16
Pediatric Cataracts David A. Plager, MD B. Christian Carter, MD VOLUME XXIX NUMBER 2 MARCH 2011 (MODULE 2 OF 3) Reviewers and Contributing Editor C. Gail Summers, MD, Editor for Pediatric Ophthalmology & Strabismus Kanwal K. Nischal, MBBS, Basic and Clinical Science Course Faculty, Section 6 Robert E. Wiggins, MD, Practicing Ophthalmologists Advisory Committee for Education Focal Points Consultants Scott R. Lambert, MD Deborah K. VanderVeen, MD Clinical Modules for Ophthalmologists

FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

  • Upload
    others

  • View
    2

  • Download
    0

Embed Size (px)

Citation preview

Page 1: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

Pediatric CataractsDavid A. Plager, MD

B. Christian Carter, MD

VOLUME XXIX NUMBER 2

MARCh 2011 (MODULE 2 Of 3)

Reviewers and Contributing Editor

C. Gail Summers, MD, Editor for Pediatric Ophthalmology & Strabismus

Kanwal K. Nischal, MBBS, Basic and Clinical Science Course Faculty, Section 6

Robert E. Wiggins, MD, Practicing Ophthalmologists Advisory Committee for Education

FocalPoints

Consultants

Scott R. Lambert, MD

Deborah K. VanderVeen, MD

Clinical Modules for Ophthalmologists

FPv29n02_0311.indd 1 1/18/11 1:27 PM

Page 2: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

Claiming CME CreditAcademy members: To claim Focal Points CME cred-

its, visit the Academy web site and access CME Central

(http://one.aao.org/CE/MyCMEPortfolio/default.aspx)

to view and print your Academy transcript and report

CME credit you have earned. You can claim up to two

AMA PRA Category 1 Credits™ per module. This will give

you a maximum of 24 credits for the 2011 subscrip-

tion year. CME credit may be claimed for up to three (3)

years from date of issue. Non- Academy members: For

assistance please send an e- mail to customer_service

@aao.org or a fax to (415) 561-8575.

Focal Points (ISSN 0891- 8260) is published quarterly by the American Acad-emy of Ophthalmology at 655 Beach St., San Francisco, CA 94109- 1336. For domestic subscribers, print with online 1- year subscription is $187 for Academy members (2 years, $337; 3 years, $477) and $252 for nonmembers (2 years, $455; 3  years, $642). International subscribers, please visit www.aao.org/focalpoints for more information. Online- only 1- year subscription is $155 for Academy members (2 years, $277; 3 years, $395) and $209 for nonmembers (2 years, $375; 3 years, $535). Periodicals postage paid at San Francisco, CA, and additional mailing offices. POSTMASTER: Send address changes to Focal

Points, P.O. Box 7424, San Francisco, CA 94120- 7424.The American Academy of Ophthalmology is accredited by the Accredita-

tion Council for Continuing Medical Education to provide continuing medical education for physicians.

The American Academy of Ophthalmology designates this educational activity for a maximum of two AMA PRA Category 1 Credits™. Physicians should only claim credit commensurate with the extent of their participation in the activity.

Reporting your CME online is one benefit of Academy membership. Non-members may request a Focal Points CME Claim Form by contacting Focal

Points, 655 Beach St., San Francisco, CA 94109- 1336.The Academy provides this material for educational purposes only. It is not

intended to represent the only or best method or procedure in every case, nor to replace a physician’s own judgment or give specific advice for case manage-ment. Including all indications, contraindications, side effects, and alternative agents for each drug or treatment is beyond the scope of this material. All information and recommendations should be verified, prior to use, with current information included in the manufacturers’ package inserts or other indepen-dent sources and considered in light of the patient’s condition and history. Reference to certain drugs, instruments, and other products in this publica-tion is made for illustrative purposes only and is not intended to constitute an endorsement of such. Some material may include information on applica-tions that are not considered community standard, that reflect indications not included in approved FDA labeling, or that are approved for use only in restricted research settings. The FDA has stated that it is the responsibility of the physi-cian to determine the FDA status of each drug or device he or she wishes to use, and to use them with appropriate informed patient consent in compliance with applicable law. The Academy specifically disclaims any and all liability for injury or other damages of any kind, from negligence or otherwise, for any and all claims that may arise out of the use of any recommendations or other information contained herein. The author(s) listed made a major contribution to this module. Substantive editorial revisions may have been made based on reviewer recommendations.

Subscribers requesting replacement copies 6 months and later from the cover date of the issue being requested will be charged the current module replacement rate.

©2011 American Academy of Ophthalmology®. All rights reserved.

ii F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1

This icon in text denotes video clips in the online edition.

http://www.aao.org/focalpoints

Focal Points Editorial Review BoardGeorge A. Stern, MD, Missoula, MT

Editor in Chief; Cornea & External Disease

William S. Clifford, MD, Garden City, KS

Glaucoma Surgery; Liaison for Practicing Ophthalmologists Advisory

Committee for Education

D. Michael Colvard, MD, fACS, Encino, CA

Cataract Surgery

Bradley S. foster, MD, Springfield, MA

Retina & Vitreous

Syndee J. Givre, MD, PhD, Raleigh, NC

Neuro-Ophthalmology

Ramana S. Moorthy, MD, fACS, Indianapolis, IN

Ocular Inflammation & Tumors

Eric P. Purdy, MD, Fort Wayne, IN

Oculoplastic, Lacrimal, & Orbital Surgery

Steven I. Rosenfeld, MD, fACS, Delray Beach, FL

Refractive Surgery, Optics & Refraction

C. Gail Summers, MD, Minneapolis, MN

Pediatric Ophthalmology & Strabismus

Focal Points StaffSusan R. Keller, Acquisitions Editor

Kim Torgerson, Publications Editor

Clinical Education Secretaries and StaffGregory L. Skuta, MD, Senior Secretary for Clinical Education,

Oklahoma City, OK

Louis B. Cantor, MD, Secretary for Ophthalmic Knowledge,

Indianapolis, IN

Richard A. Zorab, Vice President, Ophthalmic Knowledge

hal Straus, Director of Print Publications

FPv29n02_0311.indd 2 1/18/11 1:27 PM

Page 3: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1 1

IntroductionCongenital cataracts are responsible for nearly 10% of all visual loss in children worldwide, and it is estimated that 1 in 250 newborns in the United States has some form of cataract. Cataracts in children can be isolated or can be associated with systemic conditions including chro-mosomal abnormalities; craniofacial, mandibulofacial and skeletal syndromes; metabolic disorders; congenital infection; dermatologic, central nervous system, mus-culoskeletal or renal disease; or external factors such as trauma or radiation. In almost all cases of cataract associated with systemic disease, the cataracts will be bilateral (though not all bilateral cataracts are associated with systemic disease). Cataracts can also be associated with other ocular anomalies including persistent fetal vasculature, coloboma, anterior segment developmental anomaly, and aniridia.

