22 - Pediatric Glaucoma

Question 1

Glaucome primaire versus secondaire?

Answer 1

Pediatric glaucomas are a heterogeneous group of diseases that may result
* from an isolated congenital abnormality of the aqueous outflow pathways (primary glaucoma)
* from abnormalities affecting other regions of the eye (secondary glaucoma)

Question 2

Classification des Childhood Glaucoma

Answer 2

Question 3

Algorithme de classsification des Childhood Glaucomas

Answer 3

Question 4

(Primary Congenital Glaucoma)

Acquisition du primary congenital glaucoma (PCG) : AD, AR ou sporadique?

Answer 4

Although primary congenital glaucoma (PCG) usually occurs sporadically, it may be inherited as an autosomal recessive trait.

Question 5

(Primary Congenital Glaucoma)

Synonymes de Primary congenital glaucoma?

Answer 5

Also called congenital or infantile glaucoma

Question 6

(Primary Congenital Glaucoma)

Incidence du Primary Congenital Glaucoma?

Answer 6

The incidence of PCG varies in different populations, ranging from 1 in 1250 live births to 1 in 68,000.

Question 7

(Primary Congenital Glaucoma)

Concernant le Primary Congenital Glaucoma :
* H versus F ? % ?
* Atteinte unilatérale ou bilatérale?

Answer 7

PCG occurs more frequently in males (65% of cases), and it is bilateral in about two- thirds of patients.

Question 8

(Primary Congenital Glaucoma)

Pronostic visuel des patients avec Primary Congenital Glaucoma?

Answer 8

PCG results in blindness in 2%–15% of cases, and visual acuity remains worse than 20/50 in at least 50% of cases.

Neonatal- onset and late recognized PCG are associated with guarded prognoses.

Question 9

(Primary Congenital Glaucoma)

Physiopathologie?

Answer 9

• The basic pathologic defect is increased resistance to aqueous outflow through the trabecular meshwork due to abnormal development of neural crest– derived tissue of the anterior chamber angle.
• The anomaly occurs late in embryologic development.

Question 10

(Primary Congenital Glaucoma)

Onset?

Answer 10

Primary congenital glaucoma usually presents in the neonatal period or within the first 2 years of life (infantile PCG), but it can present or be recognized after 2 years of age (late onset or late- diagnosed PCG).

Question 11

(Primary Congenital Glaucoma)

Triade clinique classique? Et autres signes cliniques pouvant être présents?

Answer 11

Classic clinical triad of PCG :
* Epiphora
* Photophobia
* Blepharospasm

A red eye may be present.

Other signs include
* Clouding
* Enlargement of the cornea

Question 12

(Primary Congenital Glaucoma)

Concernant l’oedème cornéen associé :
* Gradual or sudden in onset?
* Évolution de l’oedème?
* Conséquences?

Answer 12

• Corneal edema results from elevated IOP and may be gradual or sudden in onset.
• Corneal edema is often the presenting sign in infants younger than 3 months and is responsible for the clinical triad.
• Microcystic edema initially involves the corneal epithelium but later extends into the stroma, often accompanied by one or more curvilinear breaks in Descemet membrane (Haab striae) (Fig 22-3).
• Although the edema may resolve with IOP reduction, the split in Descemet membrane persists.
• Significant corneal scarring and persistent opacification may require penetrating keratoplasty.

Question 13

(Primary Congenital Glaucoma)

Quelle est la cause de la triade clinique classique?

Answer 13

Corneal edema is often the presenting sign in infants younger than 3 months and is responsible for the clinical triad.

Question 14

(Primary Congenital Glaucoma)

Taille de la cornée?

Answer 14

Corneal enlargement occurs with gradual stretching of the cornea as a result of elevated IOP.

Question 15

(Primary Congenital Glaucoma)

Ddx des différents signes associés à un Primary Congenital Glaucoma?
* Épiphora
* Corneal edema or opacification
* Corneal enlargement
* ON cupping

Answer 15

Question 16

Vers quel âge environ peut-on faire des CV pour suivre un glaucome?

Answer 16

Although visual field testing is helpful in following disease progression in older children, results of these tests are rarely reliable in children younger than 6–8 years.

Question 17

(Primary Congenital Glaucoma)

Commentez l’AV?

Answer 17

• Vision is usually poorer in the affected eye in unilateral cases and may be poor in both eyes when glaucoma is bilateral.
• Fixation and following behavior and the presence of nystagmus should be noted.

Question 18

(Primary Congenital Glaucoma)

Erreur réfractive associé? Cause de l’erreur réfractive?

Answer 18

Refraction, when possible, often reveals myopia and astigmatism from eye enlargement and corneal irregularity.

Question 19

Normal horizontal diameter of the cornea? Et Abnormal suggestif de glaucome ou autres anomalies?
* Newborn?
* 1 yo?
* Any child?
* Différence entre les 2 yeux?

