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Pédiatrie > 23 - Childhood Cataracts and Other Pediatric Lens Disorders > Flashcards

23 - Childhood Cataracts and Other Pediatric Lens Disorders Flashcards

1
Q

Incidence worldwide of lens abnormalities

A

The incidence of lens abnormalities ranges from 1:4000 to 1:10,000 live births per year worldwide.

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2
Q

% de vision loss in children worldwide causée par cataractes?

A

Cataracts are responsible for nearly 10% of all vision loss in children worldwide.

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3
Q

Concernant les cataractes pédiatriques :
* Isolée ou associée à autres conditions?
* Congenital ou acquise?
* Inherited ou sporadique?
* Unilatérale ou bilatérale?
* Partielle ou complète?
* Progressive ou stable?

A

Pediatric cataracts can be
* Isolated or associated with a systemic condition or other ocular anomalies
* Congenital (infantile) or acquired
* Inherited or sporadic
* Unilateral or bilateral
* Partial or complete
* Stable or progressive

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4
Q

Cataracts in children can be isolated, or they can be associated with a number of conditions. Donnez des exemples d’association.

A

Cataracts in children can be isolated, or they can be associated with a number of conditions, including
* Chromosomal abnormalities
* Systemic syndromes and diseases
* Infection
* Trauma
* Radiation exposure

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5
Q

Majorité des cataractes associées avec une maladie systémique sont-elles unilatérales ou bilatérales?

A

In almost all cases of cataract associated with systemic disease, the cataracts are bilateral ; many bilateral cataracts, however, are not associated with systemic disease.

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6
Q

V ou F : many bilateral cataracts are associated with systemic disease

A

Faux.
In almost all cases of cataract associated with systemic disease, the cataracts are bilateral ; many bilateral cataracts, however, are not associated with systemic disease.

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7
Q

Les cataractes bilatérales sont-elles symétriques ou asymétriques?

A

Les deux. Significant asymmetry can be present in bilateral cases.

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8
Q

Cataracts can be associated with other ocular anomalies. Donnez des exemples.

A

Cataracts can also be associated with other ocular anomalies, including
* Persistent fetal vasculature
* Anterior segment dysgenesis
* Aniridia
* Retinal disorders (eg, coloboma, detachment)

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9
Q

Est-ce que cataractes congénitales et infantiles sont synonymes?
Onset des cataractes congénitales et/ou infantile?

A
  • Pediatric cataracts can be congenital or acquired.
  • Congenital cataracts are present at birth, although they may not be identified until later.
  • Infantile cataracts are present during the first year of life.
  • The terms congenital and infantile cataract are typically used synonymously.
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10
Q

V ou F : In general, the earlier the onset, the more amblyogenic the cataract will be, particularly in unilateral cases.

A

Vrai.
In general, the earlier the onset, the more amblyogenic the cataract will be, particularly in unilateral cases.

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11
Q

V ou F : Lens opacities that are visually significant before 10 months of age are the most likely to be detrimental to vision.

A

Faux. Lens opacities that are visually significant before 2–3 months of age are the most likely to be detrimental to vision.

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12
Q

Mode de transmission héréditaire la plus fréquente pour les cataractes? Les cataractes sont-elles davantage unilatérales ou bilatérales lorsqu’héritées?

A
  • Most hereditary cataracts show an autosomal dominant mode of transmission, and they are almost always bilateral.
  • X- linked and autosomal recessive inheritance may occur; the latter is more common in consanguineous populations.
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13
Q

Étiologies des cataractes pédiatriques

A
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14
Q

Étiologies des cataractes pédiatriques UNILATÉRALES

A
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15
Q

Étiologies des cataractes pédiatriques BILATÉRALES

A
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16
Q

V ou F :
* Cataracts can involve the entire lens (total, or complete, cataract) or only part of the lens structure.
* The location in the lens and morphology of the cataract provide information about etiology, onset, and prognosis.

A

Vrai.
* Cataracts can involve the entire lens (total, or complete, cataract) or only part of the lens structure.
* The location in the lens and morphology of the cataract provide information about etiology, onset, and prognosis.

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17
Q

Étiologie(s) associée(s) à la caractéristique morphologique suivante : Spokelike?
Autres findings possibles?

A

Étiologies :
* Fabry disease
* Mannosidosis

Other Possible Findings :
* Corneal whorls
* Hepatosplenomegaly

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18
Q

Étiologie(s) associée(s) à la caractéristique morphologique suivante : Vacuolar ?
Autres findings possibles?

A

Étiologie :
* Diabetes Mellitus
* Prématurée

Other Possible Findings :
* Elevated blood glucose level

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19
Q

Étiologie(s) associée(s) à la caractéristique morphologique suivante : Multicolored flecks ?
Autres findings possibles?

A

Étiologies :
* Hypoparathyroidism
* Myotonic dystrophy

Other Possible Findings :
* Low serum calcium level
* Characteristic facial features, tonic « grip »

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20
Q

Étiologie(s) associée(s) à la caractéristique morphologique suivante : Green « sunflower » ?
Autres findings possibles?