Pediatric CataractsOnset and Inheritance

Pediatric cataracts can be congenital or acquired. Sev-eral inheritance patterns have been identified. When

ContentsIntroduction 1

Pediatric Cataracts 1• Onset and Inheritance 1

• Evaluation 4

Surgical Management 5• Considerations 5

• Postoperative Management 8

• Complications 8

Visual Rehabilitation 9• Correction of Refractive Error 9

• Amblyopia Therapy 9

• Secondary IOL Implantation 10

Conclusion 10

Clinicians’ Corner 11

Financial Disclosures

The authors, reviewers, and consultants disclose the follow-

ing financial relationships. D. Michael Colvard, MD, FACS: (C)

Abbott Medical Optics, Bausch & Lomb; (P) OASIS Medical.

Robert E. Wiggins, MD: (C) OMIC- Ophthalmic Mutual Insurance

Company. Scott R. Lambert, MD: (C) Bausch & Lomb Surgical; (S)

National Eye Institute. Steven I. Rosenfeld, MD, FACS: (C) Inspire

Pharmaceuticals; (L) Allergan. C. Gail Summers, MD: (C) McKesson;

(L) BioMarin Pharmaceutical.

The following contributors state that they have no significant financial

interest or other relationship with the manufacturer of any commer-

cial product discussed in their contributions to this module or with

the manufacturer of any competing commercial product: B. Christian

Carter, MD; William S. Clifford, MD; Bradley S. Foster, MD; Syndee

J. Givre, MD, PhD; Susan R. Keller; Ramana S. Moorthy, MD; Daniel

Mummert; Kanwal K. Nischal, MBBS; David A. Plager, MD; Eric

P. Purdy, MD; George A. Stern, MD; Kim Torgerson; Deborah K.

VanderVeen, MD.

C = Consultant fee, paid advisory boards or fees for attending a

meeting

L = Lecture fees (honoraria), travel fees or reimbursements when

speaking at the invitation of a commercial entity

P = Patents and/or royalties that might be viewed as created a poten-

tial conflict of interest

S = Grant support

Learning ObjectivesUpon completion of this module, the reader should be able to:

• Demonstrate an understanding of the variable etiologies and morphology of pediatric cataracts

• Perform an adequate examination on children who present with cataracts, and initiate the appropriate laboratory work- up, if necessary

• Determine the appropriate surgical intervention, and identify the early and late potential surgical complications of these procedures

• Implement the appropriate postoperative visual correction, including amblyopia therapy

FPv29n02_0311.indd 1 1/18/11 1:27 PM

Page 4: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

2 F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1

inherited, familial cataracts are usually autosomal dominant and always bilateral but may be asymmet-ric. X- linked and autosomal recessive inheritances have been reported but are rare. Not all inherited cataracts are congenital.

Morphology. Cataracts can involve the entire lens (total or complete cataract) or can involve only part of the lens structure. The location in the lens and morphology of the cataract provides a great deal of information about its onset, etiology, and prognosis. Any portion of the lens anatomy can become opacified, leading to numer-ous unique phenotypic presentations, such as oil droplet cataracts seen in galactosemia and Christmas tree–like cataracts in myotonic dystrophy. The clinically most common and important morphologies of partial cata-racts follow.

Anterior polar cataracts (APCs). These cataracts are common and appear as small white dots in the center of the anterior lens capsule (Figure 1). They are typically

Figure 2 nuclear cataract. (Reproduced with permission from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal M. Parks, Md.)

1 mm in diameter but can be smaller or, rarely, larger. They are thought to be a remnant of persistent tunica vasculosa lentis. These opacities are usually not visually significant and are not expected to enlarge or progress, and therefore rarely require surgery. They are congeni-tal, usually sporadic, and can be bilateral or unilateral. Anisometropia is common, so careful refraction and periodic follow- up are indicated.

Nuclear cataracts. Nuclear opacities involve the nucleus of the lens (Figure 2). They are typically approximately 3 mm in diameter, but the opacification or irregularity of the lens fibers can extend peripherally. The density of nuclear cataracts is variable and can progress. They can be unilateral or bilateral, and inherited or sporadic.

Importantly, eyes harboring nuclear cataracts usually have some degree of microcornea. This is most readily apparent in unilateral cases. These eyes are at increased risk for developing aphakic glaucoma after cataract sur-gery, and these children need to be monitored carefully throughout life.

Lamellar cataracts. These cataracts can be identified by their discrete, round (lenticular) shape affecting one or more of the “rings” in the developing lens cortex. The opacities are larger in diameter than nuclear cataracts, typically 5 mm or more (Figure 3). They are always bilat-eral, but can be asymmetric in density. Whether symmet-rical or asymmetrical, they are potentially amblyogenic. Lamellar opacities are usually acquired and can be

Figure 1 anterior polar cataract. a. slit- lamp view. b. Ret-roillumination view. (Reproduced, with permission, from Bobrow, JC, Basic and Clinical Science Course, section 11. san Francisco: american academy of ophthalmology, 2010–2011.)

a

b

FPv29n02_0311.indd 2 1/18/11 1:27 PM

Page 5: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1 3

Like nuclear cataracts, PFV is congenital, and these eyes are nearly always microphthalmic (microcornea) unless glaucoma occurs. Like posterior lenticonus, PFV is almost always unilateral. In severe cases, the lens may

inherited. These eyes are normal size and normal in cor-neal diameter. Because onset is usually after the child’s fixation reflex has been established, the visual prognosis is typically excellent following surgery.

Posterior lenticonus (lentiglobus). Posterior lenticonus is caused by a relative thinning of the central or para- central posterior capsule. This causes initially an “oil droplet” appearance on red reflex (Figure 4). With time, as the out- pouching of the lens progresses, the cortical fibers stretch and gradually opacify. This process can take many years and progress almost imperceptibly, but if the capsule tears, total opacification of the lens can occur overnight.

Posterior lenticonus opacities are almost always uni-lateral and the affected eye is of equal size to the unaf-fected eye. These opacities are not typically inherited and although the weakness in the posterior capsule may be congenital, the cataract usually does not form until later and, therefore, behaves like an acquired cataract. Visual prognosis after surgery (when indicated) can be favorable.