Answer 19

• In newborns, the normal horizontal diameter of the cornea is 9.5–10.5 mm; a diameter greater than 11.5 mm suggests glaucoma.
• By age 1 year, the normal corneal diameter is 10.0–11.5 mm; a diameter greater than 12.5 mm suggests abnormality.
• Glaucoma should be suspected in any child with a corneal diameter greater than 13.0 mm.
• A difference as small as 0.5 mm between the 2 eyes may be significant.

Question 20

La pachymétrie est-elle généralement lower or higher chez les enfants avec du glaucome?

Answer 20

Central corneal thickness (CCT)
is typically higher in infants with glaucoma

Question 21

PIO chez l’enfant comparativement à l’adulte? Lower ou Higher?
* Moyenne chez les n-nés?
* Moyenne chez les 7-8 ans?

Answer 21

The normal mean IOP in infants and young children is lower than that in adults:
* Between 10 and 12 mm Hg in newborns
* Approximately 14 mm Hg by age 7–8 years

Question 22

(Primary Congenital Glaucoma)

Range de la PIO chez les patients avec Primary Congenital Glaucoma?

Answer 22

• In PCG, IOP commonly ranges between 30 and 40 mm Hg, and it is usually greater than 20 mm Hg even with the patient under anesthesia.
• Asymmetric IOP readings in a quiet or anesthetized child should raise suspicion of glaucoma.

Question 23

(Primary Congenital Glaucoma)

Anomalies de la CA et de l’iris associées au Primary Congenital Glaucoma?

Answer 23

An abnormally deep anterior chamber and hypoplasia of the peripheral iris stroma are common findings in PCG.

In an eye with PCG, the iris often shows a more anterior insertion compared with the insertion in a normal infant eye.

Question 24

(Primary Congenital Glaucoma)

Cupping du NO suggestif d’un glaucome?

Answer 24

• In most eyes with PCG, the cup– disc ratio exceeds 0.3, whereas in most normal newborn eyes, the cup– disc ratio is less than 0.3.
• Cup– disc asymmetry greater than 0.2 between the 2 eyes is also suggestive of glaucoma.
• In young children, optic nerve cupping may be reversible if IOP is lowered.

Question 25

(Primary Congenital Glaucoma)

Axial lenght dans le glaucome?

Answer 25

Excessive axial length in an eye, especially compared with the fellow eye, may indicate inadequate IOP control.

Question 26

(Primary Congenital Glaucoma)

Natural Hx du Primary Congenital Glaucoma (si non traité)?

Answer 26

• Almost always progresses to blindness.
• Cornea irreversibly opacifies and may vascularize.
• Cornea may continue to enlarge through the first 2–3 years of life, reaching a diameter of up to 17 mm.
• As the entire eye enlarges, pseudoproptosis and buphthalmos may result.
• Scleral thinning
• Myopic fundus changes
• Spontaneous lens dislocation can result.
• Optic nerve damage progresses, leading to complete blindness.

Rare cases of spontaneous resolution have been reported.

Question 27

(Juvenile Open- Angle Glaucoma)

Acquisition : AD, AR ou sporadique?

Answer 27

Juvenile open- angle glaucoma (JOAG) is an autosomal dominant condition

Question 28

(Juvenile Open- Angle Glaucoma)

Onset?

Answer 28

Condition that presents after 4 years of age

Question 29

(Juvenile Open- Angle Glaucoma)

Commentez la cornée et l’angle.

Answer 29

The cornea is not enlarged, Haab striae are not present, and the anterior chamber angle usually appears normal

Question 30

(Juvenile Open- Angle Glaucoma)

Management?

Answer 30

Management of JOAG is similar to that of adult primary open- angle glaucoma, but the condition frequently requires surgery.

Question 31

(Secondary Childhood Glaucoma)

Définir le Secondary Childhood Glaucoma?

Answer 31

Glaucoma caused by other ocular anomalies (congenital or acquired) or associated with systemic disease or syndromes is considered secondary.

Question 32

(Secondary Childhood Glaucoma)

Ddx de nonacquired ocular anomalies and systemic diseases and syndromes associated with secondary glaucoma

Answer 32

Question 33

(Secondary Glaucoma Associated With an Acquired Condition)

Médicament (avec des ppts aticonvulsivantes et antidépressives) pouvant causer un angle-closure glaucoma?

Answer 33

The anticonvulsant and antidepressant medication topiramate can cause acute, usually bilateral, angle- closure glaucoma secondary to ciliary effusion.

The ciliochoroidal effusion causes relaxation of zonules, resulting in extreme anterior displacement of the lens– iris complex, which leads to secondary angle- closure glaucoma and high myopia.

Peripheral iridectomy is not effective as treatment, but timely cessation of the medication is.