A

Étiologie :
* Wilson disease

Other Possible Findings :
* Kayser-Fleischer ring

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21
Q

(Anterior polar cataract)

Apparence d’une Anterior polar cataract (taille et localisation)

A

Anterior polar cataracts (APCs) are common and usually
* Less than 3 mm in diameter
* Appearing as small white dots in the center of the anterior lens capsule

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22
Q

(Anterior polar cataract)

Concernant les Anterior polar cataracts (APCs) :
* Unilatérales ou Bilatérales?
* Congénitales ou Acquises?
* Héritées ou Sporadiques?
* Progressive ou Stable?
* Atteinte visuelle?

A
  • They are congenital, usually sporadic opacities.
  • APCs can be unilateral or bilateral.
  • They are usually nonprogressive and visually insignificant, perhaps more appropriately termed anterior lens opacities.
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23
Q

(Anterior polar cataract)

Autre atteinte visuelle associée aux Anterior polar cataracts (APCs)?

A

Unilateral APCs are associated with anisometropia, which may cause amblyopia; thus, careful refraction and follow-up are indicated.

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24
Q

(Anterior polar cataract)

Concernant l’Anterior pyramidal cataract :
* Apparence?
* Projection?
* Forme d’APC?
* Progression ou stable?
* Amblyopie secondaire?

A
  • Anterior pyramidal cataracts, as the name suggests, have a pyramidal shape and project into the anterior chamber.
  • This cataract is a larger, more severe form of APC.
  • It is often associated with cortical changes that can be progressive and amblyogenic, depending on the size of the opacity.
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25
Q

(Infantile nuclear cataract)

Concernant l’Infantile nuclear cataract :
* Localisation de l’opacité?
* Dimensions de l’opcaité?
* Densité à la naissance?
* Héritée ou Sporadique?
* Unilatérale ou Bilatérale?
* Stabilité ou Progression de l’opacité?
* Autre caractéristique des yeux souvent associée avec les cataractes nucléaires infantiles?

A
  • Nuclear cataracts are opacities that involve the center, or nucleus, of the lens.
  • They are usually approximately 3 mm in diameter, but the irregularity of the lens fibers can extend more peripherally.
  • Density and size can vary.
  • Infantile nuclear cataracts may not be significantly dense at birth.
  • They can be inherited or sporadic and are more commonly bilateral.
  • These opacities are usually stable, but they can progress.
  • Eyes with nuclear cataracts may be smaller than normal.
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26
Q

(Infantile nuclear cataract)

Concernant l’Infantile nuclear cataract :
* Localisation de l’opacité?
* Dimensions de l’opcaité?
* Densité à la naissance?

A
  • Nuclear cataracts are opacities that involve the center, or nucleus, of the lens.
  • They are usually approximately 3 mm in diameter, but the irregularity of the lens fibers can extend more peripherally.
  • Density and size can vary.
  • Infantile nuclear cataracts may not be significantly dense at birth.
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27
Q

(Infantile nuclear cataract)

Concernant l’Infantile nuclear cataract :
* Héritée ou Sporadique?
* Unilatérale ou Bilatérale?
* Stabilité ou Progression de l’opacité?

A
  • They can be inherited or sporadic and are more commonly bilateral.
  • These opacities are usually stable, but they can progress.
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28
Q

(Infantile nuclear cataract)

Autre caractéristique des yeux souvent associée avec les cataractes nucléaires infantiles?

A

Eyes with nuclear cataracts may be smaller than normal.

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29
Q

(Lamellar cataract)

Synonyme de Lamellar cataract (aka autre nom)

A

Lamellar cataracts or zonular cataracts

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30
Q

(Lamellar cataract)

Structures du cristallin atteintes dans la Lamellar cataract?

A

Lamellar (zonular) cataracts affect one or more of the layers of the developing lens cortex surrounding the nucleus.

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31
Q

(Lamellar cataract)

Concernant la Lamellar cataract :
* Structures du cristallin atteintes?
* Caractéristiques de la cataracte à l’E/O?
* Dimensions de l’opacité?

A
  • Lamellar (zonular) cataracts affect one or more of the layers of the developing lens cortex surrounding the nucleus.
  • Affected lenses have a clear center, a discrete lamellar opacity, and a clear peripheral cortex.
  • Larger than nuclear cataracts, these opacities are typically 5 mm or more in diameter.
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32
Q

(Lamellar cataract)

Concernant la Lamellar cataract :
* Unilatérale ou Bilatérale

A

They can be unilateral but are more often bilateral.

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33
Q

(Lamellar cataract)

Concernant la Lamellar cataract :
* Dimensions de la cornée chez pt avec Lamellar cataract?

A

The size and corneal diameter of affected eyes are normal.

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34
Q

(Lamellar cataract)

Concernant la Lamellar cataract :
* Densité?
* Pronostic visuel?

A

Lamellar cataracts are often less dense than other forms of infantile cataracts, and therefore the visual prognosis is usually better.

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35
Q

(Lamellar cataract)

Concernant la Lamellar cataract :
* Structures du cristallin atteintes?
* Caractéristiques de la cataracte à l’E/O?
* Dimensions de l’opacité?
* Unilatérale ou Bilatérale
* Dimensions de la cornée chez pt avec Lamellar cataract?
* Densité?
* Pronostic visuel?

