Glaucoma And Cataracts In Children Flashcards

1
Q

Pediatric glaucoma

A

Glaucoma in chidlren can result from

  • congenital abnormalities with aqueous outflow (primary glaucoma-primary congenital glaucoma and juvenile glaucoma
  • other abnormalities (secondary glaucoma)
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2
Q

Primary congential glaucoma

A

They get it and there is nothing else wrong

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3
Q

Secondary childhood glaucoma

A

Glaucoma assocaited with systemic disease or something else

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4
Q

Juvenile open angle glaucoma

A

_AD

  • present after 4 years
  • corean is not enlarged
  • Haab striae is absent
  • AC appears normal
  • management similar to adult POAG, but surgery may also be required
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5
Q

Primary congenital glaucoma (PCG, congenital or infantile glaucoma)

A
  • AR or sporadic
  • higher prevalence in populations where consanguinity is common
  • VA is worse than 20/50 in many cases
  • bilateral in about 2/3 of cases
  • more frequently seen in males and females
  • blindness in 2-15% of cases
  • diagnosis at birth in 25%; within 1st year of life in 80% of cases
  • prognosis is guarded
  • pathophysiology: increases resistance to aqueous outflow due to abnormal development of AC angle tissue
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6
Q

Classic triad presentation in PCG

A

Epiphora
Photophobia
Blepharospasm

Redness: clouding and enlargement of the cornea

IOP can range from 30-40, and greater than 20 under anesthesia

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7
Q

Normal IOP in infants

A

Lower than adults

-10-12 in newborns, about 14mmHg in 7-8 year olds

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8
Q

Corneal enlargement and glaucoma

A

Gradual swelling due to increase in IOP

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9
Q

Normal diameter in newborns

A

9.5-10.5mm

>11.5 suggests glaucoma

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10
Q

By age 1, normal diameter is

A

10-11.5mm

12.5 suggests an abnormality

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11
Q

PCG signs

A
  • elevated IOP can be gradual or sudden
  • corneal edema often the presenting sign in kids under 3m
  • swelling into the stroma with breaks int he descemets membrane (horizontal Haab striae)
  • edema resolves after IOP reduction, but the descemet break persists
  • corneal scarring and opacification could results
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12
Q

DiffDx for PCG: epiphora and/or red eye

A

Conjunctivitis
NDO
Keratitis or corneal problems
Keratitis or corneal problems

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13
Q

DiffDx of PCG: corneal edema and/or opacification

A
Corneal dystrophies 
Sclerocornea, peters anomaly
Keratitis
Birth truama 
Skin disorders
Storage disease (mucopolysaccharidoses)
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14
Q

DiffDx of PCG: corneal lenargment

A

Megalocornea

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15
Q

DiffDx of PCF: ONH cupping

A
Physiological cupping 
ON hypoplasia
Optic atrophy 
ON coloboma 
ON anomalies
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16
Q

Evaluation of children with PCG

A
  • full exam in office and under anesthesia
  • VF will not be reliable in younger kids
  • poorer vision in the affected eye
  • nystagmus could be present
  • myopia and astigmatism possible because of the corneal enlargement
  • check cornea for size, clarity, Haab striae (on retro)
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17
Q

Tonopen with kids

A

Works well

  • Icare and Perkins too
  • do pressure hen they being bottle fed
  • struggles could elevate the pressures
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18
Q

IOP in PCG

A

30-40

Normal IOP in infants and younger is lower than adults

10-12mmHg in newborns about 14mmHg in 7-8yo

Asymmetry IOP in a quiet should raise suspicion of glaucoma

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19
Q

CCT in PCG

A

Portable pachymeters

-higher in children with PCG due to edema

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20
Q

AC in PCG

A

Slit lamp

  • could be abnormally deep
  • hypoplasia of the peripheral iris strain could be present
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21
Q

Gonio in PCG

A

Will show that the iris insertion more anteiror, TM, and scleral spur are indistinct

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22
Q

Cupping in PCG

A

Increased due to stretching and bowing at the optic canal

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23
Q

CD in PCG

A
  1. 30 is normal seen in most infants
    - more than that suspicious
    - asymmetry >0.20 between the 2 eyes is suspicious

Cupping can bre reversed in young kids

  • could use serial axial length to monitor patient-excessive growth could be indicative or problems
  • OCT with newer technology
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24
Q

Untreated PCG

A
  • Leads to blindness
  • Cornea opacity and vascularized
  • Enlargement could continue until about 2-3 years with diameter >15mm
  • buphthalmos (enlarged globe due to increased IOP)
  • scleral thinning
  • myopic fundus changes
  • spontaneous lens dislocation
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25
Q

