Pediatric Aphakia Treatment

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    Good visual acuity and binocular vision in children with unilateral and bilateral cataracts may be

    attained nowadays, in much greater percentage of children following the removal of the

    congenital cataracts.(16,39,4,!6,63" #he best outcomes after surgery depend on several

    $

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    variables. #his includes the e%tend of cataract, associated ocular or systemic abnormalities, early

    diagnosis and removal of cataract, optimum optical correction, and aggressive visual

    rehabilitation for several years.(4&,".

    'nderstanding of amblyopia development and reversal, especially in the early sensitive period of

    life, is much improved. urgical techni)ues of cataract removal and possibility of the optical

    correction of apha*ic eyes have also been refined. +ritical period of visual development are the

    first few months of life. isual areas in the brain are developing rapidly in response to the visual

    stimuli from the eyes( 9,13,!3"..

    -ne should however remember that not every cataract is amblyogenic since birth. f the cataract

    is not complete and central, even nuclear cataract and increases gradually with the child/s age,

    prognosis for the improvement of vision is much better. #his is why progressive cataract, such as

    lamellar cataracts, 00, posterior lenticonus, and bilateral cataracts without nystagmus, have

    )uite a good prognosis, also when the surgery is performed a little later, after the critical period

    of visual development.(6&" n case of the complete cataract, it is better to operate in the first

    wee*s of child/s life, up to $ months of age. t is true for both monocular and binocular cataracts.

    2ppropriate conditions for the development of a good visual acuity and binocular vision are thus

    created. ow, many clinicians choose the primary monocular lens implantation (-" techni)ue,which was routinely performed in the older children, over $ years of age at least. owever, it is

    still controversial surgery in newborns as it is associated with several complications(5,$4,!5,!".

    -ther solution is postoperative apha*ia correction with contact lenses($,1!,$.,3!,36,43".

    t seems that the use of the contact lenses with secondary - later in life is more beneficial.

    owever, the crucial role in achievement of the good result of treatment is played by the proper

    visual rehabilitation. t includes appropriate optical correction and intensive obturation of the

    sound eye in case of monocular cataract. +oncomitant strabismus and nystagmus should also be

    treated with the use of appropriate methods.

    Optical correction

    Aphakic glasses2ctually, apha*ic glasses are very rarely used in the correction of postoperative monocular or

    binocular apha*ia in children.

    +ontraindication to the use of apha*ic glasses is their bad optics. 7irst of all, these glasses are

    narrowing visual field to about 3&8, increasing nystagmus in the child. #hey produce mar*ed

    retinal sie disparity in the form of enlargement of the viewed ob:ects by about 3&; (41,!3".

    2bnormal eye stimulation lead to the formation of abnormal visual retinal perception . t is

    associated with the development of amblyopia and nystagmus, and fre)uently the concomitant

    strabismus. 0rismatic effect produced by highly refractile glasses, fre)uently over

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    the lac* of cooperation with child/s parents. t seems, however, that the secondary -,

    independent of child age, is much better solution in such cases.

    Epikeratophakiat was used in the past. ?pi*eratopha*ia may correct residual apha*ia and is reversible corneal

    procedure (31,3$,". owever, this procedure is associated with several complications. >oreover,epi*eratopha*ic graft is no longer commercially available. ndications to epi*eratopha*ia may

    only be cases of the contact lenses intolerance with unilateral apha*ia, in whom -

    implantation can not be performed (serious intraocular inflammation".

    Contact lenses#he best optical device in the postoperative apha*ia is contact lenses. n children following

    unilateral cataract removal these lenses are the primary treatment associated with the obturation

    of the normal eye. +ontact lenses should be selected immediately after the congenital cataract

    surgery. @efraction should be measured with the use of computer as well as *eratometric

    measurement of the corneal toricity. >anual auto*eratorefractometer, @etinoma% Aplus (i*on"

    is used for this purpose. #here is no need to use general anesthesia (see +hapterB +oncomitanttrabismusC fig. $&".

    ?ach type of lenses should be worn during wa*ing hours and removed at night (6$"

    Types of contact lenses

    #here are 3 types of the contact lenses for pediatric useB

    1. @igid gas permeable (@G0" lenses.

    $. ilicon elastomer lenses.

    3. ygrogel lenses.

    Rigid gas permeable (RGP)lenses are the best choice in the treatment of apha*ia in children.

    owadays, the ma:ority of clinicians apply this type of contact lenses (!,$&,3&,35,43,". pecial

    fitting considerations. are re)uired in case of microphthalmic eyes following the operation of

    congenital cataract, with very steep cornea, and medium postoperative astigmatism. 2lso small

    diameter of the cornea, narrow lid slit with closely ad:acent and highly tense lids re)uire the use

    of @G0 lenses..

    enses, properly selected and fitted e%actly to the sie of cornea, are ordered for each treated

    child individually. 2fter refraction and *eratometry measurement, in case of @G0, lenses are

    selected from the trial lens set, and fluoresceine pattern should be evaluated.

