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Glaucoma > Childhood Glaucoma > Flashcards

Childhood Glaucoma Flashcards

1
Q

What are childhood glaucomas?

A

developmental glaucomas with congenital anomalies of the filtration angle

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2
Q

What are the common PE findings in childhood glaucoma?

A

Enlarged/Cloudy Corneas
Haab striae
Increased IOP

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3
Q

What is newborn glaucoma? Infantile glaucoma? Late diagnosed Glaucoma?

A

Birth- 1 month old
2 months - 2 y/o
> 2 y/o

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4
Q

How is most childhood glaucoma inherited?

A

Majority of cases are sporadic; however, 10-40% have AR inheritence

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5
Q

What are the two major genes involved in childhood glaucoma?

A

CYP1B1= member of cytochrome P450 system, located in the GLC3A locud
LTBP2: transforming growth factor Beta binding protein, located in GLC3C locus

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6
Q

How are most cases of juvenile glaucoma inherited?

A

AD inheritence predominates; linked to mutations in TIGR/MYOC gene on GLC1A locus (codes for myocillin)

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7
Q

What genetic mutations are associated with Axenfeld-Rieger syndrome?

A

PITX2 and FOXC1

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8
Q

What genetic mutations are associated with Peters Anomaly?

A

PITX2, FOXC1, CYP1B1, PAX6

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9
Q

What are the classic presenting symtpoms in primary congenital glaucoma (PCG)?

A

epiphora + photophobia +blepharospasm

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10
Q

What classic PE finding occurs with PCG?

A

until age 3, increased IOP leads to corneal stretching, buphthalmos and Haab striae

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11
Q

Is there a gender predominance for PCG? What percentage of cases occur bilaterally?

A

occurs more commonly in males (65%) and bilaterally in 70 % of patients

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12
Q

What age ranges for diagnosis of PCG carry the best prognosis? The Best?

A

Worst prognosis: diagnosed after 1 year

Best prognosis: diagnosed b/w 3 mos - 1 yr

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13
Q

Of the PCG cases, what percentage are diagosed as newborn glaucoma?

A

25% of cases diagosed as newborn glaucoma, has a worse prognosis than infantile glaucoma

50% of cases will progress to blindness

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14
Q

What is the treatment for PCG?

A

Surgical Intervention

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15
Q

How old are patients that are diagnosed with juvenile glaucoma? What is the inheritance pattern?

A

aged 4-35 y/o

AD inheritence

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16
Q

How is juvenile glaucoma managed?

A

Most patients require trabeculectomy or glaucoma drainage devices

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17
Q

What is Axenfeld Rieger Syndrome?

A

a spectrum of d/o characterized by bilateral anomalous development of neural crest cell derived anterior segment structures

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18
Q

What is the inheritence pattern for Axenfeld Rieger Syndrome? What percentage of patients develop glaucoma?

A

Mainly AD inheritence; 50% of patients will develop glaucoma in middle/late childhood

19
Q

What is the Axenfeld Anomaly?

A

Posterior embryotoxon (opaque ring around the cornea; prominent and anteriorly displaced schwalbes line) with multiple adherent iris strands

20
Q

What is the Rieger Anomaly?

A

Axenfeld anomaly + iris hypoplasia + corectopia

21
Q

What is Rieger Syndrome?

A

Rieger Anomaly + development defects of dental/facial bones, redundant periumbilical skin, pituitary anomalies, or hypospadias

22
Q

What is Peters Anomaly?

A

typically bilateral, a developmental condition p/w annular corneal opacity (leukoma) in the central visual axis corresponding to a defect in K endothelium and DM +/- adhesions b/w iris strands

23
Q

What is the inheritence pattern for Peters Anomaly?

A

usually sporadic; however, AD and AR pattens exist

24
Q

What percentage of patients with Peters Anomaly go on to develop glaucoma?

A

50% of patients with angle abnormalities will develop glaucoma

25
Q

What is aniridia?

A

a bilateral congenital disorder characterized by iris hypoplasia, often associated with other abnormalities

26
Q

What percentange of patients with aniridia develop glaucoma? Why does it develop?

A

50-75% of patients will develop glaucoma

Develops due to anterior rotation of iris stump, covering the TM, leading to synechial angle closure

27
Q

What is the inheritence pattern for aniridia?

A

Most cases are familial, transmitted with AD inheritance
20% of sporadic cases are associated with chromosomal deltion that includes wilms tumor 1 gene (WTG1)
Rarely associated with Gillespie Syndrome)

28
Q

What is Sturge-Weber Syndrome?

A

phakomatoses with ipsilateral facial cutaneous hemangioma and ipsilateral cavernous hemangioma of the choroid + ipsilateral leptomeningial angiomas
typically unilateral

29
Q

What percentage of children with Sturge-Weber develop glaucoma?

A

30-70 % of children will develop glaucoma

more commonly develops in children with nevus flammeus that involves the eyelids

30
Q

What is the genetic mutation associated with neurofibromatosus? What is the predominant inheritence pattern?

A

associated with 11q17 mutation

AD inheritence in 50% of cases

31
Q

What is a classic finding in NF, that when seen in infants should prompt NF workup?

A

Ectropion Uveae

32
Q

What other PE findings occur in NF?

A 
Ectropion Uveae
Lisch Nodules (pigmented iris hamartomas)
ON gliomas
Eyelid neurofibromas
glaucoma
33
Q

What are causes of secondary glaucomas in children?

A

CE during 1st year of life
Post-Op complications
Small K diameter

34
Q

What is normal corneal diameter in children? What is the minimum cutoff to diagnose an enlarged cornea in a child?

A

nL newborn cornea is 10 mm

eyes with congenital glaucoma have k diameter > 12 mm during 1st year of life

35
Q

What are anesthetic agents that dont change IOP in children after administration?

A

Chloral hydrate

ketamine

36
Q

What is the characteristic appearance of the AC angle in a child with PCG?

A

Angle is deep with high anterior iris insertion
angle recess is absent
iris root appears as a “scalloped line of glistening tissue” (known as a barkan membrane)

37
Q

What is the definitive management of PCG?

A

surgical intervention (goniotomy and trabeculotomy are preferred methods)

goniotomy if cornea is clear; trab is k is cloudy

38
Q

Describe goniotomy

A

the angle is visualized, a needle knife is passed across the AC, and a superficial cut is made in the uveal TM

Incision is made above iris root, passing from untreated to treated areas

39
Q

Describe a trabeculotomy

A

schlemms canal is cannulated via an ab externo approach, and the TM is opened by breaking schlemms canal into the AC

40
Q

When should trabeculectomy or drainage device implantation occur?

A

Should be considered when >2 unsuccessful angle surgeries have been performed, when Rx therapy is inadequate, or in children with other forms of glaucoma

41
Q

What is cyclodestruction? When is it used?

A

it decreases IOP by destroying regions of the CB, causing decreased aqueous production
used in refractory cases

42
Q

What medical therapy is considered first line in children? 2nd line?

A

Topical Beta blockers are first line, CAIs are second line

43
Q

What topical glaucoma medication is contraindicated in children? Why?

A

Alpha adrenergics are contraindicated in children < 3 due to side effect profile (apnea, hypotension, bradycardia, hypotonia, hypothermia, and somnolence)

44
Q

What cases of childhood glaucoma require lifelong follow up?

A

All cases of childhood glaucoma require lifelong follow up

Glaucoma (8 decks)

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