Secondary childhood glaucoma

A
  • from other congenital or acquired anomaly
  • anteiror segment abnormalities (aniridia, axenfeld-rigger, peters anomaly, sclerocornea, microcornea, iris ectropion, ROP, iris, or ciliary tumors)
  • systemic diseases (SW syndrome, NF, lens related disorders like Marfan syndrome or Ehlers Danlos syndrome
  • from steroid use, uveitis, infection, ocular truama, after cataract surgery
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26
Q

Aphakic glaucoma

A
  • occurs following cataract surgery
  • common in about 15-50%
  • a long term risk that can develop years or just weeks after surgery
  • higher risk in kids that had cataract surgery in infancy
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27
Q

Mechanism of aphakic glaucoma

A

Unclear

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28
Q

Angles in aphakic glaucoma

A

Appear open, but outflow could be compromised due to

  • abnormally development of the angles
  • susceptibility to surgically induced inflammation
  • loss of lens support
  • or other factors
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29
Q

Treatment for aphakic glaucoma

A

Anterior vitrectomy to relieve any iris blood, OR surgical iridectomy

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30
Q

Treatment for childhood glaucoma

A
  • most of the time is surgery
  • primary congenital glaucoma is effectively treated with angle surgery. Goniotomy (incision across the TM) or trabeculotomy (also opening the TM)
  • these surgeries are not so effective in secondary pediatric glaucoma
  • treatment of most secondary glaucoma’s in chidlren is simialr to the treatment of open angle or secondary glaucoma’s in adutls
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31
Q

If PCG is detected early enough

A

80% have IOP control with 1 or 2 angle surgeries

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32
Q

If surgery is not successful or indicated (like in secondary glaucaomts)

A

Trabeculectomy with or without antifibrotic therapy (like MMC) can be done

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33
Q

Medical therapy in PCG

A

Has lower success rates and greater risks in kids

But serves a vital role in pre post and long term care of the kids with other glaucoma’s

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34
Q

Risks for BBlockers

A

Hypotension, bradycardia, hallucinations

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35
Q

Precautions with BBlcolers

A

Avoids in premature or small infants; caution in babies with astham or cardiac problems, use punctal occlusion

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36
Q

A agonists risk

A

Bradycardia, CNS, depression

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37
Q

Precautions with A agonist

A

Contraindication in kids < 2

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38
Q

CAI risks

A

Cornea edema

SSJ

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39
Q

CAI precautions

A
  • contraindicated in infants with renal problems
  • contraindicated in sulfonamide hypersenticitiy
  • caution in cornea disease
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40
Q

Oral CAI risks

A

Metabolic acidosis, SSJ. HA. Nausea, dizziness, failure to thrive

41
Q

Precautions of oral CAI

A

Contraindication in infants with renal problems/insuffieincies
Contraindicated in sulfonamide hypersensitivity
Monitor weight gain

42
Q

PGs risk

A

Uveitis

43
Q

Precautions in PGs

A

Avoid in uveitis

44
Q

Parasympathetomimetics risks

A

Vomiting, diarrhea, dizziness, risk or pupillary block

45
Q

Parasympatehtimimoetics precaution

A

Avoid in uveitis

Caution in astham, cardiac diseas

46
Q

Oral medications in PCG

A

CAI can be effective in kids wto delay surgery, but be catusion of the systemi adverse effects

47
Q

If PCG is present at birth prognosis

A

Poor

-a high proportion of these patients will become legally blind

48
Q

If corneal diameter is greater than 14mm at diagnosis of PCG

A

Poor

49
Q

If onset of PCG is later

A

Prognosis is better because angle surgery and medications can be effective

50
Q

Vision loss in glaucoma could be due to a combination of any of the following

A
  • corneal scarring and opacification
  • ONH damage
  • myopic astigmatism (myopia from the axial length from high IOP and astigmatism from the expansion of the anterior seg)

Need to treat refractive errros and amblyopia

51
Q

Follow up in PCG

A

Very important

  • may need to exam under anesthesia fro accurate assessment
  • if IOP is less than 20mmHg, but there appears to be progressive corneal enlargement, myopia progression, or cupping, further intervention is needed
  • relapse could occur in later years-life-long monitoring and managment needed
52
Q

Pediatric lens disorders

A
  • such as cataracts, or abnormalities in the size, shape, location or development. Significant sources of visual impairment in kids
  • cataracts: responsible for 10% of VI in kids worldwide
53
Q

