Complications of Pediatric Cataract Surgery

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Oct 17, 2014 - ... Department of Ophthalmology, Boston Children's Hospital, Fegan 4, 300 Longwood Ave., Boston, MA 02115, ..... Borghol Kassar R, Menezo Rozalen JL, Harto Castano MA, ... and Refractive Surgery 2013;39(3):419–424.
Seminars in Ophthalmology

ISSN: 0882-0538 (Print) 1744-5205 (Online) Journal homepage: http://www.tandfonline.com/loi/isio20

Complications of Pediatric Cataract Surgery Mary C. Whitman & Deborah K. Vanderveen To cite this article: Mary C. Whitman & Deborah K. Vanderveen (2014) Complications of Pediatric Cataract Surgery, Seminars in Ophthalmology, 29:5-6, 414-420 To link to this article: http://dx.doi.org/10.3109/08820538.2014.959192

Published online: 17 Oct 2014.

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Seminars in Ophthalmology, 2014; 29(5–6): 414–420 ! Informa Healthcare USA, Inc. ISSN: 0882-0538 print / 1744-5205 online DOI: 10.3109/08820538.2014.959192

REVIEW

Complications of Pediatric Cataract Surgery Mary C. Whitman and Deborah K. Vanderveen

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Department of Ophthalmology, Boston Children’s Hospital, Harvard Medical School, Boston, Massachusetts, USA

ABSTRACT Purpose: Cataract surgery in young children poses different challenges and potential complications compared to those encountered in adult populations. We performed a literature review of the complications of pediatric cataract surgery. Methods: Literature review of complications of pediatric cataract surgery. Results: Complications in children vary based on the age of the patient at surgery and the cause of the cataract. Common events discussed include increased inflammatory response, opacification of the posterior capsule, lens reproliferation, pupillary membrane, and amblyopia; less common events include infections, significant bleeding, and retinal detachment. Conclusion: Complications after cataract surgery in children are often associated with a robust inflammatory reaction or secondary opacity and, in infants, glaucoma. Late complications can occur decades later, so that long-term follow-up is required. Though surgery carries significant risks, the consequences of no surgery and irreversible deprivation amblyopia in very young children should be considered. Keywords: Aphakia, aphakic glaucoma, complications, congenital cataract, pediatric cataract

INTRODUCTION

or due to trauma (penetrating or blunt, possibly with injury to other parts of the eye), or be a result of treatments such as radiation or corticosteroid therapy. Some children with cataracts have syndromes such as trisomy 21 or have developmental delays, which make management during the postoperative period and visual rehabilitation phase challenging. When consenting families for pediatric cataract surgery, we review the risks for cataract surgery that include immediate events, such as bleeding, inflammation, or risk of infection in the eye, and also discuss the risks of long-term potential complications, including opacity of the posterior capsule, secondary membranes, reproliferation of lens material, glaucoma, dislocation of the intraocular lens (if placed), retinal detachment, loss of vision or the eye, and need for further treatment or surgery for any of these complications. We also emphasize that while some of these events may occur early, some occur many years in the future. In addition, children must undergo general anesthesia for each surgical procedure. Finally, the importance of compliance with the visual

There are many potential causes of pediatric cataract, and the timing of onset and the cause of the cataract can affect the risk of surgical complications. Children may have congenital cataract, present at birth or shortly thereafter, which may be unilateral or bilateral. These may be caused by associated diagnoses such as genetic disorders, prenatal infections, persistent fetal vasculature (PFV), or may be idiopathic. In infants, visually significant congenital cataracts need to be removed promptly to prevent irreversible deprivation amblyopia. Many of the eyes with congenital cataract, however, also have other abnormalities that increase the risk of complications. Developmental cataracts, not present at birth but developing during early childhood, may also occur. Surgery for these cataracts can sometimes be delayed if vision is developing normally, though lamellar or posterior cataracts may progress quickly and require intervention. Some cataracts are acquired in association with other ocular diagnoses such as uveitis,

Received 18 May 2014; revised 19 June 2014; accepted 29 June 2014; published online 24 September 2014 Correspondence: Mary Whitman, Department of Ophthalmology, Boston Children’s Hospital, Fegan 4, 300 Longwood Ave., Boston, MA 02115, USA. E-mail: [email protected]

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Pediatric Cataract Complications rehabilitation program is discussed, since amblyopia in young children can result despite successful surgery, and certainly will occur without surgery. In this review, we will discuss management of the expected consequences of cataract surgery as well as the postoperative complications, and the intraoperative problems that can make postoperative complications more likely.