    -ptical power of the lens to be ordered is calculated from the special table of the contact lens

    power and may range from 1$.& =sph to 3&.& =sph. @adius of the lens is a mean value of $

    measurements but steeper by &.1 mm than the flattest corneal reading, and ultimately dependenton the corneal toricity and fluoresceine patterns.(see 7ig. 1" 2n overall lens diameter must be

    ad:usted to the diameter of the cornea, smallest possible to enable an appropriate movement of

    about 1 mm around the center. n the youngest children, lens diameter is mar*edly lower than

    that in adults and is within the range of .5 to 9.! mm. Dright has found that a good fitting

    relationship cannot be found with silicone elastomer material (6$".

    4

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    Fig ! Fl"orescein pattern of RGP lens in infant.

    0roperly fitted lens provides a normal e%change of tears between the cornea and lens. t enables

    removal of metabolic wastes and dead con:unctival and corneal epithelial cells as well as

    maintenance of e%cellent o%ygenation of the cornea. t guarantees normal metabolism andbreathing of the cornea, comparable to those in the eyes without contact lenses.

    @G0 is the healthiest lens for the small developing eye, in comparison with other types of the

    contact lenses.

    t is also easily applicable and simple in the everyday care that is very important for parents (see

    7ig. $".

    !

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    Fig # RGP lens application in the aphakic child.

    $ilicone elastomerlenses are highly permeable for o%ygen, even higher than @G0 lenses. n the

    seventies and eighties of the last century, such contact lenses were commonly used (1,1,$6"

    2ctually, @G0 contact lenses are used in the apha*ia correction following the congenital cataract

    surgical treatment.

    -ptic power of the silicone elastomer lenses is available according to the individual orders.

    @adius of the posterior lens curvature is only 5.4 to .$ mm that does not always permit proper

    fitting, especially in small children with steep cornea. =iameter of lens is only 11.& mm. uch a

    lens is too large for small eyeball in newborns and infants. #herefore, some problems with

    application and removal of lens by parents are noted. =ue to the properties of silicon elastomer

    material in which lipidmucin deposits cumulate easily and may lead to the corneal and

    con:unctival complications, such a lens should be worn during wa*ing hours only. 2dvantages

    and disadvantages of the contact lenses are shown in #able .

    TA%&E 'AA*TAGE$ A* '$AA*TAGE$ OF AR'O+$ CO*TACT &E*$ ,ATER'A&$

    Ad-antages isad-antages

    RGP

    igh o%ygen permeability

    ome initial discomfortmall diameter =ifficult fitting procedure

    ndividual fitting =aily wear

    0osition inside tear film ?%pensive

    >ovements with blin*ing ery rarely possible adherence

    to the corneal surface+onstant tears e%change beetwen cornea

    and lens

    2ppropriate corneal nourishment

    @emoval of the metabolic wastes

    o penetration of lipidmucin deposits

    o bacterial growth

    ac* of neovasculariation

    ac* of giant papillary

    eutraliing of astigmatism (to 3 ="

    ?asy to handle

    $ilicone elastomer

    uperior corneal o%ygenation

    imited power of lenses?%tended wear possibility imited base curve

    ow loss rate ot good fitting relationship

    Dearing comfort =ifficult fitting procedure

    6

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    eutraliing of only small astigmatism ?%pensive

    ipidmucin deposits accumulation

    +orneal complications

    Giant papillary

    0oor moistening of lens surface

    0ossible adherence to the cornealurface

    ydrogel lenses. in principle, should be used in children over 4 years of life. #hese are

    commercially manufactured lenses of selected parameters only. -ptic power ranges from E4&.&

    to

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    n pediatric apha*ia lens power needed to its correction are very high and is associated with big

    central thic*ness of the lens. t ma*es very low o%ygen permeability. t may lead to several

    corneal, and con:unctival ( giant papillary, con:unctivitis, neovasculariation, infective *eratitis,

    corneal edema, endothelial polymegathism, abrasions, acute red eye reaction".t is very

    unhealthy lens for the small and developing eyeball. ts only advantage is a low price. #his lensis used in only e%ceptional cases.

    'ntraoc"lar lens ('O&)

    >ore and more clinicians implant - every year. t results, first of all, from the improvement of

    surgical techni)ues and preferences of parents for - implants versus correction with contact

    lens, facilitating later visual rehabilitation. ntraocular lens implantation is also associated with

    lower further costs (!!".

    'se of - in newborns and infants is still controversial. +hanges in refraction in the growing

    child are mar*ed and significantly differ in the individual children. #he most rapid growth and

    development of the eyeball ta*es place up to $ years of age, reaching the values similar to those

    in adults in about 6 E 5 years.. n this time, length of the eyeball increases from 15.& m to $1.& E

    $$.& mm (in adultsB $3.& to $4.& mm", and corneal refractive power from !3 =sph to 4! =sph (in

    adultsB 43 =sph to 4$ =sph". @espectively to these changes, power of - needed for

    implantation is also changing. 0rost(4!" carried out the detailed statistical studies in the group of

    13&& 0olish children, evaluating changes in the above mentioned 3 parameters in children

    followed up from the birth to 14 years of age (see +hapter -perative #echni)ues in 0ediatric

    +ataract urgeryC 7ig. 5 E 1$"

    t would advocate the secondary - implantation.

