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)
2
Q
Primary congential glaucoma
A
They get it and there is nothing else wrong
3
Q
Secondary childhood glaucoma
A
Glaucoma assocaited with systemic disease or something else
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
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
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
7
Q
Normal IOP in infants
A
Lower than adults
-10-12 in newborns, about 14mmHg in 7-8 year olds
8
Q
Corneal enlargement and glaucoma
A
Gradual swelling due to increase in IOP
9
Q
Normal diameter in newborns
A
9.5-10.5mm
>11.5 suggests glaucoma
10
Q
By age 1, normal diameter is
A
10-11.5mm
12.5 suggests an abnormality
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
12
Q
DiffDx for PCG: epiphora and/or red eye
A
Conjunctivitis
NDO
Keratitis or corneal problems
Keratitis or corneal problems
13
Q
DiffDx of PCG: corneal edema and/or opacification
A
Corneal dystrophies Sclerocornea, peters anomaly Keratitis Birth truama Skin disorders Storage disease (mucopolysaccharidoses)
14
Q
DiffDx of PCG: corneal lenargment
A
Megalocornea
15
Q
DiffDx of PCF: ONH cupping
A
Physiological cupping ON hypoplasia Optic atrophy ON coloboma ON anomalies
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)
17
Q
Tonopen with kids
A
Works well
- Icare and Perkins too
- do pressure hen they being bottle fed
- struggles could elevate the pressures
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
19
Q
CCT in PCG
A
Portable pachymeters
-higher in children with PCG due to edema
20
Q
AC in PCG
A
Slit lamp
- could be abnormally deep
- hypoplasia of the peripheral iris strain could be present
21
Q
Gonio in PCG
A
Will show that the iris insertion more anteiror, TM, and scleral spur are indistinct
22
Q
Cupping in PCG
A
Increased due to stretching and bowing at the optic canal
23
Q
CD in PCG
A
- 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
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
25
Secondary childhood glaucoma
- 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
26
Aphakic glaucoma
- 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
27
Mechanism of aphakic glaucoma
Unclear
28
Angles in aphakic glaucoma
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
29
Treatment for aphakic glaucoma
Anterior vitrectomy to relieve any iris blood, OR surgical iridectomy
30
Treatment for childhood glaucoma
- 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
31
If PCG is detected early enough
80% have IOP control with 1 or 2 angle surgeries
32
If surgery is not successful or indicated (like in secondary glaucaomts)
Trabeculectomy with or without antifibrotic therapy (like MMC) can be done
33
Medical therapy in PCG
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
34
Risks for BBlockers
Hypotension, bradycardia, hallucinations
35
Precautions with BBlcolers
Avoids in premature or small infants; caution in babies with astham or cardiac problems, use punctal occlusion
36
A agonists risk
Bradycardia, CNS, depression
37
Precautions with A agonist
Contraindication in kids < 2
38
CAI risks
Cornea edema
| SSJ
39
CAI precautions
- contraindicated in infants with renal problems
- contraindicated in sulfonamide hypersenticitiy
- caution in cornea disease
40
Oral CAI risks
Metabolic acidosis, SSJ. HA. Nausea, dizziness, failure to thrive
41
Precautions of oral CAI
Contraindication in infants with renal problems/insuffieincies
Contraindicated in sulfonamide hypersensitivity
Monitor weight gain
42
PGs risk
Uveitis
43
Precautions in PGs
Avoid in uveitis
44
Parasympathetomimetics risks
Vomiting, diarrhea, dizziness, risk or pupillary block
45
Parasympatehtimimoetics precaution
Avoid in uveitis
| Caution in astham, cardiac diseas
46
Oral medications in PCG
CAI can be effective in kids wto delay surgery, but be catusion of the systemi adverse effects
47
If PCG is present at birth prognosis
Poor
| -a high proportion of these patients will become legally blind
48
If corneal diameter is greater than 14mm at diagnosis of PCG
Poor
49
If onset of PCG is later
Prognosis is better because angle surgery and medications can be effective
50
Vision loss in glaucoma could be due to a combination of any of the following
- 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
Follow up in PCG
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
Pediatric lens disorders
- 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
