)
Shaken baby syndrome (SBS) is a triad of medical symptoms:
subdural hematoma
retinal hemorrhage
brain swelling
These findings lead doctors to infer child abuse caused by intentional shaking.
In a majority of cases there is no visible sign of external trauma.

77. Shaken Baby Syndrome (Abusive Head Trauma)
SBS is often fatal and can cause severe brain damage, resulting in lifelong disability.
Estimated death rates (mortality) among infants with SBS range from 15% to 38%. Up to half of deaths related to child abuse are reportedly due to shaken baby syndrome.
Nonfatal consequences of SBS include varying degrees of visual impairment (including blindness), motor impairment (e.g. cerebral palsy) and cognitive impairments.

78.
Extensive retinal hemorrhages found in multiple layers of the retina and in all 4 quadrants. A large schisis cavity (hemorrhagic cyst) is seen overlying the macula.

79. Retinal Hemorrhages Quiz

80. A child with abnormal eye findings should have a dilated eye examination.
It is safe to dilate a baby’s eyes at any age.

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