Lens disorders can be assocaited wtih systemic conditions

A
Chromosomal abnormalities 
Systemic diseases 
Infection
Truama
Radiation
Metabolic abnormalities
54
Q

Lens disorders could be

A

Isolated

Associated with a systemiccondtion

55
Q

Other ocular conditions that could lead to lens abnormalities include

A
  • persistent fetal vasculature/persistent hyperplastic primary vitreous
  • anterior segment dysgenesis
  • aniridia
  • optic nerve coloboma
  • retinal disorder
56
Q

Characteristics of lens disorders

A
  • congenital (amblyogenic) or acquired
  • inserted (AD, AR, X linked) or sporadic
  • unilateral or bialteral (systemic conditions)
  • partial or complete
  • stable or progressive
57
Q

Anteiror pole cataract

A
  • common
  • less than 3mm in diameter
  • white dot in the center of the anteiror lens capsule
  • unilateral or bialteral
  • non progressive
  • visually insignificant
  • unilateral cones can be assocaited with anisometropia and amblyopia
58
Q

Nuclear cataract

A
  • involves the nucleus
  • density and size varies
  • about 3mm in diameter
  • stable, then progresses
  • unilateral and bilateral (more often)
  • inherited or sporadic
  • glaucoma risk
59
Q

Lamellar (zonular) cataract

A
  • round
  • lamellar/zonular in one or more layers of the cortex around the nucleus
  • about 5mm in diameter
  • unilateral, but often bilateral
  • better prognosis than nuclear catacts because onset later
60
Q

Posterior lenticonus (Lentiglobus)

A
  • due to progressive thinning of the central postieror capsule
  • oil droplet appearance with red reflex
  • later, cortical fibers at the outpouching gradually opacify
  • unilateral, normal eye size
  • visual prognosis after surgery is good
61
Q

Posterior subcapsular cataract

A
  • less common in kids
  • progressive
  • from: steroid use, uveitis, radiation, in NF2
62
Q

Persistent fetal vasculature (persistent hyperplastic primary vitreous PHPV)

A
  • common cause of unilateral cataract
  • features include: prominent hyaloid remnants, latte mittendorf (hyaloid artery did not completely regress/nasally), Bergmesiter papilla (glial tissue at the base of the remnant)
  • may also feature a microphthalmic eye
  • elongated ciliary processes
  • thick fibrous persistent hyaloid artery
  • traction on the ONH can also be seen
  • servers cases can have progressive cataract formation
  • AC shallowing
  • secondary glaucoma
63
Q

Cataract evaluation in kids

A
  • red reflex is screened
  • ret
  • any central opacity or cortical distortion of 3mm or more is significant
  • history: development, systemic disorder, family history, visual behavior at home
  • can do slit lamp on family right there
  • visual function: fixation behavior, objection to occlusion
64
Q

Opacities with large clear areas around them

A

Allows good visual development

65
Q

If there is nystagmus or strab in childhood lens opacity

A

The cataract is visually significant.

66
Q

VA for kids with cataracts

A

Teller acuity cards

Cardiff cards

67
Q

Slit lamp for lens

A

To visualize the cataract and any assocaited AC abnormalities

68
Q

If you cannot examine the post pole bc of cataract

A

B scan

69
Q

Other assessment for kid with cataract

A
  • corneal diameter
  • iris
  • AC depth
  • lens postion
  • IOP
  • posterior seg
70
Q

When to do labs on kids with cataracts

A

In the case of bilateral, no hereditary cataracts

71
Q

Genetic eval for kid with cataract

A

If assocaited with disorder

72
Q

Visual outcome of cataract depends on

A
  • age of onset
  • type of cataract
  • timing of surgery
  • Choice of correction
  • amblyopia treatment
73
Q

Early surgery for kids for cataracts

A

Does not ensure good prognosis

74
Q

Things to consider for cataract surgery for kids

A
  • when to perform the surgery

- whether to use an IOL

75
Q

The younger the kid for cat surgery

A

The greater the urgency because of deprivation amblyopia

76
Q

A visually significant unilateral cataract should be removed before ______ of age

A

6 weeks of age

77
Q

Removal of cataracts in older kids

A

Should be based on the level of interference of vision

  • when VA decreases to 20/50 or worse
  • or in teenagers when VA requirements are not met for drivers license
78
Q

Implant or not in kids

A

IOL choice depends on

  • age
  • laterality of the cataract

IOL in infants/younger chidlren is debated because of the higher rate of complications
-needling more surgery compared to CLs