Persistent fetal vasculature (PFV). PFV, formerly called persistent hyperplastic primary vitreous (PHPV), is caused by failure of the fetal hyaloid vascular complex to regress. Clinically there is a retrolental membrane of varying size and density attached to the posterior lens surface. The membrane can be small and centrally located or may extend out to attach to the ciliary processes for 360° (Fig-ures 5 and 6).

Figure 4 Posterior lenticonus. (Reproduced with permission from simon JW, Basic and Clinical Science Course, sec-tion 6. san Francisco: american academy of ophthalmol-ogy; 2009–2010. Courtesy of david a. Plager, Md.)

Figure 5 Mild persistent fetal vasculature. note the persis-tent vascular stalk attached to the posterior lens capsule. (Reproduced with permission from Raab El, Basic and Clinical Science Course, section 6. san Francisco: ameri-can academy of ophthalmology; 2010–2011. Courtesy of david a. Plager, Md.)

Figure 3 lamellar cataract. Retroillumination shows size of the lamellar opacity. (Reproduced, with permission, from Raab El, Basic and Clinical Science Course, section 6, american academy of ophthalmology, 2010–2011. Courtesy of david a. Plager, Md.)

FPv29n02_0311.indd 3 1/18/11 1:27 PM

Page 6: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

4 F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1

be pushed forward, f lattening the anterior chamber and causing secondary glaucoma.

Posterior subcapsular cataracts (PSCs). These cata-racts are not common in children. When present, they are acquired, bilateral, and tend to be progressive. Sec-ondary causes for the cataracts should be sought such as exogenous or endogenous steroids, uveitis, or retinal degeneration. PSCs can also be seen with delayed onset following radiation of ocular, orbital, or craniofacial tumors. This type of cataract can be seen with neuro-fibromatosis type II, which may be the first observable manifestation of this systemic disorder.

Evaluation

All newborns deserve screening eye examinations, which should include an evaluation of the red reflexes with a direct ophthalmoscope. This test, known as the illumi-nation test, red reflex test, or Brückner test, can be used for routine ocular screening by nurses, pediatricians, and family practitioners. Ability to retinoscope through the child’s undilated pupil can help the ophthalmologist to

estimate the visual significance of an axial lens opacity in a preverbal child.

history. The ophthalmologist should elicit a detailed his-tory of the child’s growth, developmental milestones, feeding and digestive behavior, other developmental anomalies, skin lesions, and family history. A slit- lamp examination of immediate family members can reveal small, previously undiagnosed lens opacities that are visually insignificant but may reveal an inherited cause for the child’s cataracts.

Visual function. The mere presence of a cataract does not imply that surgery to remove it is indicated. That determination requires assessment of the visual signifi-cance of the lens opacity.

In infants less than 2 months of age, a normal fixation ref lex has yet to develop. Therefore the lack of strong fixation in an infant of this age with a cataract is not nec-essarily abnormal. In general, anterior capsular opacities are not visually significant unless they occlude the entire pupil, blocking out the red reflex. Central or posterior lens opacities of sufficient density that are greater than 3 mm in diameter are usually visually significant. Opac-ities that have a significant area of red ref lex around them through an undilated pupil or opacities that have clear areas within them will frequently allow for good visual development in infancy and can be observed. Stra-bismus in unilateral cataract and nystagmus in bilateral cataracts are both late signs that the opacities are visu-ally significant and the optimal time for treatment has passed, though surgery can still result in significant improvement.

In preverbal children greater than 3 months of age, standard clinical assessment of fixation behavior, fixa-tion preference, and objection to occlusion provide addi-tional evidence of visual significance of the cataract(s). Special tests such as preferential looking cards and visual evoked potentials can provide additional quantitative information, but they are generally not necessary to determine the visual significance of a cataract.

In school- age and older children, surgery for bilateral cataract should be suggested when the child’s level of visual function interferes with his or her visual needs. For instance, a child in kindergarten may function well with vision in the 20/70 to 20/100 range, while grade school and high school students have higher visual demands that require intervention at an earlier stage. For unilateral cat-aracts, vision that cannot be improved past the 20/50 to 20/70 range with optical and amblyopia treatment alone is a reasonable level to suggest cataract surgery.

Figure 6 severe persistent fetal vasculature. note 360° trac-tion on the ciliary processes by the vascularized retrolental membrane. (Reproduced with permission from Raab El, Basic and Clinical Science Course, section 6. san Fran-cisco: american academy of ophthalmology; 2010–2011. Courtesy of david a. Plager, Md.)

FPv29n02_0311.indd 4 1/18/11 1:27 PM

Page 7: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1 5

age have been shown to have an increased likelihood of best- corrected acuity of less than 20/100 when compared to infants whose surgery took place prior to 10 weeks.

Dense unilateral congenital cataracts have a more well- defined time frame by which the visual axis should be cleared to allow for maximum treatment effective-ness. Birch and Stager have shown a bilinear relationship between the age of the infant at surgery for unilateral cataracts and the ultimate visual outcome. Infants who undergo surgery prior to 6 weeks of age have a better visual potential than those whose surgery and optical rehabilitation are delayed, but very early surgery may increase the risk of aphakic glaucoma.

Wound Construction and Closure. Wound location and architecture, as well as the potential use of an IOL, depend on the age of the patient. Although some pediat-ric cataract surgeons prefer scleral tunnel incisions, the clear corneal approach has been shown to be equally safe and effective. The advantages of clear corneal incisions include preservation of conjunctival tissue in a popula-tion at risk for aphakic glaucoma, as well as increased maneuverability of instrumentation. Most pediatric cataract surgeons do not consider a pars plana/plicata approach to confer any benefit to offset the increased difficulty and potential posterior complications of that approach. Although a temporal approach can be used, the protection that the brow and an intact Bell’s phe-nomenon confer to children make the superior approach most common.

Regardless of approach, suturing the cataract inci-sion with absorbable suture such as 10- 0 Vicryl (poly-glactin 910; Ethicon, Somerville, New Jersey) is highly recommended. Frequent eye- rubbing and rambunctious behavior postoperatively in children add to the potential for postoperative wound leak.