A
  • Lamellar (zonular) cataracts affect one or more of the layers of the developing lens cortex surrounding the nucleus.
  • Affected lenses have a clear center, a discrete lamellar opacity, and a clear peripheral cortex.
  • Larger than nuclear cataracts, these opacities are typically 5 mm or more in diameter.
  • They can be unilateral but are more often bilateral.
  • The size and corneal diameter of affected eyes are normal.
  • Lamellar cataracts are often less dense than other forms of infantile cataracts, and therefore the visual prognosis is usually better.
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36
Q

(Posterior lenticonus)

Définir le Posterior lenticonus.
* Cause?
* Lenticonus versus Lentiglobus?

A
  • Posterior lenticonus is a cone- shaped deformation of the posterior lens surface caused by progressive thinning of the central capsule
  • When the deformation is spherical, it is referred to as lentiglobus
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37
Q

(Posterior lenticonus)

Évolution du Posterior lenticonus :
* Apparence initiale au RR?
* Apparence lors progression?
* Apparence lorsque Small tear develops

A
  • This thinning initially causes the lens to have an “oil droplet” appearance on red reflex examination.
  • As the outpouching of the lens progresses, the surrounding cortical fibers gradually opacify (Fig 23-4B).
  • This process can take many years, but if the capsule develops a small tear, rapid, total opacification of the lens can occur (Fig 23-4C).
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38
Q

(Posterior lenticonus)

Concernant le Posterior lenticonus :
* Opacité Unilatéral ou Bilatéral?
* Dimensions d’un oeil affecté?
* Congénitale ou Acquise?
* Pronostic visuel?

A
  • The opacities are almost always unilateral, and the affected eye is normal in size.
  • Although the weakness in the posterior capsule may be congenital, the cataract does not usually form until later and thus behaves like an acquired cataract.
  • The visual prognosis after cataract surgery is usually favorable.
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39
Q

(Posterior subcapsular cataract)

Fréquence des Posterior subcapsular cataracts chez enfant versus chez adulte?

A

Posterior subcapsular cataracts (PSCs) are less common in children than in adults.

DONC : adultes&raquo_space; enfants

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40
Q

(Posterior subcapsular cataract)

Concernant les Posterior subcapsular cataracts (PSCs) :
* Héritées ou Acquises?
* Unilatérales ou Bilatérales?
* Stable ou Progression?

A
  • They are usually acquired and are often bilateral.
  • PSCs tend to progress.
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41
Q

(Posterior subcapsular cataract)

Causes de Posterior subcapsular cataracts (PSCs)?

A

Causes of PSC include
* corticosteroid use
* uveitis
* retinal abnormalities
* radiation exposure
* trauma

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42
Q

Phacomatose associée aux Posterior subcapsular cataracts (PSCs)?

A

PSCs can be seen in association with neurofibromatosis type 2 and may be the first observed manifestation of this disorder.

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43
Q

(Sectoral cataract)

Causes d’une Sectoral cataract?

A

Wedge- shaped cortical cataracts are occasionally seen in children.

These opacities may be idiopathic, or they may be associated with
* Occult posterior segment tumor
* Previous blunt trauma
* Vitreoretinopathies
* Retinal coloboma with fibrous bands attached to the posterior lens capsule

Careful posterior segment examination is indicated to rule out these associated pathologies.

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44
Q

(Peripheral vacuolar cataract)

Concernant la Peripheral vacuolar cataract :
* Type de patientèle?
* Cataracte la plus souvent rencontrée en concomitance de quelle autre maladie?
* Atteinte visuelle?
* Pronostic?

A
  • These asymptomatic peripheral lens vacuoles are sometimes seen in premature infants.
  • The cataracts are most often encountered during examination for retinopathy of prematurity.
  • They are rarely visually significant and usually resolve spontaneously.
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45
Q

Étiologie la plus fréquente d’une cataracte pédiatrique unilatérale?

A

Persistent fetal vasculature (PFV; previously called persistent hyperplastic primary vitreous) is the most common cause of a unilateral cataract.

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46
Q

(Persistent fetal vasculature)

Concernant la cataracte dans un contexte de Persistent fetal vasculature :
* Unilatérale ou Bilatérale?
* Isolée ou associée avec anomalies?
* Sporadique ou Transmission autosomale ?

A
  • Persistent fetal vasculature (PFV) is the most common cause of a unilateral cataract.
  • PFV is typically an isolated, sporadic ocular malformation, but bilateral cases may be associated with systemic or neurologic abnormalities.
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47
Q

(Persistent fetal vasculature)

Dimension d’un oeil dans contexte de Persistent fetal vasculature?

A

Usually, affected eyes are smaller than normal.

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48
Q

(Persistent fetal vasculature)

Features of mild PFV (x3)?

A

Features of mild PFV are
* Prominent hyaloid vessel remnants
* A large Mittendorf dot
* Bergmeister papilla

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49
Q

(Persistent fetal vasculature)

Caractéristiques d’un PFV plus sévère?

A

At the other end of the spectrum are
* Microphthalmic eyes
* Dense retrolental plaques
* A thick, fibrous persistent hyaloid artery
* Elongated ciliary processes (classic for PFV), which may be visible through the dilated pupil
* Prominent radial iris vessels

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50
Q

(Persistent fetal vasculature)

V ou F : Traction on the optic disc may cause distortion of the posterior retina

A

Vrai. Traction on the optic disc may cause distortion of the posterior retina.

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51
Q

(Persistent fetal vasculature)

Caractéristques de l’opacité du cristallin dans le PFV?

