Lens Diseases Arturo Perez Arteaga, Gian Maria Cavallini, Boris Malyugin, Bojan Pajic
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1Lens Diseases (Clinical)
2

Congenital Lens Anomalies1

  • • Congenital Lens Anomalies
    • Bojan Pajic, Brigitte Pajic-Eggspuehler
    • Jasna Ljubic (Switzerland)
  • • Congenital Cataract
    • Arturo Perez Arteaga (Mexico)
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Congenital Lens Anomalies
Bojan Pajic, Brigitte Pajic-Eggspuehler, Jasna Ljubic (Switzerland)
 
Lenticonus Posterior
 
KEY FACTS
  • Can be associated with persistent hyperplastic primary vitreous (PHPV)
  • Ectasia of the posterior lens surface
  • Commonly bilateral, not always symmetric
  • Frequently associate with cataract
 
CLINICAL FINDINGS
  • Myopia and astigmatism, frequently progressive in power and axis
  • Monocular diplopia and anisometropia
  • Visual acuity decreasing
  • Cataract
  • Amplyopia
 
ANCILLARY TESTING
  • Biomicroscopy with the slitlamp
  • Topographic
  • Wavefront analysis
 
DIFFERENTIAL DIAGNOSIS
  • Keratoconus
  • Cataract without ectasia
  • Myopia magna with retinal astigmatism
  • Megalocornea
  • Megalophthalmus anterior
  • Microspherophakie
  • Ectopia lentis
 
TREATMENT
  • Mild expression
  • Prescribe best optical correction
  • May tolerated contact lenses for ever in cases of an isometropia and advanced myopia and astigmatism
  • Amblyopia treatment
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Fig. 1: Lenticonus posterior
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Fig. 2: Wavefront analysis of a lenticonus posterior. The PSF prove a higher order aberration. Topography analysis of the same patient is regular (here not shown)
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  • Advanced expression
  • Cataract surgery with implantation of an intraocular lens depending of the patient age
  • Amblyopia treatment
  • Options for rehabilitation are best optical correction with classes, contact lens correction, intraocular lens implantation
 
PROGNOSIS
  • Significant visual impairment is unusual
  • If not treated early in the childhood an amblyopia may lead to a significant visual acuity decrease
 
Lentiglobus
 
KEY FACTS
  • Lentiglobus is a spheric deformation of the lens surface
  • Posterior lentiglobus (90%), typically a unilateral condition
  • Usually involves only the outermost layers of the adult nucleus and the cortex
  • A hyaloid remnant is often, but not always, seen adherent to the globus
  • The rarer anterior lenticonus (lentiglobus) is often bilateral
  • An opacity is usually associated with the defect
  • Lentiglobus is associated with congenital glaucoma
  • Anterior polar cataracts, posterior lentiglobus, and unilateral PHPV generally are not associated with a systemic disorder
  • In a child who is otherwise healthy, approximately one-third of cataract cases are idiopathic
 
CLINICAL FINDINGS
  • Associated with opacities in the region of the bulge of the posterior lentiglobus
  • Visual acuity decrease
  • Binocular visual function decrease
  • Strabismus
  • Amplyopia
  • Pendular nystagmus
 
ANCILLARY TESTING
  • Biomicroscopy investigation with the slitlamp
  • Skiascopy
  • Stereo Test Lang, Hirschberg-Test, Brueckner-Test
  • Best visual acuity measurement, Moiré measurement
    7
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Fig. 3: Ultrasound biometry shows a very lens thickness in the case of lentiglobus
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  • Systemic metabolic investigation
  • Glaucoma exclusion
  • Ultrasound examination
  • In the case of a congenital cataract in an otherwise healthy child, galactokinase deficiency must be excluded
  • Fifty percent of all hereditary cataracts are new mutations
  • Between 8.3% and 23% of cataracts are familial, with autosomal dominant heredity being the most frequent mode of inheritance
 
DIFFERENTIAL DIAGNOSIS
  • Leukokorie
  • Any opacity in the anterior segment
  • Lenticonus anterior and posterior
 