Capsular Staining Techniques. When desired, anterior capsular staining can be accomplished with f luorescein sodium, indocyanine green (ICG), and Vision Blue (0.1% trypan blue, DORC International, Zuidland, The Neth-erlands), but the former 2 options have been largely supplanted by the increasing popularity of the latter. A dye- enhanced anterior capsulorrhexis can prove valuable in numerous settings, such as complete (white) cataracts of infancy, as well as scenarios in which the status of the anterior capsule is poorly defined (traumatic cataract).

Anterior Capsule Management. The pediatric anterior capsule has a highly elastic quality that makes its man-agement very different from capsules in adults. This elas-ticity is inherent at birth, and tends to decrease with

Ocular Examination. A slit- lamp examination should be performed to classify the morphology of the cataract and to identify any associated abnormalities of the cornea, iris, lens, and anterior chamber. Infants can be held in place at a conventional slit lamp, but a portable hand- held slit lamp is a helpful substitute.

If the cataract allows some view of the posterior seg-ment, careful observation of the optic disc, retina, and fovea should be obtained. If no view is present, B- scan ultrasonography can help rule out possible retinal and vitreal pathology.

Work-up. Unilateral cataracts are not usually associated with occult systemic or metabolic disease, and expensive laboratory tests are not warranted.

For bilateral cataracts, if a positive family history of infantile or childhood cataracts can be elicited or exam-ination of the parents’ lenses shows congenital lens opacities, a systemic and laboratory evaluation can be obviated. A basic laboratory evaluation for bilateral cat-aracts of unknown etiology in apparently healthy chil-dren would include:

• Urine for reducing substances and amino acids• TORCH (toxoplasmosis, rubella, cytomegalic

inclusion disease, Herpes simplex) titers and VDRL screening (if not done already in a newborn screen)

• Blood for calcium, phosphorous, glucose, and perhaps red cell galactokinase levels

Any further work- up should be directed by other abnor-malities in growth and development and input should be sought from a pediatric geneticist or developmental pediatrician.

Surgical ManagementConsiderations

Timing. The timing of a surgical intervention in the set-ting of visually significant pediatric cataracts depends not only on the age of onset of the opacity, but also upon whether or not the condition is unilateral or bilateral. The window of time during which visual deprivation does not induce irreversible harm to the visual system is known as the latent period. A critical period of visual development then follows, rendering a significant visual setback if the visual axis is not cleared.

For dense bilateral cataracts present at birth, a distinct latent period has remained difficult to quantify. Infants with visually significant opacities who undergo lensec-tomy and anterior vitrectomy at a point after 10 weeks of

FPv29n02_0311.indd 5 1/18/11 1:27 PM

Page 8: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

6 F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1

age. Scleral rigidity is also reduced in the pediatric eye, resulting in a positive vitreous pressure upon entering the eye. The lens is moved anteriorly into an already ana-tomically shallow anterior chamber, further stretching the anterior capsule.

Although a “can- opener” capsulotomy with a cys-totome is a viable option, most experienced pediatric cataract surgeons prefer either continuous- tear capsu-lorrhexis or vitrectorhexis because they are less prone to radial tear. Vitrectorhexis is most advantageous in chil-dren younger than 2 years whose anterior capsules are particularly elastic. A vitrector handpiece is inserted via a 20- gauge incision, while an anterior chamber main-tainer is inserted through a second paracentesis. The port is directed downward toward the capsule. Once the capsule is engaged, the handpiece is moved in a circular fashion using a rapid cutting rate to generate a capsular opening slightly smaller that of the intended optic if an IOL is planned (Figure 7).

Manual continuous curvilinear capsulorrhexis remains the gold standard for stability in pediatric cata-ract surgery; however, the elasticity of the anterior cap-sule makes the “run- away” capsulorrhexis a risk even in experienced hands. A capsular tear can be generated with a cystotome in a similar manner to adult cataract surgery, but the force vectors applied to the tear are different for children and adults. The more elastic the capsule, the closer toward 90° away from the intended direction of tear should the force be directed. Regrasp-ing the f lap frequently after a small progression of the capsulorrhexis is recommended (Figure 8). Modifications of this technique, such as 2- incision push- pull capsulor-

rhexis, have been developed to increase the reliability of completion of a manual capsulorrhexis.

ONLINE VIDEO:

Infant Capsulorrhexis, 1 min 07 sec

Lens Aspiration. The nucleus of a pediatric lens is soft but does not require phacoemulsification for removal. The cortex likewise is not rigid and maintains a strong adhesion to the lens capsule that surrounds it.

Depending on the patient’s age and plans for IOL implantation and posterior capsulotomy, a single- port irrigation/aspiration (I/A) handpiece may be used versus a small- incision bimanual technique. Children who will be unable to sit for a YAG capsulotomy within 2 years of IOL implantation, or whose capsule cannot be made clear, will require a posterior capsulotomy and anterior vitrectomy at the time of surgery. These children are per-haps best suited for the bimanual technique, where the anterior capsulotomy, lens aspiration, posterior capsu-lectomy, and anterior vitrectomy can be performed with the same instrument.

If possible, central lens material should be removed last, preventing any inadvertent anterior movement of the capsule and subsequent posterior capsular damage. This is especially true for posterior lenticonus cataracts, in which a posterior capsular defect may be pre- existing.

Aphakia Versus Pseudophakia. The decision to implant an IOL versus leaving the child aphakic depends on many factors, including age of the child, laterality, cataract

Figure 7 anterior capsulorrhexis has been performed with vitrectomy instrument and all lens cortex has been aspi-rated. note the persistent fetal vasculature plaque attached to the posterior lens capsule.

Figure 8 to manage the intended direction of the anterior capsular flap, the force vector must be different in the more elastic capsule of an infant compared to the less elastic capsule of a teenager. (Reproduced with permission from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2009–2010. schematics courtesy of david a. Plager, Md.)

FPv29n02_0311.indd 6 1/18/11 1:27 PM

Page 9: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1 7

type, eye size, family preference, and comorbidities such as uveitis. In general, IOLs have become the standard of care in children over age 1 year in the absence of other contraindications.

The role of IOL use in infants is more controversial. In addition to the technical considerations of operating on the small, soft eyes of infants, IOL implantation at less than 6 months has also been associated with a higher rate of reoperation in the first 6 postoperative months, often secondary to visual axis opacification. This is most often due to cortical proliferation and Elschnig pearl for-mation because the cataract surgery was done while the lens was still rapidly growing.