A
  • Varying degrees of lens opacification occur.
  • The opacity usually consists of a retrolental plaque that is densest centrally and may contain cartilage and fibrovascular tissue.
52
Q

(Persistent fetal vasculature)

Natural Hx of more severe form of PFV/Autres anomalies oculaires associées à la forme plus sévère?

A

The natural history of more severely affected eyes is usually one of progressive cataract formation and anterior chamber shallowing, causing secondary glaucoma.
The glaucoma can occur
* Acutely because of rapid, total lens opacification and swelling, or
* It may develop gradually, over years.

Other features of severe PFV :
* Congenital retinal nonattachment
* Ciliary body detachment
* Vitreous hemorrhage
* Optic nerve dysmorphism

53
Q

(Persistent fetal vasculature)

Ddx du PFV dans contexte de leucocorie? Caractéristiques permettant de distinguer le PFV?

A
  • Retinoblastoma may be part of the initial differential diagnosis of PFV because of leukocoria.
  • The presence of microphthalmia and cataract are important factors in the differentiation of these disorders, as retinoblastoma is rarely found in microphthalmic eyes, and cataracts are very unusual in retinoblastoma.
54
Q

(Evaluation of Pediatric Cataracts)

V ou F :
* Anterior capsule opacities are almost always visually significant.
* Central or posterior lens opacities of sufficient density that are greater than 6 mm in diameter are usually visually significant.

A

Faux.
* In general, anterior capsule opacities are NOT visually significant unless they occlude the entire pupil.
* Central or posterior lens opacities of sufficient density that are greater than 3 mm in diameter are usually visually significant.

55
Q

(Evaluation of Pediatric Cataracts)

V ou F : Opacities that have a large area of surrounding normal red reflex or that have clear areas within them may allow good visual development.

A

Vrai.
Opacities that have a large area of surrounding normal red reflex or that have clear areas within them may allow good visual development.

56
Q

(Evaluation of Pediatric Cataracts)

Lors de l’évaluation de la fonction visuelle chez un patient avec cataracte(s), quels signes peuvent indiquer une atteinte visuelle significative (x2)?

A

Strabismus associated with a unilateral cataract and nystagmus associated with bilateral cataracts indicate that the opacities are visually significant.

Although these signs may also indicate that the optimal time for treatment has passed, cataract surgery may still improve visual function.

57
Q

(Evaluation of Pediatric Cataracts)

Which tests provide additional evidence of the visual significance of the cataract(s) in preverbal children older than 2 months?

A

In preverbal children older than 2 months, standard clinical assessment of fixation behavior, fixation preference, and objection to occlusion provide additional evidence of the visual significance of the cataract(s).

58
Q

(Evaluation of Pediatric Cataracts)

Autres tests permettant information quantitative?

A

Preferential looking tests and visual evoked potentials can provide quantitative information.

59
Q

(Evaluation of Pediatric Cataracts)

Dans quelle situation le glare testing peut être utile pour évaluer la baisse d’AV?

A

In older children, particularly those with lamellar or posterior subcapsular cataracts, glare testing may be useful for assessing decreased vision.

60
Q

(Evaluation of Pediatric Cataracts)

V ou F : Unilateral cataracts are NOT usually associated with systemic disease; laboratory tests are therefore NOT warranted in these cases.

A

Vrai.
Unilateral cataracts are not usually associated with systemic disease; laboratory tests are therefore NOT warranted in these cases.

61
Q

(Evaluation of Pediatric Cataracts)

Évaluation de la cataracte pédiatrique.

A
62
Q

(Evaluation of Pediatric Cataracts)

Ix nécessaire lorsque cataractes sont bilatérales? Si oui, quoi?

A
  • In contrast, bilateral cataracts are associated with many metabolic or other systemic diseases.
  • If the child has a positive family history of isolated congenital or childhood cataract or if examination of the parents shows lens opacities (and there are no associated systemic diseases to explain their cataracts), systemic evaluation and laboratory tests are NOT necessary.
  • A basic laboratory evaluation for bilateral cataracts of unknown etiology is outlined in Table 23-3.
  • Further workup should be directed by the presence of other systemic abnormalities.
  • Evaluation by a geneticist may be helpful for determining whether there are associated disorders and for counseling the patient’s family regarding recurrence risks.
63
Q

(Cataract Surgery in Pediatric Patients)

For optimal visual development,
* A visually significant _____ cataract should be removed before age _____ ;
* Visually significant _____ cataracts, before age _____.

A
  • In general, the younger the child, the greater the urgency to remove the cataract, because of the risk of deprivation amblyopia.
  • For optimal visual development, a visually significant unilateral cataract should be removed before age 6 weeks;
  • Visually significant bilateral cataracts, before age 10 weeks.
64
Q

(Cataract Surgery in Pediatric Patients)

Indication d’opérer des cataractes unilatérales ou bilatérales chez older children?

A
  • For older children with bilateral cataracts, surgery is indicated when the level of visual function interferes with the child’s visual needs.
  • Although children with best- corrected visual acuity of roughly 20/70 may function relatively well in early grade school, their participation in activities such as unrestricted driving may be restricted.
  • Surgery should be considered when visual acuity decreases to 20/40 or worse.
  • For older children with unilateral cataract, cataract surgery is indicated when visual acuity cannot be improved beyond 20/40.
65
Q

(Cataract Surgery in Pediatric Patients)

Facteurs influençant le choix de LIO (x2)

A

The choice of optical device for correction of aphakia depends primarily on the age of the patient and the laterality of the cataract.

