Surgical Techniques in Ophthalmology (Pediatric Ophthalmic Surgery) Ashok Garg, Jorge L Alio
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Anterior Segment

Congenital Lens AnomaliesCHAPTER 1

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
  • Amblyopia
 
Ancillary Testing
  • Biomicroscopy with the slit-lamp
  • Topography
  • Wavefront analysis.
 
Differential Diagnosis
  • Keratoconus
  • Cataract without ectasia
  • Myopia magna with retinal astigmatism
  • Megalocornea
  • Megalo-ophthalmus anterior
  • Microspherophakia
  • Ectopia lentis.
 
Treatment
  • Mild expression
  • Prescribe best optical correction
  • May tolerated contact lenses for ever in cases of anisometropia and advanced myopia and astigmatism
  • Amblyopia treatment
  • 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.
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Clinical Findings
  • Associated with opacities in the region of the bulge of the posterior lentiglobus
  • Visual acuity decrease
  • Binocular visual function decrease
  • Strabismus
  • Amblyopia
  • Pendular nystagmus.
 
Ancillary Testing
  • Biomicroscopy investigation with the slit-lamp
  • Skiascopy
  • Stereo Test Lang, Hirschberg-Test, Brueckner-Test
  • Best visual acuity measurement, Moiré measurement
  • Systemic metabolic investigation
  • Glaucoma exclusion
  • Ultrasound examination
  • In the case of a congenital cataract in an otherwise healthy child, galactokinase deficiency must be excluded
  • 50% 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 disease, i.e. galactosemia
  • Treatment of a associated eye disease, 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 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.
 
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
  • 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 accommodation
  • Visual acuity decreases
  • Binocular visual function decreases
  • Amblyopia may occur
  • Diplopia
 
Ancillary Testing
  • Biomicroscopy investigation with the slit-lamp
  • 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
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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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    Fig. 3: Ultrasound biometry shows a very lens thickness in the case of lentiglobus
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    Fig. 4: Lens coloboma
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  • Best possible correction with glasses or contact lenses before surgery. If the visual acuity is not satisfying than cataract surgery is suggested.
  • 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.
 
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
  • Ophthalmoscopically, a persistent hyaloid artery appears as a single vessel extending from the optic disc 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 disc.
  • 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 amblyopia can occur.
 
Ancillary Testing
  • Biomicroscopy investigation with the slit-lamp
  • Skiascopy
  • Best visual acuity measurement, Moiré measurement
 
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.
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    Fig. 5: Higher order aberration analysis of lens coloboma
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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