A large multicenter randomized clinical trial to evalu-ate the visual outcome of children with unilateral con-genital cataract when treated with primary pseudophakia versus aphakia and contact lens correction completed enrollment in early 2009. The Infant Aphakia Treatment Study (IATS) will also look at postoperative complica-tions as well as parental stress issues for both treatment modalities, with the goal of better defining the role of IOL implantation in infants. The earliest data from this study indicate equivalent grating visual acuity at 1 year of age between children treated with contact lenses and IOLs after cataract surgery performed prior to age 6 months. In addition, an increase in the rate of intraoperative com-plications and the need for additional intraocular surger-ies were found in the IOL group. Continued follow- up on this patient population will further clarify the risks and benefits to IOL implantation in infants.

Pseudophakic Options. The gamut of IOL designs that is available for the adult cataract surgeon are also available when lens implantation is desired in children, although all IOL use in children is considered off- label. Probably the most popular lens implanted in children at this time is the hydrophobic acrylic lens. This soft, foldable IOL can be implanted through a clear corneal incision and has proven to be quite biocompatible in the pediatric eye; however, the broad haptics make this lens a poor choice for the ciliary sulcus.

Multifocal lens technology is not widely embraced in children. The potential advantages of the multifocal lens are lost in children due to continued growth of the eye and the resulting refractive changes that can occur well into the late teens. The disadvantage may be the reduc-tion in contrast sensitivity in the diffractive IOLs and its effect on the developing visual system.

Refractive Selection. Surgeons have to consider numer-ous factors in selection of the appropriate IOL power including age of the child, refraction of the fellow eye

and, if possible, family genetics. The myopic shift that occurs in pseudophakic children has been shown to fol-low a logarithmic regression curve similar to that seen in aphakic children, though the rate of myopic shift appears to be lessened when an IOL is implanted. The variability in refractive change decreases with age, allow-ing a more accurate prediction of postoperative refrac-tion in older children. The refractive outcome of infants younger than 1  year undergoing IOL implantation is quite variable.

The knowledge of this myopic shift has directed most pediatric cataract surgeons toward planned hyperopia in the immediate postoperative period, allowing for a refractive error to approach emmetropia or minimal myopia in adulthood. Tables such as Figure  9, when used for bilateral cataracts or used in combination with the current refraction of the fellow eye in a unilateral cataract, provide a guideline for the amount of hyper-opia desired postoperatively. Variability in refractive outcomes after IOL implantation in children has been demonstrated using all of the commonly used IOL calcu-lation formulas, with increasing variability seen in the youngest and smallest eyes. Postoperatively, the residual hyperopia is corrected with spectacles or contact lenses, which can be adjusted as the refractive growth occurs.

Posterior Capsule Management and Anterior Vitrec-tomy. It is generally accepted that all infants and most toddlers should have a posterior capsulectomy performed at the time of cataract extraction (Figure 10). Although some variability exists among study populations, poste-rior capsules left intact in many pseudophakic children will develop visual axis opacification within 2  years. Therefore, if the child is felt to be of an age at surgery at

Figure 9 table of suggested goal for immediate postopera-tive refraction according to age. (Reprinted from Plager da, et al. Refractive change in pediatric pseudophakia: 6- year follow- up, J Cat Ref Surg, 2002;28:810–815.)

6.00

5.00

4.00

3.00

2.00

1.00

0.00

–1.00

–2.003 4 5 6 7

Age8 9 10 11 12 13

Ref

ract

ion

FPv29n02_0311.indd 7 1/18/11 1:27 PM

Page 10: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

8 F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1

which he or she could sit for a YAG capsulotomy within 2 years, then leaving the capsule intact at the time of sur-gery is a reasonable option. Should the capsule opacify prematurely or the child not be able to tolerate a YAG capsulotomy, a capsulectomy and anterior vitrectomy via pars plana approach can be performed. Regardless of patient age, a posterior capsule that incorporates a dense plaque or is of compromised structural integrity, such as posterior lenticonus, needs to be cleared from the visual axis at the time of surgery.

The posterior capsulotomy can be performed from the limbal or pars plana route. In children left aphakic, the limbal route is the less invasive option, allowing the lens aspiration, posterior capsulotomy, and anterior vitrec-tomy to all be performed through the same 20- gauge inci-sion. For planned pseudophakia, a pars plana approach after the IOL has been implanted allows for wider access to the posterior capsule and vitreous, making it the pre-ferred approach for many pediatric cataract surgeons.

The location of the pars plana entry wound varies according to patient age. Due to the incomplete develop-ment of the pars plana at birth, the sclerotomy must be placed closer to the limbus at younger ages. A distance of less than 2 mm posterior from the limbus in infants less than 1 year of age, 2 to 2.5 mm between 1 and 4 years, and up to 3 mm when older than 4 years have been rec-ommended. An anterior vitrectomy of up to one- third of the vitreous should help decrease secondary obscuration of the visual axis.

Subconjunctival antibiotic and/or steroid injections are optional, as is a hard shield placed over the eye at the end of surgery.

Postoperative Management

After examination on the first postoperative day, a broad spectrum topical antibiotic is prescribed for use 3 to 4 times each day and can be given in the form of a com-bination steroid- antibiotic compound. Given the robust postoperative inflammation seen in pediatric eyes when an IOL has been implanted, prednisolone 1% should be started at least 4 times per day and titrated to the degree of inf lammation that is encountered in the first post-operative weeks. Oral prednisone may sometimes be required, and a dilating agent such as atropine 1% oph-thalmic solution can be added to prevent formation of synechiae.

Complications

Surgical Complications. Tears in the posterior capsule can occur at any stage of the procedure, compounded by the fact that inherent defects in the capsule are not infrequent in children, as is seen in posterior lentico-nus cataracts. A small rent in the posterior capsule can be rounded by a vitrector, thus eliminating the need for YAG capsulotomy in the future.

The pars plana approach to posterior capsulotomy and anterior vitrectomy is associated with its own set of potential surgical complications, though the rate is very low. A sclerotomy site that is positioned excessively pos-teriorly can cause posterior segment complications such as retinal detachment or vitreous hemorrhage. Trauma to the capsular bag can occur during creation of the scle-rotomy if the microvitreoretinal blade is not inserted in an adequately posterior direction.

Postoperative Complications. The increased tissue reactivity of the pediatric eye predisposes to an increased inf lammatory response in the early postoperative period, inducing posterior synechiae, pigmented and fibrous deposits on the IOL, and secondary fibrin mem-brane formation. Risk factors include microphthalmos and age less than 6 months at the time of surgery when associated with IOL implantation.

Secondary opacification of the visual axis due to prolif-eration of lens epithelial cells can also occur and is com-mon in infants when an IOL is placed. This opacification is rarer in infants who are left aphakic or in older chil-dren even when an IOL is placed (Figure 11).