66
Q

(Cataract Surgery in Pediatric Patients)

  • Âge où il est acceptable de mettre une LIO?
  • Conséquences si LIO lorsque l’enfant est plus jeune?
A
  • Intraocular lens (IOL) implantation in children aged 1–2 years and older is widely accepted.
  • The use of IOLs in younger infants, however, is associated with a higher rate of complications and larger shifts in refractive error with age.
  • Early surgical intervention followed by consistent contact lens wear and patching of the uninvolved eye usually allows development of some useful vision.
  • In most infants who are left aphakic, secondary IOL implantation can be performed after 1–2 years of age.
67
Q

(Cataract Surgery in Pediatric Patients)

V ou F : In most infants who are left aphakic, secondary IOL implantation can be performed after 1–2 years of age.

A

Vrai. In most infants who are left aphakic, secondary IOL implantation can be performed after 1–2 years of age.

68
Q

(Cataract Surgery in Pediatric Patients)

Type de cataracte unilatéral avec higher incidence of adverse events after lensectomy?

A

Infants with** mild PFV **have a higher incidence of adverse events after lensectomy compared with children with other forms of unilateral cataract, but visual outcomes are similar in both groups.

69
Q

(Cataract Surgery in Pediatric Patients)

Concernant le Management of the Anterior Capsule :
* Tearing characteristics chez l’enfant ou l’adulte?
* Elasticity of the capsule chez l’enfant versus chez l’adulte?

A
  • To enable access to the lens nucleus and cortex during cataract surgery, a capsulorrhexis is performed.
  • Because the tearing characteristics of the pediatric capsule are quite different from those of the adult capsule, lens removal techniques are modified for pediatric patients so that the risk of inadvertent extension of the tear is minimized.
  • The elasticity of the capsule is greatest in younger patients, especially infants, making continuous curvilinear capsulorrhexis more difficult.
70
Q

(Cataract Surgery in Pediatric Patients)

Solution pouvant être utilisée lorsque dense cataracts that obscure the red reflex dans le but d’améliorer la visibilité de la capsule antérieure?

A

In children with dense cataracts that obscure the red reflex, visibility of the anterior capsule can be enhanced with application of trypan blue ophthalmic solution 0.06% to the capsule.

71
Q

(Cataract Surgery in Pediatric Patients)

V ou F : Ultrasonic phacoemulsification is required dans la chx de cataracte chez les enfants

A

Faux.
Ultrasonic phacoemulsification is not required, as the lens cortex and nucleus are generally soft in children of all ages.

72
Q

(Cataract Surgery in Pediatric Patients)

Pourquoi est-il important de bien retiter tout le matériel cortical de la cataracte pédiatrique?

A

Removing all cortical material is important because of the propensity for reproliferation of pediatric lens epithelial cells.

73
Q

(Cataract Surgery in Pediatric Patients)

Types de LIO utilisés chez l’enfant :
* Acrylic?
* PMMA?
* Silicone?

A
  • Single- piece foldable acrylic IOLs, which can be placed through a 3-mm clear corneal or scleral tunnel incision, have become popular in pediatric cataract surgery, although larger single- piece polymethyl methacrylate (PMMA) lenses are also still used.
  • Silicone lenses have not been well studied in children.
74
Q

(Cataract Surgery in Pediatric Patients)

Indications capsulectomie

A
  • Many pediatric cataract surgeons leave the posterior capsule intact if the child is approaching the age when an Nd:YAG laser capsulotomy in an awake patient could be performed (usually 5 years of age).
  • Primary capsulectomy is usually preferred for younger children.
  • Studies have shown that in early childhood, the lens capsule opacifies, on average, within 18–24 months of surgery, but this can vary considerably.
75
Q

(Cataract Surgery in Pediatric Patients)

Myopic shift or Hypermetric in refractive error à 1 an versus 10 ans chez un enfant aphake? Erreur réfractive approximative en D?

A
  • Studies have shown that in aphakic pediatric eyes, a variable myopic shift in refractive error of approximately 7.00–8.00 D occurs from 1 year to 10 years of age, with a wide standard deviation.
  • This suggests that if a child is made emmetropic with an IOL at 1 year of age, refractive error at 10 years of age can be –8.00 D or greater.
  • Refractive change in children younger than 1 year is even more unpredictable.
76
Q

(Cataract Surgery in Pediatric Patients)

Facteurs à considérer lors choix de LIO (x 3)

A

Lens implantation in children requires consideration of
* The age of the child
* The target refractive error at the time of surgery
* The refractive error of the contralateral eye

77
Q

(Cataract Surgery in Pediatric Patients)

Est-il préférable de choisir une LIO pour rendre l’enfant emmetrope de l’implantation de la LIO ou rendre l’enfant emmetrope lorsqu’adulte (donc lentille moins forte rendant l’enfant hypermétrope)?

A

Some surgeons implant IOLs with powers that are expected to be required in adulthood, allowing the child to grow into the selected lens power.
* Thus, the child initially requires hyperopic correction.