Lens disorders can be assocaited wtih systemic conditions
```
Chromosomal abnormalities
Systemic diseases
Infection
Truama
Radiation
Metabolic abnormalities
```
54
Lens disorders could be
Isolated
| Associated with a systemiccondtion
55
Other ocular conditions that could lead to lens abnormalities include
- persistent fetal vasculature/persistent hyperplastic primary vitreous
- anterior segment dysgenesis
- aniridia
- optic nerve coloboma
- retinal disorder
56
Characteristics of lens disorders
- congenital (amblyogenic) or acquired
- inserted (AD, AR, X linked) or sporadic
- unilateral or bialteral (systemic conditions)
- partial or complete
- stable or progressive
57
Anteiror pole cataract
- 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
Nuclear cataract
- 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
Lamellar (zonular) cataract
- 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
Posterior lenticonus (Lentiglobus)
- 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
Posterior subcapsular cataract
- less common in kids
- progressive
- from: steroid use, uveitis, radiation, in NF2
62
Persistent fetal vasculature (persistent hyperplastic primary vitreous PHPV)
- 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
Cataract evaluation in kids
- 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
Opacities with large clear areas around them
Allows good visual development
65
If there is nystagmus or strab in childhood lens opacity
The cataract is visually significant.
66
VA for kids with cataracts
Teller acuity cards
| Cardiff cards
67
Slit lamp for lens
To visualize the cataract and any assocaited AC abnormalities
68
If you cannot examine the post pole bc of cataract
B scan
69
Other assessment for kid with cataract
- corneal diameter
- iris
- AC depth
- lens postion
- IOP
- posterior seg
70
When to do labs on kids with cataracts
In the case of bilateral, no hereditary cataracts
71
Genetic eval for kid with cataract
If assocaited with disorder
72
Visual outcome of cataract depends on
- age of onset
- type of cataract
- timing of surgery
- Choice of correction
- amblyopia treatment
73
Early surgery for kids for cataracts
Does not ensure good prognosis
74
Things to consider for cataract surgery for kids
- when to perform the surgery
| - whether to use an IOL
75
The younger the kid for cat surgery
The greater the urgency because of deprivation amblyopia
76
A visually significant unilateral cataract should be removed before ______ of age
6 weeks of age
77
Removal of cataracts in older kids
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
Implant or not in kids
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
Lensectomy without IOL implant
- 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
Lensectomy with IOL implantation
Single piece acrylic foldable IOL are common in pediatric cataract surgery
81
Concerns with lensectomy with IOL implantation
- 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
Post op care for lensectomy
- 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
Amblyopia treatment and lensectomy
- 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
Congenital aphakia
- the absence of the lens at brith
| - an abnormal eye
85
Lens coloboma
- 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
Dislocated lens (subluxation, ectopic)
- 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
Marfan sybdrome
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
Patient characteristics of marfans
- flexible joints
- tall
- scoliosis
- chest deformities
Life expectancy is half the population
80% have ocular problems
89
Lens in marfans
- 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
Homocystinuria
```
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
Homocysteine lens
- inferior subluxation
- ZONULES ARE BROKEN unlike marfan
- treat with coenzymes and dietary supplements
92
Treatment for subluxation
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
Considered the most sensitive for retinal correspondence (also detect suppression)
Bagolini lenses
94
How does bagolini lenses work
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
Uncrossed diplopia on bagolini
Crosses down low
| Eso
96
Crossed diplopia on bagolini lenses
Crosses up high
| Exo
97
Right eye sees ____ in bagolini
The line that looks like this /
98
Four levels of sensory fusion
- 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