Aphakic or pseudophakic glaucoma remains a postop-erative risk for many children undergoing lensectomy, and the etiology of this condition continues to be poorly understood. The incidence of aphakic glaucoma has been reported to be as high as 41%, with an onset of disease averaging from 3.1 years to 6.8 years after lensectomy,

Figure 10 Following anterior capsulorrhexis, cortex removal, posterior capsulotomy, and anterior vitrectomy. note the anterior and posterior capsulotomies are concentric, which will allow the edges to fuse and a soemmering ring to form. this should help keep the visual axis clear.

FPv29n02_0311.indd 8 1/18/11 1:27 PM

Page 11: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1 9

disparity and awkwardness of wearing one aphakic lens, they are a less desirable option for monocular aphakia.

Aphakic contact lenses are a more popular option for the correction of aphakia in infants. Extended- wear sili-cone lenses such as the Silsoft (Bausch and Lomb, Roch-ester, New York) contact lens are probably the most common choice of pediatric cataract surgeons, though some prefer rigid gas- permeable lenses. Keratometric val-ues are not required to fit infants with the Silsoft lens, and 1 to 2 diopters of astigmatism can be masked by their wear. These lenses can be worn for 1- week dura-tions and are available in powers ranging from +12 to +32 diopters. Contact lens power can be adjusted eas-ily in the office by retinoscopic over- refraction. Apha-kic contact lenses can be a more expensive option than an intraocular lens or glasses for visual rehabilitation in part, because frequent lens loss and power changes, par-ticularly in the first year of wear, are common.

Hyperopic spectacle correction is the typical treatment of residual refractive error for both unilateral and bilat-eral pseudophakia, but in cases of unilateral pseudopha-kia with large residual refractive errors (greater than +8D), contact lens wear can be considered.

In children younger than 2 years, either aphakic or pseudophakic refractive correction should be purpose-fully overcorrected by 2 to 3 diopters, allowing the near point to be placed roughly at the child’s fingertips or a slightly greater distance. With toddlers and children more than 2 years of age, refractive correction should be targeted to optimize visual acuity at distance, with a +3.00 bifocal for near tasks.

Amblyopia Therapy

Amblyopia is frequently present in aphakic and pseu-dophakic children, both as a result of the preoperative deprivation and the postoperative anisometropia in cases of unilateral cataracts. Poor compliance with aphakic spectacles or contact lenses as well as inaccurate refrac-tion in the office further delay or prohibit the patient from obtaining the best visual outcome possible. Part- time patching therapy of the sound eye in unilateral cataract is almost always necessary, but it is frequently employed at some point in the visual rehabilitation of bilaterally affected patients as well. The only role for pharmacologic amblyopia therapy is in the setting of unilateral aphakia when the sound eye is significantly hyperopic, thus inducing a more significant amount of visual distortion than what would occur in a low hyper-ope or myope.

although it can present very soon after cataract surgery. Patients presenting with nuclear cataracts or persistent fetal vasculature have been noted to have an increased frequency of development of postoperative glaucoma, but this association is likely more related to the associ-ated microphthalmos than the type of lenticular opacity.

The incidence of postoperative endophthalmitis in children is similar to that reported in adult surgery, with a rate of 0.071% documented from 24,000 pediatric cata-ract and intraocular glaucoma cases. Endophthalmitis can be difficult to detect in young children due to lim-ited subjective information from the patient and the dif-ficulty in detecting subtle early findings.

Although some studies have reported incidences of retinal detachment in the range of 1%, the number appears to be slightly higher when follow- up periods are increased. A less hyperopic aphakic refractive error, as compared to an age- adjusted aphakic norm, and postop-erative wound dehiscence appear to be most predictive of this complication.

ONLINE VIDEO:

Infant Cataract Procedure, 2 min 47 sec

Visual RehabilitationCorrection of Refractive Error

The child who is left aphakic may be treated with aphakic glasses or contact lenses. Aphakic spectacles are effective in bilateral aphakia, but due to issues of retinal image

Figure 11 secondary opacification of the visual axis caused by epithelial proliferation behind the iol in an infant eye. note the presence of a well- centered round anterior capsu-lorrhexis and the absence of synechiae.

FPv29n02_0311.indd 9 1/18/11 1:27 PM

Page 12: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

10 F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1

Secondary IOL Implantation

As aphakic children grow older, they sometimes become intolerant of contact lenses or tire of wearing aphakic spectacles. If the eye is adequate size to contain an IOL, secondary IOL implantation may be an alternative. The most important factor in determining the site of implan-tation within the eye is the degree of capsular support that remains from the primary lensectomy. A preopera-tive dilated exam may reveal an adequate capsular shelf, on which a secondary IOL can be placed in the ciliary sulcus, but sometimes examination of the sulcus intra-operatively is required before this determination can be made.

If an adequate capsular shelf exists, an IOL can be placed in the sulcus or occasionally in the capsular bag. The broad haptics and lack of angulation of the single- piece foldable hydrophobic acrylic lenses, which work so well for in-the- bag implantation, make this lens unsuit-able for sulcus fixation due to the possibility of pro-longed iris chafe and chronic uveitis. For some children, especially those operated on in early infancy who have a well- developed Soemmering ring, secondary in-the- bag placement of the IOL is an option.

Eyes that do not have adequate capsular remnants for sulcus fixation need to have a suture- fixated posterior chamber IOL (PCIOL) or placement of an anterior cham-ber IOL (ACIOL). Neither alternative is ideal, especially for children who have many decades ahead of them in which to develop long- term complications such as suture breakage and dislocation of the PCIOL, or uve-itis, glaucoma, and hyphema in association with now- outdated rigid, closed- loop ACIOLs. Newer open- loop ACIOL designs may improve their long- term safety, but

no long- term data are yet available to support this. The aphakic iris claw lens seems to have a better long- term record in children, but it is not currently available in the United States.

ONLINE VIDEO:

Secondary In-the-Bag IOL Placement, 3 min 45 sec

ConclusionThe diagnosis of pediatric cataracts was once considered a diagnosis strictly associated with poor visual acuity; however, current diagnostic and therapeutic options for the pediatric cataract surgeon have allowed for greatly improved outcomes when employed in a timely fashion. The approach for each individual varies with the age of the patient and morphology of the cataract. Forthcom-ing studies, such as the IATS, will further ophthalmolo-gists’ ability to best treat these children and minimize vision loss from this condition.