Other surgeons aim for emmetropia at the time of lens implantation
* Especially in unilateral cases, believing that this approach improves the treatment of amblyopia and facilitates development of binocular function by decreasing anisometropia in the early childhood years.
* These children usually become progressively more myopic and may eventually require a second procedure to address the increasing anisometropia.

78
Q

(Cataract Surgery in Pediatric Patients)

Rx à prescrire en PO d’une chx de cataracte? Différencre entre Aphakie versus implantation LIO en PO?

A
  • If all cortical material is adequately removed, postoperative inflammation is usually mild in a child without a lens implant.
  • Topical antibiotics, corticosteroids, and cycloplegics are commonly applied for a few weeks after surgery.
  • Topical corticosteroids should be used more aggressively in children who have under gone IOL implantation.
  • Some surgeons administer intracameral corticosteroids at the time of surgery, and others use oral corticosteroids postoperatively, especially in very young children and in children with heavily pigmented irides.
  • Some surgeons administer intracameral antibiotics in addition to topical antibiotics
79
Q

(Cataract Surgery in Pediatric Patients)

Tx de l’erreur réfractive chez enfant avec aphakie bilatérale? Que type de Power faut-il favoriser et pourquoi?

A
  • For infants with bilateral aphakia, glasses are the safest and simplest method of correction.
  • They can be easily changed according to the refractive shifts that occur with growth of the eye.
  • Until the child can use a bifocal lens properly, the power selected should make the eye myopic, because most of an infant’s visual activity occurs at near.
  • Contact lenses may also be used in bilaterally aphakic patients, but they require more effort on the part of both the caregiver and the physician than do glasses.
80
Q

(Cataract Surgery in Pediatric Patients)

Options de tx de l’erreur réfractive chez enfant avec aphakie unilatérale? Avec les pour et les contres

A
  • For infants with unilateral aphakia, contact lenses are the most common method of correction.
  • Advantages of contact lenses include relatively easy power changes and the potential for extended wear with certain lenses.
  • Disadvantages include easy displacement by eye rubbing, the expense of replacement, and the risk of microbial keratitis.
  • Aphakic glasses are occasionally used in infants with unilateral aphakia who are unable to tolerate contact lenses, but these glasses are suboptimal owing to the amblyogenic effect of aniseikonia and the difficulty of wearing glasses that are much heavier on one side.
81
Q

(Cataract Surgery in Pediatric Patients)

Pertinence du Patching en PO de chx de la cataracte chez patient amblyope?

A
  • After optical correction of aphakia, patching of the better eye is necessary in patients with unilateral cataract and in some patients with bilateral cataracts if the visual acuity is asymmetric.
  • The amount of patching is based on the degree of amblyopia and the age of the child.
  • Avoidance of full- time occlusion in the neonatal period may allow stimulation of binocular vision and may help prevent strabismus.
82
Q

(Cataract Surgery in Pediatric Patients)

Complications Following Pediatric Cataract Surgery

A
  • Strabismus is very common in children following surgery for either unilateral or bilateral cataracts.
  • The risk of glaucoma is increased in children who have cataract surgery in infancy and in those with small eyes, but glaucoma often does not develop until several years after surgery.
  • Corneal decompensation is very rare in children.
  • Retinal detachments are also rare and are most likely to occur when other ocular abnormalities are present.
  • The incidence of macular edema is unknown, as it is difficult to detect ophthalmoscopically in young children and optical coherence tomography is usually not possible.
  • Postoperative endophthalmitis rarely occurs in children after cataract surgery.
83
Q

(Cataract Surgery in Pediatric Patients)

Exemples de facteurs influençant le Visual outcome after cataract surgery

A

Visual outcome after cataract surgery depends on many factors, including
* Age at onset and type of cataract
* Timing of surgery
* Choice of optical correction
* Treatment of amblyopia

Optimal vision requires careful, long- term postoperative management, particularly regarding amblyopia.

Even when congenital cataracts are detected late (after age 4 months), cataract removal combined with a strong postoperative vision rehabilitation program can achieve good vision in some eyes.

84
Q

(Cataract Surgery in Pediatric Patients)

V ou F : Early surgery by itself does not ensure a good outcome.

A

Vrai. Early surgery by itself does not ensure a good outcome.

85
Q

Type de cataracte? Et maladie associée?

A

Green Sunflower Cataract

Mx de Wilson

86
Q

Type de cataracte? Et maladies associées?

A

Multicolored flecks (Christmas tree cataract)

Hypoparathyroidism
Myotonic dystrophy

87
Q

Type de cataracte?

A

Antrior polar cataract

88
Q

Qu’est-ce que la cataracte antérieure pyramidale?

A

Une forme plus sévère de l’anterior polar cataract (APC)

89
Q

Pathologie associée à une Snowstorm cataract?

A

Snowstorm = diabète aigu

90
Q

Type de cataracte associé à la maladie de Fabry?

A

Spokelike SCP

91
Q

Pathologie associée à une Oil Droplet cataract?

A

Galactosémie

92
Q

Type de cataracte associé à la Galactosémie?

A

Oil Droplet cataract

93
Q

Nommez les différentes structures énumérées. À quelle pathologie sont-elles associées?

A

Persistent fetal vasculature (PFV)
* 1 : The tunical vasculosa lentis surrounds the lens
* 2 : Hyaloid vascular system
* 3 : Glial sheath of the hyaloid artery (Bergmeister papilla)
* 4 : Mittendorf’s dot = white opacity on the posterior surface of the lens

94
Q

(Congenital Aphakia)

Définir Congenital Aphakia?
Prévalence?