TREATMENT
  • Pediatric consultation for treatment the basis desease, i.e. galactosemia
  • Treatment of a associated eye desease, i.e. congenital glaucoma
  • Amblyopia treatment for child younger than 10 years old
  • In cases with a central lens opacity, a trial of a long-acting cycloplegic agent may be used to improve visual acuity
  • Cataract surgery with implantation of an intraocular lens depending of the patient age with days or weeks after diagnosis if the opacity is clinical relevant
  • Importance of capsulorhexis posterior with vitrectomy anterior performance during cataract surgery of juvenile cataract
  • Options for rehabilitation are best optical correction with classes, contact lens correction, intraocular lens implantation
 
PROGNOSIS
  • Significant visual impairment is unusual
  • If not treated early in the childhood an amblyopia may lead to a significant visual acuity decrease
 
Lens Coloboma
 
KEY FACTS
  • Lens has a natural tendency to assume a more spherical shape
  • This phenomenon accounts for accommodation, when the circular muscle of the ciliary body contracts, allowing the zonules to relax
  • This tendency probably also explains lens coloboma, in which the lens zonules are missing in the area of a ciliary body coloboma and the lens appears notched in that area
    9
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Fig. 4: Lens coloboma
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Fig. 5: Higher order aberration analysis of lens colobom
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  • Because there are no zonules in the area of the coloboma, the lens takes on its more natural spherical shape, forming a notch in this area.
 
CLINICAL FINDINGS
  • Spherical shape
  • Refraction may get more myope
  • Decrease of accomodation
  • Visual acuity decrease
  • Binocular visual function decrease
  • Amblyopia may occur
  • Diplopia
 
ANCILLARY TESTING
  • Biomicroscopy investigation with the slitlamp
  • Looking for lentodonesis
  • Skiascopy
  • Best visual acuity measurement, Moiré measurement
  • Ultrasound examination inclusively with measurement of the lens thickness
  • Wavefront analysis of the higher order aberration
 
DIFFERENTIAL DIAGNOSIS
  • Leukokorie
  • Any opacity in the anterior segment
  • Lenticonus anterior and posterior
  • Lentiglobus
  • Primary lentodonesis
 
TREATMENT
  • Amblyopia treatment for child younger than 10 years old
  • Best possible correction with glasses or contact lenses before surgery. If the visual acuity is not satisfying than cataract surgery is suggest.
  • In cases with a central lens opacity, a trial of a long-acting cycloplegic agent may be used to improve visual acuity.
  • Cataract surgery with implantation of an intraocular lens depending of the patient age with days or weeks after diagnosis if the opacity is clinical relevant
  • Importance of capuslorhexis posterior with vitrectomy anterior performance during cataract surgery of juvenile cataract
  • Options for rehabilitation are best optical correction with classes, contact lens correction, intraocular lens implantation
    11
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Fig. 6: PSF HOA analysis which shows two focal points with consecutive diplopia
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Fig. 7: Mittendorf dot
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PROGNOSIS
  • Significant visual impairment is unusual
  • If not treated early in the childhood an amblyopia may lead to a significant visual acuity decrease
 
Mittendorf Dot
 
KEY FACTS
  • A failure to retract the anterior portion of the hyaloid artery completely may result in the formation on the posterior capsule of the lens
  • Evidence of remnants of the hyaloid artery can be found in most patients
  • In about 10% of all children, the attachment of the hyaloid artery can be seen on the posterior lens capsule inferior and slightly nasal to the posterior pole
  • A Mittendorf dot, also called spurious posterior polar cataract, can be fairly large and appear as a round, dense capsular opacity.
  • The hyaloid artery can be seen in premature infants, and occasionally the vessel persists into adult life
 
CLINICAL FINDINGS
  • A ophthalmoscopically, a persistent hyaloid artery appears as a single vessel extending from the optic disk anteriorly through Cloquet's canal.
  • It may be filled with blood but usually is bloodless after birth and can extend as far anteriorly as the posterior capsule of the lens
  • Usually, the insertion on the posterior capsule is located inferonasal to the visual axis
  • Occasionally, after the vessel has regressed, only the circular point of insertion remains
  • A remnant of the posterior primary vitreous can occasionally be identified on the optic disk.
  • This remnant, representing the embryonic point of exit of the hyaloid vascular system from the optic nerve head, is known as Bergmeister's papilla
  • Visual acuity decrease can be seen in dependency of the position of the hyaloid artery at the posterior lens membrane
  • An amplyopia can occur
 