David A. Plager, MD, is a professor of ophthalmology and the director of the Pediatric Ophthalmology and Strabismus Service at the Indiana University School of Medicine in Indianapolis, Indiana.

B. Christian Carter, MD, is in private practice in Char-lottesville, Virginia. He is also a clinical assistant profes-sor of ophthalmology at the University of Virginia School of Medicine.

FPv29n02_0311.indd 10 1/18/11 1:27 PM

Page 13: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1 11

Clinicians’Corner

1. What laboratory work- up do you recommend when you see bilateral cataracts?

Dr. Lambert: If there is a family history of hereditary cat-aracts, I generally do not perform a laboratory work- up. In all other children, I perform laboratory testing tai-lored to their particular clinical findings. The laboratory test that has had the highest yield for me is urine amino acid testing for male infants with bilateral cataracts. The urine amino acids have been elevated in 3 babies I have screened. None of the infants were known to have devel-opmental problems at the time of cataract surgery. On further testing, all of them were found to have Lowe syn-drome (oculocerebrorenal syndrome). Early diagnosis is important because of the associated developmental prob-lems and proximal renal tubular dysfunction that needs to be managed by a nephrologist.

I also routinely screen children with cataracts for serum calcium and phosphorus. I screen some infants for galactosemia with a comprehensive galactose-mia panel that tests for galactose-1-P- uridyltransferase (GALT) enzyme activity, Gal-1 P concentrations, and com-mon GALT mutations. I also screen children with lamel-lar cataracts for galactokinase enzyme activity.

I do not routinely screen all children for rubella anti-body titers because of the high rate of rubella vaccination in the United States, but I do screen infants with mothers who have immigrated to the United States from coun-tries with high rates of congenital rubella syndrome.

Dr. VanderVeen: For bilateral infantile cataracts with no family history of pediatric cataract, most importantly, I consult with the child’s pediatrician to be sure that a complete physical examination has been performed and that there are no signs of other anomalies or systemic disease. If there are any dysmorphic features or addi-tional findings, a genetics consultation is obtained. The

Clinicians’ Corner provides additional viewpoints on

the subject covered in this issue of Focal Points. Con-

sultants have been invited by the Editorial Review

Board to respond to questions posed by the Acade-

my’s Practicing Ophthalmologists Advisory Committee

for Education. While the advisory committee reviews

the modules, consultants respond without reading the

module or one another’s responses. – Ed.

FPv29n02_0311.indd 11 1/18/11 1:27 PM

Page 14: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

12 F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1

Clinicians’Corner

lens surface to the optic disc on ultrasonography, do you recommend that the surgery be performed in conjunction with a retinal specialist?

Dr. Lambert: On rare occasion, I have had a hyaloid ves-sel bleed during cataract surgery, resulting in a vitreous hemorrhage. For this reason, if there is a patent hyaloid vessel, I cauterize it with intraocular diathermy prior to extirpating it from the retrolenticular plaque. If the PFV is associated with nonattachment of the retina or the retina cannot be visualized, I refer these patients to a vitreoretinal surgeon for their management. I don’t base this decision on whether a stalk can be seen by ultraso-nography, but on the severity of the PFV and the likeli-hood of retinal involvement.

Dr. VanderVeen: If the stalk is attenuated, there is usu-ally very little bleeding, particularly when the vessel is cut transversely. Persistent pupillary vessels likewise bleed transiently and minimally. The hyaloid vessel or vessels within a PFV plaque can be cauterized prior to cutting in order to avoid significant intraocular bleeding. If the ultrasound or visualization shows a thick hyaloid stalk, or if there is any evidence of optic nerve or peripap-illary retinal distortion, then I would refer the patient to a retinal specialist.

4. What is your success rate in terms of visual out-come for children with monocular cataracts who are operated upon within the first month of life ver-sus after 1 month?

Dr. Lambert: I no longer operate on children with mon-ocular cataracts during the first month of life, because in my experience this seems to increase their risk of devel-oping glaucoma. The literature suggests that the visual results are just as good if the surgery is performed when children are 4 to 6 weeks of age versus <4 weeks of age. If the child is compliant with his or her optical correction (contact lenses or glasses if pseudophakic) and patching therapy, my experience is that he or she will generally have an excellent visual result.

Dr. VanderVeen: While there is a suggestion that visual outcomes may be slightly improved by surgery before 4  weeks, there may also be a slightly higher rate of complications. Therefore, when an infant presents

child must be cleared for general anesthesia by the pedia-trician, and I usually suggest a preoperative chest X- ray, which would detect an unrecognized cardiomyopathy. Laboratory testing, if not already done, may include ToRCHS (toxoplasmosis, rubella, cytomegalic inclusion disease, Herpes simplex, and syphilis) titers and calcium, phosphorus, and glucose levels in the blood. Testing for galactokinase deficiency may be considered if the cata-ract appears typical for this disorder or if there are other concerns about diet or growth. However, laboratory tests are almost universally unrevealing in our population, and I do not order them routinely.

2. What types of pediatric cataracts are amenable to medical management?

Dr. Lambert: Unilateral anterior polar cataracts only rarely need surgical treatment. However, some of these children develop anisometropic amblyopia, and for this reason they should be followed on a regular basis dur-ing early childhood. On the other hand, bilateral anterior polar cataracts are usually larger and may be associated with miosis. I treat some of these children with chronic pharmacological mydriasis. Unilateral posterior polar cataracts can sometimes be managed with part- time patching of the fellow eye until it becomes clear how much they are degrading vision. Likewise, some children with lamellar cataracts can be followed until they are older if the vision is carefully monitored.

Dr. VanderVeen: Small or partial cataracts may be man-aged medically. A general rule is that if the view to the fovea through the undilated pupil is clear using direct ophthalmoscopy, or if retinoscopy is possible, then the cataract is not (yet) visually significant. Anterior opaci-ties, partial or scattered lens opacities with clear inter-vening lens, or central opacities <3mm (as long as the pupil is >3mm) can often be watched. Larger or posterior opacities are more amblyogenic. Cataracts are often asso-ciated with refractive error or strabismus, so these addi-tional amblyogenic factors must be addressed in addition to deciding about surgical intervention.

3. how often is intraocular bleeding a problem in chil-dren with cataracts related to persistent fetal vas-culature (PfV)? If you see a stalk from the posterior

FPv29n02_0311.indd 12 1/18/11 1:27 PM

Page 15: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1 13

IOLs implanted in their anterior segments by other sur-geons and these children seem to be doing okay, but I’m concerned about the long- term effects of ACIOLs on the corneal endothelium and angle of a child who may live for another 70 or 80 years.