INFANTILE CATARACTS

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Infant Aphakia Treatment Study The Infant Aphakia Treatment Study (IATS) was a randomized, controlled, multicenter trial of infants with unilateral congenital cataract, and age57 months at surgery, comparing primary intraocular lens (IOL) implantation to aphakia and contact lens use.1–3 At both one and five years, there were no significant differences in visual acuity outcomes between the groups.4,5 Data on complications were collected meticulously, and all participating surgeons were experienced pediatric cataract surgeons. Intraoperative complications tabulated in IATS included iris prolapse, iris damage, hyphema, retained cortex, corneal clouding, lens fragment in vitreous, and posterior capsule rupture. These complications were more common in cases in which an IOL was implanted and mostly occurred during placement of the IOL.2 Within the first year of the study, the adverse events reported included reproliferation of lens material, pupillary membrane, corectopia, retinal detachment, wound dehiscence, IOL exchange, vitreous wick, and glaucoma. By five years, at least one adverse event had occurred in 56% of contact lens patients and 81% of IOL patients, the most common being lens reproliferation into the visual axis, pupillary membranes, and corectopia in the IOL group, and diagnosis of glaucoma or glaucoma suspect in the contact lens group.5 Though a common cause of unilateral cataract in infancy is persistent fetal vasculature (PFV; also known as persistent hyperplastic primary vitreous, PHPV), eyes were excluded from enrollment in IATS if the corneal diameter was59 mm or for PFV with inpulling of the ciliary processes. Even so, the rate of complications for eyes with milder forms of PFV enrolled in IATS was higher than for eyes without PFV.2

Aphakic Glaucoma and Pseudophakic Glaucoma Glaucoma is one of the most common complications after cataract surgery in infants. The typical reported incidence of aphakic glaucoma ranges from 15–45%, !

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depending on the population studied. Over one-third of cases are diagnosed within a year of cataract removal,6 but glaucoma can also develop years or decades later.7,8 The cause(s) of aphakic glaucoma are unknown, but several possible mechanisms have been suggested, including chronic trabeculitis from inflammation, blockade of the angle from retained lens material,9 changes in the trabecular meshwork due to exposure to lens epithelial cells,10 chemical factors from the vitreous, and an abnormal anterior segment, leading to both the cataract and maldevelopment of the angle. There are several risk factors for aphakic glaucoma, several of which are correlated with each other, including congenital onset of cataract, microcornea, and younger age (3 months) at cataract surgery.7,11–13 A poorly dilating pupil and congenital rubella are also risk factors. Though some have suggested that placement of an IOL at initial surgery is protective from glaucoma, usually the cohorts are biased with IOL placement only performed for older children.14,15 In the IATS trial, there was no significant difference at one year or at five years in the rate of glaucoma or diagnosis of glaucoma suspect.16 Aphakic glaucoma is usually first treated with medications: topical beta-blockers and prostaglandin inhibitors, and/or systemic carbonic anhydrase inhibitors (alphagan should not be used in infants or young children due to the risks of central nervous system depression). If the pressure cannot be normalized with medications, then glaucoma surgery should be performed. Angle surgery, such as goniotomy or trabeculotomy, as would be used for congenital glaucoma, is effective in some cases.17 Some patients will require a trabeculectomy (with mitomycin c) or tube shunt, and some may continue to require medications after surgery. If it is recognized and managed appropriately, children with aphakic glaucoma can have good visual acuity.18,19 The major cause of vision loss in young children with unilateral cataract or glaucoma is amblyopia. Children are also susceptible to steroid-induced ocular hypertension, which must be monitored in the perioperative period.20 The rise in intraocular pressure occurs earlier and in more dramatic fashion than in the adult population, and should be treated until steroids can be discontinued.