ANCILLARY TESTING
  • Biomicroscopy investigation with the slitlamp
  • Skiascopy
  • Best visual acuity measurement, Moiré measurement
    13
 
DIFFERENTIAL DIAGNOSIS
  • Any opacity in the anterior segment
  • Lenticonus posterior
 
TREATMENT
  • Best possible correction with glasses or contact lenses
  • Amblyopia treatment for child younger than 10 years old
 
PROGNOSIS
The condition rarely interferes with vision.14
Congenital Cataract
Arturo Perez Arteaga (Mexico)
 
Introduction
A congenital cataract is an opacification of the lens present at birth. Not all cataracts are visually significant, but if a lenticular opacity is located in the visual axis, it is considered visually significant and because may lead to blindness, should require treatment. If the cataract is small, in the anterior portion of the lens, or in the periphery, no visual loss may be present; a close monitoring long-life is mandatory.
 
Investigation
The lens forms during the invagination of surface ectoderm overlying the optic vesicle. The embryonic nucleus develops by the sixth week of gestation. Surrounding the embryonic nucleus is the fetal nucleus. At birth, the embryonic and fetal nuclei make up most of the lens. In the postnatal period, cortical lens fibers are laid down from the conversion of anterior lens epithelium into cortical lens fibers. Unilateral cataracts are usually isolated sporadic incidents. They can be associated with ocular abnormalities (e.g. posterior lenticonus, persistent hyperplasic primary vitreous, anterior segment digenesis, and posterior pole tumors), trauma, or intrauterine infection, particularly rubella. Bilateral cataracts are often inherited and associated with other diseases. They require a full metabolic, infectious, systemic, and genetic investigation. The common causes are hypoglycemia, trisomy (e.g. Down's, Edwards' and Patau's syndromes), myotonic dystrophy, infectious diseases (e.g. toxoplasmosis, rubella, cytomegalovirus, and herpes simplex), and prematurity.
 
Differential Diagnosis
All close family members should be examined. Infectious causes of cataracts should be investigate and include rubella (the most common), chickenpox, cytomegalovirus, herpes simplex, herpes zoster, poliomyelitis, influenza, Epstein-Barr virus, syphilis, and toxoplasmosis. In unilateral cataracts, prenatal and family history should be taken; slitlamp examination in both eyes (dilated pupil); dilated fundus examination; laboratory studies include TORCH titers and VDRL test, are mandatory. In bilateral cataracts, prenatal and family history, slitlamp examination in both eyes (dilated pupil), dilated fundus examination, genetics evaluation and laboratory studies include CBC, BUN, TORCH titers, VDRL, urine for reducing substances, red cell galactokinase, urine for amino acids, calcium, and phosphorus.15
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Figs 1A and B: Congenital cataract
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Some other causes of leukokoria should be excluded like persistent hyperplasic primary vitreous, prematurity retinopathy and retinoblastoma.
 
Treatment
Cataract surgery is the treatment of choice and should be performed when patients are younger than 17 weeks to ensure minimal or no visual deprivation. Most ophthalmologists opt for surgery much earlier, ideally when patients are younger than 2 months, to prevent irreversible amblyopia and sensory nystagmus in the case of bilateral congenital cataracts. The delay in surgery is because of fear to glaucoma, since glaucoma occurs in 10% of congenital cataract surgery. A link to long-term visual rehabilitation is mandatory; the patient must be followed up, during the entire life.
 
Prognosis
Congenital cataracts usually are diagnosed at birth. If a cataract goes undetected in an infant, permanent visual loss may ensue. Of persons with unilateral cataracts, 40% develop vision of 20/60 or better. Of persons with bilateral congenital cataracts, 70% develop vision of 20/60 or better. Prognosis is poorer in persons with other ocular or systemic involvement.