IOL in kids 1-2 years is more common
Better prognosis with early intervention contact lenses and amblyopia treatment

Secondary IOL can then be performed after about 2 years of age

79
Q

Lensectomy without IOL implant

A
  • performed through a small limbal or pars plana incision
  • ultrasonic phacoemulsifcation is not required for the soft cortex and nucleus in children
  • important to remove all cortical material because of chance of reproliferation of the epithelial cells of the lens
  • posterior capsule opacification occurs rapidly in these kids. YAG would be necessary within 18 months of the surgery
  • sufficiency peripheral lens capsules should be left for a second PC IOL implant later
80
Q

Lensectomy with IOL implantation

A

Single piece acrylic foldable IOL are common in pediatric cataract surgery

81
Q

Concerns with lensectomy with IOL implantation

A
  • calculating the proper IOL power since the eye continues to elongate in the first decade of life
  • difficulty getting accurate Ks and axial length
  • using the adult power formula
  • infection and bleeding can occur
  • strab is associated with cataracts
  • risk of glacuaom increases in kids that’s had lens extraction in infancy
82
Q

Post op care for lensectomy

A
  • topical ABX, corticosteroids and cycloplegic are used for a few weeks
  • steroids more aggressive in kids with IOL implant since postop inflammation could be greater compared to cases with no IOL
83
Q

Amblyopia treatment and lensectomy

A
  • ASAP after surgery
  • correction (CLs or glasses) needed within a week of surgery
  • patching of the better seeing eye is important
  • amount of patching is based on the age of the kid and the severity of the amblyopia
84
Q

Congenital aphakia

A
  • the absence of the lens at brith

- an abnormal eye

85
Q

Lens coloboma

A
  • flattening of notching of the lens periphery
  • usually inferonasally
  • assocaited with iris, ONH, or retina coloboma (all due to the abnormal closure of the embryonic fissure
  • no dislocation
  • absent or stretched zonules
  • usually stable
86
Q

Dislocated lens (subluxation, ectopic)

A
  • detached CB
  • free in the posterior chamber or prolapsed into anterior chamber
  • amount of subluxation varies
  • familial or sporadic
  • associated with systemic conditions, or trauam
87
Q

Marfan sybdrome

A

lens subluxation is very common

patients have abnormalities of

  • cardiovascular system (enlargement of the aortic root, dissecting aneurysm, floppy mitral valve)
  • musculoskeletal system
  • ocular systems

AD

88
Q

Patient characteristics of marfans

A
  • flexible joints
  • tall
  • scoliosis
  • chest deformities

Life expectancy is half the population
80% have ocular problems

89
Q

Lens in marfans

A
  • subluxation is superior
  • there is iris transillumination marked at the itis base
  • pupil is small and poor dialtion
  • increased axial length with myopia
  • spontaneous RD in 20-30s
90
Q

Homocystinuria

A
Rare
AR
Homocysteine accumulates in plasma and is secreted in urine 
Can affect
-eyes
-skeletal system
-CNS
-vascular system (abnormalities at large to medium arteries or vein, vascular occlusion at organs, HTN, and cardiomegaliy common)
91
Q

Homocysteine lens

A
  • inferior subluxation
  • ZONULES ARE BROKEN unlike marfan
  • treat with coenzymes and dietary supplements
92
Q

Treatment for subluxation

A

Difficult
-especially in severe subluxation because of optical distortion looking through the lens periphery (severe myopic astigmatism)

If visual function is not improving correction, will need t oconsider lens removal. Followed by CL or glasses

Scleral-sutured IOLs could result in suture breakage

93
Q

Considered the most sensitive for retinal correspondence (also detect suppression)

A

Bagolini lenses

94
Q

How does bagolini lenses work

A

Plano, clear lenses that produce a line image 90 degrees from the orientation of the striations when the patient views a point light source

95
Q

Uncrossed diplopia on bagolini

A

Crosses down low

Eso

96
Q

Crossed diplopia on bagolini lenses

A

Crosses up high

Exo

97
Q

Right eye sees ____ in bagolini

A

The line that looks like this /

98
Q

Four levels of sensory fusion

A
  • zero degree: no fusion, monocular/suppression
  • first degree (superimposition): testing uses two very dissimilar targets. Patients will have diplopia as the targets are difficult to suppress (used with anti suppression therapy)
  • secondary degree (flat fusion): targets uses identical targets with suppression checks. Pateitns do not have diplopia. The image is single but not in stereo. This requires motor fusion
  • their degree (stereopsis): ultimate sensory fusion characterized by stereopsis. The requires both motor and sensory fusion