Question 34

(Glaucoma Following Cataract Surgery)

V ou F : Aphakic glaucoma is a common form of secondary glaucoma in childhood.

Answer 34

Vrai. Aphakic glaucoma is a common form of secondary glaucoma in childhood.

Question 35

(Glaucoma Following Cataract Surgery)

% avec open-angle aphakic glaucoma after congenital cataract surgery?

Answer 35

The reported incidence of open- angle aphakic glaucoma after congenital cataract surgery varies from 15% to 50% or higher.

Question 36

(Glaucoma Following Cataract Surgery)

Onset?

Answer 36

Aphakic glaucoma often develops years after cataract surgery, but it can occur within weeks to months of surgery and remains a lifelong risk.

Question 37

(Glaucoma Following Cataract Surgery)

Children avec highest risk for glaucoma development following cataract surgery?

Answer 37

The children at highest risk for glaucoma development following cataract surgery are those who have surgery during infancy, and the risk appears to be even higher in patients with microcornea or persistent fetal vasculature.

Question 38

(Glaucoma Following Cataract Surgery)

L’angle est-il fermé ou ouvert à la gonioscopie?

Answer 38

• The mechanism of aphakic glaucoma is unclear.
• The anterior chamber angle usually appears open on gonioscopy; the outflow channels are compromised by some combination of abnormal development of the anterior chamber angle and perhaps susceptibility of the infant eye to surgically induced inflammation, loss of lens support, retained lens epithelial cells, or vitreous factors.

Question 39

Primary Tx for most types of childhood glaucoma : médical ou chirurgical?

Answer 39

The primary treatment for most types of childhood glaucoma is surgery.

Question 40

V ou F : only 10% of infants with PCG presenting from 3 months to 1 year of age, IOP is controlled with 1 or 2 angle surgeries.

Answer 40

Faux. In approximately 80% of infants with PCG presenting from 3 months to 1 year of age, IOP is controlled with 1 or 2 angle surgeries.

Question 41

Autres options chirurgicales en 2e ligne?

Answer 41

When angle surgery is not successful in a child or is not indicated (as is the case in many forms of secondary glaucoma) and medical therapy is inadequate, additional surgical options include
* Trabeculectomy with or without antifibrotic therapy (eg, mitomycin C [MMC])
* Tube shunt implantation
* Cyclodestructive procedures

Question 42

Long-term risk d’une trabéculectomie chez l’enfant? Comment réduire ce long-term risk?

Answer 42

Although the success rate of trabeculectomy improves with the use of antifibrotics such as MMC, the long- term risk of bleb leaks and endophthalmitis also increases.

Long- term risk is reduced by using a fornix- based rather than a limbus- based conjunctival flap.

Question 43

Patients les plus à risque de Tx failure avec la trabéculectomie?

Answer 43

Patients younger than 1 year and those who are aphakic are more prone to treatment failure.

Question 44

Which is less prone to leaking and infection between trabeculectomy and tube shunts?

Answer 44

Although most children with implanted tube shunts must remain on adjunct topical medical therapy to control IOP after surgery, their blebs are thicker and are less prone to leaking and infection than those of patients who have under gone MMC- augmented trabeculectomy.

Question 45

Success rate of tube shunt implantation surgery with Molteno, Baerveldt, and Ahmed devices?

Answer 45

The reported success rate of tube shunt implantation surgery with Molteno, Baerveldt, and Ahmed devices varies between 54% and 85%.

Question 46

Potential complications of tube shunt implantation?

Answer 46

Potential complications include
* shunt failure
* tube erosion or migration
* tube– cornea touch
* cataract
* restrictive strabismus
* endophthalmitis.

Question 47

Indication de laser cyclodestruction and cyclocryotherapy?

Answer 47

Laser cyclodestruction and cyclocryotherapy are generally reserved for resistant cases or those not amenable to other surgical procedures.

Question 48

Mécanisme d’action du laser cyclodestruction and cyclocryotherapy?

Answer 48

These techniques decrease ciliary body production of aqueous humor, which results in lower IOP.

Question 49

Lequel a un plus grand risque de complications : laser cyclodestruction and cyclocryotherapy?

Answer 49

Cyclocryotherapy (freezing the ciliary processes through the sclera) may be successful, but the complication rate is high.
* Repeated applications are often necessary, and the risk of phthisis and blindness is significant (approximately 10%).

Transscleral cyclophotocoagulation with the Nd:YAG or diode laser has a lower risk of complications.
* The short- term success rate is approximately 50%.
* Patients usually require more than one treatment.

Endoscopic cyclophotocoagulation (ECP) has been used in children with glaucomas that are difficult to treat.
* In ECP, a microendoscope applies laser energy to the ciliary processes under direct visualization.
* Success rates of up to 50% have been reported.
* Although this is an intraocular procedure, the complication rate may be lower than that of external cyclodestructive procedures.