Posterior Capsular Opacity, Lens Re-proliferation, and Pupillary Membranes Posterior Capsular Opacity (PCO) is caused by proliferation of residual lens epithelial cells on the posterior capsule. In children under five years of age, posterior capsulotomy and anterior vitrectomy

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416 M. C. Whitman and D. K. Vanderveen are routinely performed as part of the initial cataract extraction to lower the incidence of PCO (which is virtually 100% without posterior capsulotomy). Additionally, reproliferation of lens material occurs in virtually all pediatric patients to some degree. In young children who are left aphakic, this results in development of the Sommering’s ring behind the iris, or Elschnig pearl formation may occur outside of the capsular leaflets. Even with IOL implantation, lens material will fill in within the capsular bag and may migrate centrally on the posterior capsule or on the anterior vitreous face. Pupillary membranes are inflammatory membranes across the pupil. All of these cause a secondary opacification of the visual axis that needs to be cleared if decreased visual responses are noted. Older children who have simple PCO may undergo office laser capsulotomy with Nd:YAG laser, and those who cannot cooperate for this can have laser capsulotomy performed under general anesthesia. Children with both PCO and reproliferation of cortical lens material or with pupillary membranes will require a second intraocular surgery (and general anesthesthetic).

Inflammation is especially worrisome in children with uveitic cataracts. The surgery is often difficult due to posterior synechiae and pupillary membranes. The surgeon (and rheumatologist) needs to aggressively manage inflammation before surgery, often with systemic medications. As with adults, inflammation should be controlled for at least three months before surgery. In young children, however, when amblyopia is a concern, there is pressure to operate as soon as possible. These children should be aggressively managed to get the eye quiet as soon as possible so that surgery can be scheduled. IOLs can be used successfully, if inflammation is controlled.27 Children are also at risk for TASS (Toxic Anterior Segment syndrome), an acute, severe intraocular inflammation with diffuse corneal edema, presenting 12–24 hours after cataract surgery.28 It represents a sterile endophthalmitis, and is thought to be a reaction to contamination or pH imbalance of solutions, viscoelastics, intraocular medications, or other items that enter the eye. In pediatric cases, TASS has been associated with ethyline-oxide sterilized vitrectomy packs.29

Infection Bleeding Bleeding after cataract surgery ranges from simple subconjunctival hemorrhage, to significant intraocular hemorrhages. Vitreous hemorrhage is not uncommon in eyes with PFV, with up to 10% incidence.12 Hyphema can be caused by damage to the iris with vitrector, instruments, or during corneal suturing. Swan syndrome, resulting from abnormal vessels at the cataract wound, can later cause spontaneous hyphema, which can be recurrent.21

In children, the incidence of infectious endophthalmitis is low (51:1000). Staph and strep are the most common organisms identified. Cases of pediatric endophthalmitis are associated with nasolacrimal duct obstruction or upper respiratory infection at the time of cataract extraction. The outcome of endophthalmitis is often poor, with over half of the patients having LP or NLP vision.30,31

Retinal Detachment Inflammation Inflammation is expected after cataract surgery, and children have a more robust inflammatory reaction than adults. Significant postoperative inflammation can lead to complications that include secondary membranes that block vision and posterior synechiae, and necessitate a second surgery. Intracameral preservative-free triamcinolone22,23 or dexamethasone24 decreases inflammation in children and is especially helpful for those undergoing IOL implantation. Dexamethasone has routinely been administered subconjunctivally at the end of pediatric cases. Adding low-molecular-weight heparin to the irrigating solution has been tried as a way to decrease inflammation, but is not very effective.25 Intraocular cefuroxime does not decrease fibrin formation, indicating fibrin formation is not due to bacterial contamination.26

In children, as in adults, cataract extraction increases the risk of future retinal detachment. Rabiah et al.32 reviewed a series of 1,017 eyes of 579 patients with cataract extraction without IOL implantation at age 516 yrs. With a mean follow-up of 6.8 years (range 2–18.3 years), they found an incidence of retinal detachment of 3.2% (33/1017). The mean time from surgery to detachment was 6.8 years (range 0.4 to 14.8 years). The risk factors they found associated with retinal detachment were more myopic (less hyperopic) aphakic refraction and wound dehiscence. Posterior capsulotomy and anterior vitrectomy were not found to be risk factors. More recently, in Denmark, in a series of 1,043 eyes of patients aged 0–17 at the time of surgery, 25 developed retinal detachment a mean of 9.1 years after surgery. They calculated the 20–year risk as 7% overall, but only 3% among patients with isolated cataract. Retinal detachment was more common in patients with intellectual Seminars in Ophthalmology

Pediatric Cataract Complications disabilities and those with other ocular or systemic anomalies.33