A
  • Congenital aphakia, the absence of the lens at birth, is rare.
  • This condition is usually associated with a markedly abnormal eye.
95
Q

(Spherophakia)

Définir Spherophakia?
Condition unilatérale ou bilatérale?
Complications?

A
  • A lens that is spherical and smaller than normal is termed spherophakic.
  • This condition is usually bilateral.
  • The lens may dislocate and prolapse into the anterior chamber, causing secondary glaucoma.
96
Q

(Coloboma)

Définir lens coloboma?

A
  • A lens coloboma involves flattening or notching of the lens periphery.
  • It may be associated with a coloboma of the iris, optic nerve, or retina, all of which are caused by abnormal closure of the embryonic fissure.
97
Q

(Coloboma)

V ou F : The term lens coloboma is a misnomer. Et pourquoi?

A

The term lens coloboma is a misnomer because the lens defect is caused by absence or stretching of the zonular fibers in the affected area (usually inferonasally) and is not directly due to abnormal embryonic fissure closure.

98
Q

(Coloboma)

Localisation fréquente du lens coloboma?

A

Usually inferonasally

99
Q

(Coloboma)

Localisation de la dislocation de la lens lors d’un significant lens coloboma?

A

In more significant colobomatous defects, lens dislocations may occur superiorly and temporally.

100
Q

(Dislocated Lenses in Children)

Différence entre dislocated, luxated, ectopic and subluxated?

A
  • When the lens is not in its normal position, it is said to be dislocated.
  • Subluxated (or subluxed) lenses are partly dislocated.
  • Luxated (or luxed), or ectopic, lenses are completely detached from the ciliary body; they are free in the posterior chamber, or they have prolapsed into the anterior chamber.
101
Q

(Dislocated Lenses in Children)

Ddx conditions associated with dislocated lenses?

A
102
Q

(Dislocated Lenses in Children)

Conditions associées avec des Spontaneous lens dislocation (x3)?

A

Spontaneous lens dislocation has been reported in
* Aniridia
* Buphthalmos associated with congenital glaucoma
* Congenital megalocornea with zonular weakness

103
Q

(Isolated Ectopia Lentis)

Concernant l’Isolated Ectopia Lentis :
* Localisation de la luxation?
* Condition unilatérale ou bilatérale?
* AD, AR, X-linked ou sporadique?
* Onset?
* Pathologie et mutation génétique parfois associée au Ectopia Lentis (sans les autres atteintes systémiques habituelles)?

A
  • In simple ectopia lentis, the lens is displaced superiorly and temporally.
  • The condition is usually bilateral and symmetric.
  • Most commonly, it is inherited as an autosomal dominant trait.
  • Onset can be at birth or later in life.
  • Glaucoma is common in the late- onset type.
  • Some patients with heterozygous mutations in FBN1, which cause Marfan syndrome, do not have the systemic findings associated with this syndrome and have only ectopia lentis.
104
Q

(Isolated Ectopia Lentis)

Complication souvent associée au late-onset type?

A

Glaucoma

105
Q

(Ectopia Lentis et Pupillae)

Concernant l’Ectopia Lentis et Pupillae :
* Localisation de la luxation?
* Condition unilatérale ou bilatérale?
* AD, AR, X-linked ou sporadique?

A

Ectopia lentis et pupillae is a rare autosomal recessive condition in which there is bilateral displacement of the pupil, usually inferotemporally, and dislocation of the lens in the opposite direction.

Déplacement pupille : inférotemporal
Déplacement lentille : supéronasal

106
Q

(Ectopia Lentis et Pupillae)

Autres signes associés (en plus du déplacement de la pupille et de la lentille)?

A

The condition is characterized by
* Small, round lenses (microspherophakia);
* Miosis;
* Poor pupillary dilation with mydriatics.

107
Q

(Ectopia Lentis et Pupillae)

Cause possible?

A

Ectopia lentis et pupillae may be the result of a membrane extending from a posterior origin to attach to the proximal pupil margin

108
Q

(Marfan Syndrome)

Principaux organes atteints?

A

The syndrome consists of abnormalities of the cardiovascular, musculoskeletal, and ocular systems.

109
Q

(Marfan Syndrome)

Mutation génétique associée?
Type de transmission?
% de patients avec Hx familiale négative?

A
  • It is inherited as an autosomal dominant trait, but family history is negative in 15% of cases.
  • Marfan syndrome is caused by mutations in FBN1, which provides instructions for making the protein fibrillin-1, the major constituent of extracellular microfibrils.
110
Q

(Marfan Syndrome)

Caractéristiques physiques?

A

Affected patients are characteristically
* Tall
* With long limbs (long membres) and fingers (arachnodactyly);
* Loose, flexible joints;
* Scoliosis;
* Chest deformities.

111
Q

(Marfan Syndrome)

Manifestations cardiaques?

A

Cardiovascular abnormalities are a source of significant mortality and manifest as
* Enlargement of the aortic root
* Dilation of the descending aorta
* Dissecting aneurysm
* Floppy mitral valve.

112
Q

(Marfan Syndrome)

The life expectancy of patients with Marfan syndrome is about one third that of the normal population.