Question 50

V ou F : Generally, medical therapy for adult glaucomas has lower success rates and greater risks than medical therapy for childhood glaucomas.

Answer 50

Faux. Generally, medical therapy for childhood glaucoma has lower success rates and greater risks than medical therapy for adult glaucomas.

However, it serves several important purposes in preoperative, postoperative, and long- term management, particularly in childhood glaucoma other than PCG.

Question 51

Solution pouvant limitée absorption systémique des gouttes ophtalmiques?

Answer 51

Although punctal occlusion may be used to reduce systemic absorption of topical medications, it may be impractical in many young children.

Question 52

ES des gouttes de glaucome chez la clientèle pédiatrique?

Answer 52

Medical therapy for pediatric glaucoma also carries unique risks because of the greater dose per body weight and the limited number of controlled clinical trials in children.

Question 53

Concernant les BB :
* Efficacité pour réduction de la PIO (en %)?
* ES?
* Nom du BB cardiosélectif B1?

Answer 53

• Therapy with topical β- adrenergic antagonists, or β- blockers, may reduce IOP by 20%–30%.
• The major risks of this therapy are respiratory distress (caused by apnea or bronchospasm) and bradycardia, both of which occur mostly in small infants and in children with a history of bronchospasm.
• Betaxolol, a cardioselective β1- adrenergic antagonist, may be safer than a nonselective β- blocker for use in patients with asthma, but its pressure- lowering effect is less than that of nonselective agents.

Question 54

Concernant les Topical carbonic anhydrase inhibitors (CAIs) :
* Efficacité pour réduction de la PIO (en %)?
* ES?
* Sécuritaire chez l’enfant?

Answer 54

• Topical carbonic anhydrase inhibitors (CAIs) are effective in children, but they produce a smaller reduction in IOP (<15%) than do β- blockers.
• Corneal edema is a risk of topical CAIs; thus, they should be used with caution in children with coexisting corneal disease.

Question 55

Commentez l’utilisation des analogues des prostaglandines chez l’enfant?

Answer 55

• Prostaglandin analogues are effective in many pediatric patients.
• Their low systemic risk and once- daily dosing are advantageous.

Question 56

Concernant les α2- adrenergic agonists :
* Noms de deux Rx de la classe?
* ES?
* À quel âge il y a contre-indication relative?

Answer 56

• Apraclonidine may be useful for short- term IOP reduction, but there is a high incidence of tachyphylaxis and allergy with use of this drug in young children.
• Brimonidine effectively reduces IOP in some cases of pediatric glaucoma.
• Both medications can produce somnolence and respiratory depression in infants and young children.
• Infants and young children are particularly susceptible to brimonidine’s adverse effects.
• Therefore, brimonidine should be used with caution in children younger than 6 years, and it is relatively contraindicated in children younger than 2 years because of the risk of respiratory depression.

Question 57

Pronostic?

Answer 57

• The prognosis for control of IOP and preservation of vision is poor for patients with PCG who present at birth; at least half of these patients become legally blind.
• If the horizontal diameter of the cornea is greater than 14 mm at diagnosis, the visual prognosis is similarly poor.
• Up to 90% of cases in the “favorable prognostic group” (onset at 3–12 months of age) can be controlled with angle surgery and medications.
• The remaining 10%, and many of the remaining cases of primary and secondary glaucomas, often present a lifelong challenge.

Question 58

V ou F : Vision loss in childhood glaucoma is multifactorial.

Answer 58

Vrai.

Vision loss in childhood glaucoma is multifactorial.
It may result from
* Corneal scarring and opacification
* Optic nerve damage
* Myopic astigmatism
* Associated anisometropic and strabismic amblyopia

Question 59

Causes de vision loss in childhood glaucoma?

Answer 59

Vision loss in childhood glaucoma is multifactorial.
It may result from
* Corneal scarring and opacification
* Optic nerve damage
* Myopic astigmatism
* Associated anisometropic and strabismic amblyopia

Question 60

Est-ce qu’une PIO < 20 mmHg sous anesthésie générale est considérée comme un bon contrôle du glaucome?

Answer 60

• If the IOP is less than 20 mm Hg under anesthesia but clinical evidence shows persistent corneal edema or enlargement, progressive optic nerve cupping, or myopic progression, further intervention should be pursued despite the IOP reading.
• In contrast, a young child who has an IOP of about 20 mm Hg but shows evidence of clinical improvement may be observed carefully.

Question 61

V ou F : Relapse can occur years later, with elevated IOP and subsequent vision loss.

Answer 61

Vrai.
* Relapse can occur years later, with elevated IOP and subsequent vision loss.
* Parents, and patients themselves as they become older, should be educated about the need for lifelong monitoring and management.