Dr. VanderVeen: I have not implanted an ACIOL in a child and have preferred sutured posterior chamber lenses when there is insufficient capsular support for in-the- bag or sulcus implantation. Newer iris- supported lenses may be a reasonable choice, though few have been implanted in pediatric eyes and long- term effects are not yet studied.

7. What is your preferred method for secondary IOL implantation in an eye without adequate capsular support? Do you perform pars plana vitrectomy in these cases?

Dr. Lambert: I have sutured IOLs in the sulcus in the past, but I have not been happy with the results. One of the Prolene sutures suspending the IOL broke in one patient and the IOLs became tilted in other patients. We have an excellent contact lens service at Emory, and in most cases we have been able to successfully fit aphakic chil-dren with contact lenses even when they were deemed “contact lens intolerant” by others.

Dr. VanderVeen: Fortunately most children with acquired aphakia have reasonable capsular support so that in-the- bag or sulcus implantation can be accomplished. If there is inadequate support, I would opt for a scleral-fixated, sutured IOL, with previously described standard tech-niques. I usually create scleral f laps, use 9-0 Prolene suture, and choose an IOL with an eyelet for the suture. Vitrectomy should be performed, as needed, and I use an anterior approach.

Scott R. Lambert, MD, is the R. Howard Dobbs Professor of Ophthalmology at Emory University, Atlanta, Georgia.

Deborah K. VanderVeen, MD, is a practicing ophthal-mologist at Children’s Hospital, Boston, and an assistant professor of ophthalmology at Harvard Medical School, Boston, Massachusetts.

with congenital cataract(s), I generally plan for surgery when the child is about 1 month old, with the youngest patients being 3+ weeks of age at the time of surgery. I have 10 to 15 eyes that were operated on at less than 4 weeks, and all others (>150) were 4 weeks or older, so it is really difficult to really compare the outcomes. Over-all, we have found that microcornea and PFV are risk factors for glaucoma or other secondary complications, rather than younger age at surgery.

5. What are your guidelines for implantation of an IOL at different ages?

Dr. Lambert: I almost always recommend IOL implan-tation in children 7 months of age or older. For infants 6 months of age or younger with bilateral cataracts, I prefer to leave them aphakic and optically correct them with either contact lenses or spectacles. When they are older I implant secondary IOLs if the parents are having problems with the contact lenses or they are bothered by the cosmesis of aphakic spectacles. For infants 6 months of age or younger with a unilateral cataract, I discuss the pros and cons of IOL implantation with the parents. I explain to them that while the visual outcome associated with IOL and contact lens correction are comparable, IOL implantation is associated with an increased incidence of intraoperative complications and additional intraocular surgeries to clear visual axis opacities. I then let the par-ents choose the treatment.

Dr. VanderVeen: I do not generally offer IOL implantation for infants less than 6 months of age, but I do explain the options of an aphakic contact lens versus IOL for those 6 to 12 months of age. Because the parents will have to deal with the contact lens or spectacle wear, and often patching after surgery, I find that active participation in decision-making is better for most families. The parents tend to be happy with whichever choice they make as long as the advantages and disadvantages of each option are explained prior to surgical intervention. Infants more than 1 year of age generally receive primary IOL implantation.

6. Do you have any experience with anterior chamber IOLs (ACIOLs)? Do you advise their use in children?

Dr. Lambert: I do not implant IOLs in the anterior cham-ber of children. I have examined a few children who had

FPv29n02_0311.indd 13 1/18/11 1:27 PM

Page 16: FocalPoints - Pediatric Ophthalmology...from Raab El, Basic and Clinical Science Course, section 6. san Francisco: american academy of ophthalmology; 2010–2011. Courtesy of Marshal

14 F o C a l P o i n t s : M o d u l E 2 , 2 0 1 1

The Infant Aphakia Treatment Study Group. A randomized

clinical trial comparing contact lens with intraocular lens

correction of monocular aphakia during infancy: grating

acuity and adverse events at age 1 year. Arch Ophthalmol.

2010;128:810–818.

Trivedi RH, Wilson ME Jr, Facciani J. Secondary intraocular lens

implantation for pediatric aphakia. J AAPOS. 2005;9:346–352.

Vasavada AR, Trivedi RH, Nath VC. Visual axis opacification

after AcrySof intraocular lens implantation in children.

J Cataract Refract Surg. 2004;30:1073–1081. Erratum in:

J Cataract Refract Surg. 2004;30:1826.

Wilson ME Jr, Trivedi RH, Pandey SK. Pediatric Cataract Surgery:

Techniques, Complications, and Management. Philadelphia, PA:

Lippincott Williams & Wilkins; 2005.

Wilson ME, Trivedi RH, Bartholomew LR, Pershing S. Compar-

ison of anterior vitrectorhexis and continuous curvilinear

capsulorhexis in pediatric cataract and intraocular lens

implantation surgery: A 10- year analysis. J AAPOS. 2007;11:

443–446.

Suggested Reading

Amaya L, Taylor D, Russell- Eggitt I, Nischal KK, Lengyel D. The

morphology and natural history of childhood cataracts. Surv

Ophthalmol. 2003;48:125–144.

Birch EE, Cheng C, Stager DR Jr, Weakley DR Jr, Stager DR Sr.

The critical period for surgical treatment of dense congenital

unilateral cataract. Invest Ophthalmol Vis Sci. 1996;37:1532–1538.

Hamada S, Low S, Walters BC, Nischal KK. Five- year experi-

ence of the 2- incision push- pull technique for anterior and

posterior capsulorrhexis in pediatric cataract surgery.

Ophthalmology. 2006;113:1309–1314.

Lambert SR, Lynn MJ, Reeves R, Plager DA, Buckley EG,

Wilson ME. Is there a latent period for the surgical treatment

of children with dense bilateral congenital cataracts? J AAPOS.

2006;10:30–36.

Plager DA, Kipfer H, Sprunger DT, Sondhi N, Neely DE.

Refractive change in pediatric pseudophakia: 6- year follow- up.

J Cataract Refract Surg. 2002;28:810–815.

Plager DA, Yang S, Neely D, Sprunger D, Sondhi N. Complica-

tions in the first year following cataract surgery with and

without IOL in infants and children. J AAPOS. 2002;6:9–14.

029033A

FPv29n02_0311.indd 14 1/18/11 1:27 PM