A

Faux. The life expectancy of patients with Marfan syndrome is about half that of the normal population.

113
Q

(Marfan Syndrome)

% des patients avec atteintes oculaires? Atteinte oculaire la plus fréquente?

A

Ocular abnormalities occur in more than 80% of affected patients, with lens subluxation being the most common.

114
Q

(Marfan Syndrome)

Atteintes oculaires associées au Syndrome de Marfan?

A
  • In approximately 75% of cases, the lens is subluxated superiorly.
  • Typically, the zonules that are visible are intact and unbroken.
  • Examination of the iris usually shows iridodonesis and may reveal transillumination defects near the iris base.
  • The pupil is small and dilates poorly.
  • The corneal curvature is often decreased.
  • The axial length is increased, and affected patients are frequently myopic.
  • Retinal detachment can occur spontaneously, usually in the second and third decades of life.
115
Q

(Homocystinuria)

Type de transmission : AD, AR, X-linked ou sporadique?

A
  • Homocystinuria is a rare autosomal recessive condition, occurring in approximately 1 in 100,000 births.
  • The classic form is usually caused by mutations in the gene encoding cystathionine - synthase, which causes homocysteine to accumulate in the plasma and to be excreted in the urine.
116
Q

(Homocystinuria)

Prévalence?

A
  • Homocystinuria is a rare autosomal recessive condition, occurring in approximately 1 in 100,000 births.
  • The classic form is usually caused by mutations in the gene encoding cystathionine - synthase, which causes homocysteine to accumulate in the plasma and to be excreted in the urine.
117
Q

(Homocystinuria)

Manifestations extraoculaires?

A
  • The skeletal features are similar to those of Marfan syndrome.
  • Affected patients are usually tall and have osteoporosis, scoliosis, and chest deformities.
  • CNS abnormalities occur in approximately 50% of patients; intellectual disability and seizures are the most common.
  • Vascular complications are common and secondary to thrombotic disease, which affects large or medium- sized arteries and veins anywhere in the body.
  • Hypertension and cardiomegaly are also common.
  • Anesthesia carries a higher risk for patients with homocystinuria because of thromboembolic phenomena; thus, this disorder should be identified before patients undergo general anesthesia.
118
Q

(Homocystinuria)

Commentez le main ocular finding?
Localisation?
Onset?

A
  • The main ocular finding is lens subluxation, most frequently inferiorly, but the direction of subluxation is not diagnostic.
  • Subluxation typically begins between the ages of 3 and 10 years.
  • The lenses may dislocate into the anterior chamber, a finding suggestive of homocystinuria.
  • The zonular fibers are frequently broken, in contrast with those in Marfan syndrome.
119
Q

(Homocystinuria)

Dx et Tx?

A
  • The diagnosis is confirmed by the detection of disulfides, including homocystine, in the urine.
  • Medical management of homocystinuria is directed toward normalizing the biochemical abnormality.
  • Dietary management (low- methionine diet and supplemental cysteine) may be attempted.
  • Supplementation with the coenzyme pyridoxine (vitamin B6) diminishes systemic problems in approximately 50% of cases.
120
Q

(Weill- Marchesani Syndrome)

A
121
Q

(Weill- Marchesani Syndrome)

A
122
Q

Quelle pathologie est associée comme étant « l’inverse du Syndrome de Marfan »? Pourquoi?

A

Patients with Weill- Marchesani syndrome can be thought of as clinical opposites of patients with Marfan syndrome in that the former are characteristically
* Short
* With brachydactyly
* Short limbs.

123
Q

(Weill- Marchesani Syndrome)

Type de transmission : AD, AR, X-linked ou sporadique?

A
  • Inheritance can be autosomal dominant or recessive.
  • Mutations in the ADAMTS10 gene have been identified in patients with this disorder.
124
Q

(Weill- Marchesani Syndrome)

Mutation génétique associée?

A
  • Inheritance can be autosomal dominant or recessive.
  • Mutations in the ADAMTS10 gene have been identified in patients with this disorder.
125
Q

Weill-Marchesani Syndrome

Morphologie de la lentille?
Complications associées à cette morphologie?

A
  • The lenses are microspherophakic.
  • With time, the lenses may dislocate into the anterior chamber and cause pupillary block glaucoma.
  • For this reason, prophylactic laser peripheral iridectomy or lensectomy may be indicated.
126
Q

(Sulfite Oxidase Deficiency)

Prévalence?
2 principaux systèmes atteints?
Prognostic?

A
  • Sulfite oxidase deficiency (molybdenum cofactor deficiency) is a very rare hereditary disorder of sulfur metabolism manifested by severe neurologic disorders and ectopia lentis.
  • The enzyme deficiency interferes with conversion of sulfite to sulfate, resulting in increased excretion of sulfite in the urine.
  • The diagnosis can be confirmed by the absence of sulfite oxidase activity in skin fibroblasts.
  • Neurologic abnormalities include infantile hemiplegia, choreoathetosis, and seizures.
  • Irreversible brain damage and death usually occur by 5 years of age.
127
Q

Anomalies réfractives/optiques associées à une dislocation du cristallin?

A
  • With mild subluxation, the patient may be only myopic, and corrected visual function may be good.
  • More severe dislocation causes optical distortion, because the patient is looking through the far periphery of the lens.

Pédiatrie (17 decks)

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