    -n the other hand, amber et al. ($!" reported the results of studies comparing - implantation

    to the primary apha*ia with contact lens correction. #he authors report that the number of the

    secondary surgical operations is larger in the group with - (visual a%is opacification

    2-CglaucomaC secondary -" than in the group of patients wearing contact lenses. Fut mean visual

    acuity has been slightly better in the - group. -ther clinicians thin* that mean visual acuity is

    similar in both groups of the treated children (1!, 1,!4,!!".

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    ,onoc"lar and binoc"lar aphakia treatment

    +ongenital monocular or binocular cataracts removal in the first wee*s of child/s life, optical

    correction and good visual rehabilitation create proper conditions for the development of good

    visual acuity and binocular vision. n monocular apha*ia, an occlusive patch must always be

    worn over the sound eye for 9&; of the wa*ing day.(4$,44,61" 7re)uent followup visits are

    necessary to reduce the occlusion as soon as possible, when vision and fi%ation of apha*ic eye

    become correct. t is evaluated with tests used in opto*inetic nystagmus (-A" or using variants

    of preferential loo*ing (0". >onocular fi%ation is being evaluated with eye fundus e%amination

    with visuscope (see +hapter +oncomitant trabismusC figs. 13, 14, 19". Dhen satisfactory visual

    acuity has been attained, strabismus and nystagmus are being treated. @esidual amblyopia is

    treated simultaneously with penaliation methods or atropine application to the sound eye. n

    such a way binocular peripheral vision is being stimulated.+oncomitant strabismus more fre)uently accompanies unilateral apha*ia whereas the nystagmus

    most often accompanies bilateral apha*ia.(4,1$"

    ?sotropia is seen in over 5&; of cases of both unilateral and bilateral apha*ia, while e%otropia in

    about $&; of cases. ypertropia is also very fre)uent, especially in the unilateral apha*ia.

    -bli)ue overaction encounters for about $!; of cases.(35 "

    +oncomitant strabismus is treated both nonsurgically and surgically. Frief scheme of treatment is

    shown in #able .

    TA%&E '' $C0E,E FOR TREAT,E*T OF C0'& AFTER +*'&ATERA& OR

    %'&ATERA& CO*GE*'TA& CATARACT RE,OA&

    9

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    #o correct the angle of strabismus, prismatic glasses are used together with e%ercises in

    hypercorrection glasses. Fotulinum to%in may also be in:ected to decrease the angle of

    strabismus or nystagmus. n case of deviations more than 3& 0= or noncompliance with

    nonsurgical treatment, surgical treatment of strabismus is necessary. -rthoptic rehabilitation is

    carried out for many years, even to E 1& years of life. =etailed strabismus management is

    described in the +hapter +oncomitant trabismu >anagement,.

    atisfactory visual acuity (&.6 E 1.&" and some degree of binocularity may be achieved in manypatients congenital unilateral and bilateral cataract, was reported by many authors

    (9,1&,$!,4$,44,!3".

    2lso my previous published studies have documented that such a good resu*ts can be achieved

    if the surgery is performed in the first wee*s of age, operated eye being fitted with contact lens,

    and ade)uate visual rehabilitation being carried out for the whole period of visual development.

    -ver 1&year observation of these children therapy enables the conclusion, that good visual

    acuity of &.5 E 1.& at distance, and 1.& at near with low fusion range is attainable in unilateral

    congenital cataract. ensory fusion and stereoacuity was achieved only by children after

    binocular congenital cataract removal. uch e%cellent results of treatment are not possible in all

    children due to various causes. #hey were achieved by about 3&; of the treated children.(3".

    Gregg and 0ar*s (15" reported a case of patient with unilateral infantile cataract who hadbifoveal fusion with good stereoacuity. #hese studies show that binocular vision with stereopsis

    may also be obtained in patients with unilateral congenital cataract.

    1&

    VISUAL REHABILITATION

    UNILATERAL APHAKIA BILATERAL APHAKIA

    OCCLUSION OF NORMAL EYE

    DAILY RGP WEARING

    PENALIZATION

    PLEOPTIC TREATMENT

    PRISMATIC CORRECTION

    ORTHOPTIC TREATMENT

    BTA INJECTIONS

    STRABISMUS/ NYSTAGMUS SURGERY

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    owever, achievement of so satisfactory results re)uire e%cellent cooperation of

    ophthalmologist with the treated child and parents. t is re)uired as much art as science.

    ?specially important is the thrust to the doctor and great patience and devotion of parents

    because the treatment is longlasting and complicated. owever, the prie is worth such an

    effort.

    References

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