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Loss of Vision Although loss of vision can be caused by posterior capsular opacity or pupillary membranes (‘‘secondary cataract’’) that can be treated, or be the end result of other complications such as endophthalmitis or retinal detachment that result in poor outcomes despite treatment, the leading cause of vision loss and poor outcomes in young children and infants with cataract is amblyopia. Amblyopia is particularly challenging in unilateral cases, since the phakic eye will naturally be the preferred eye. Amblyopia therapy needs to be started promptly after surgery. The IATS protocol called for patching one hour per day per month of life (i.e., five hours per day in a five-month-old) until eight months, then half of waking hours, through at least age five.1 Compliance with correction of residual refractive error and patching, if indicated, is required. Even children who have an IOL implanted have significant refractive error in childhood, since the growth of the pediatric eye will result in a myopic shift, requiring over-correction regardless of the initial postoperative refraction. Because of the difficulties of maintaining amblyopia therapy in unilateral cases, better VA outcomes are usually found in bilateral cataract cases compared to unilateral cases.34,35 While amblyopia is not a complication of cataract surgery, lack of amblyopia treatment will result in a poor visual outcome despite successful surgery, and its importance should be recognized. On the other hand, avoidance of surgery will also result in (deprivation) amblyopia. Amblyopia remains a major barrier to good outcomes after cataract surgery in young children (58 years of age).

Need for Further Surgery in Children after Primary Cataract Surgery The most common reason for additional surgery is posterior capsule opacification. In children who did not have primary posterior capsulotomy, over 60% of one- to six-year-olds require intervention within two years from surgery.36,37 Among children 6-13 years of age at the time of surgery, only 19% require YAG capsulotomy within two years.37 Other additional surgeries include glaucoma surgery, secondary IOL for eyes left aphakic initially, and strabismus surgery. In the IATS study, 63% of patients in the IOL group required additional intraocular surgery within the first year vs. 12% in the contact lens group, most commonly for clearing the visual axis (60% IOL group, 11% CL group). Additionally, 18% of the contact lens group and 10% of the IOL group had strabismus !

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surgery within the first year.2,38 By five years, 37% percent of contact lens patients and 43% of IOL patients had strabismus surgery.5 Other studies, including a greater variety of cataract patients, also report strabismus in 24–34% of cataract patients.39,40

OTHER RISKS AND COMPLICATIONS Corneal Decompensation Children undergoing cataract surgery are also subject to other risks and complications seen in adults, such as damage to the corneal endothelium. Children with a history of cataract extraction had decreased number of endothelial cells with polymegathism and pleomorphism, unrelated to the presence of IOL.41 On long-term follow-up, a 9.2% loss of endothelial cells was noted over 12 years.42 No studies have assessed the corneas of adults who had cataract surgery as children to determine if they progress to corneal decompensation.

Loss of Accommodation In both adults and children, cataract extraction eliminates the ability to accommodate. This is a more profound loss for children, however, since most adults undergoing cataract surgery have already been presbyopic for years. Surprisingly, many children have better pseudoaccommodation than might be expected. One series of children with monofocal IOLs implanted at age 5 or older, and with low residual refractive error, showed that 50% of eyes and 75% of patients had 20/40 or better vision uncorrected at both distance and near, perhaps due to astigmatism.43 There are a few reports of using multifocal IOLs in children, but there have been no randomized controlled trials.44,45

Associated Ocular Abnormalities and Diagnoses The patient with a congenital cataract has an error in eye development, and even in cases of unilateral cataract, there can be abnormalities of the other eye. In patients treated for unilateral cataract, 41% had at least one abnormality of the phakic eye, including decreased vision (20%), nystagmus (19%), cataract (15%), iris heterochromia (9%), myopia (6%), microphthalmos (6%), pupillary miosis (2%), congenital glaucoma (2%), optic nerve abnormality (2%), aniridia (1%), and corneal opacity (1%). These abnormalities were often not apparent at the time of initial treatment, underscoring the importance of prompt treatment for the cataractous eye, as the phakic eye may have other abnormalities.46

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Traumatic Cataracts Another cause of cataracts in children is ocular trauma. These cases present a higher risk of complications due to the initial trauma and damage to other ocular structures. The surgeon must worry about zonular instability, capsular holes, and vitreous loss. Complications (especially corneal scarring, posterior synechiae, posterior capsular tear, and iris distortion) are more common with open globe injury than blunt trauma.47,48 Many trauma patients need scleral-fixation of an IOL, if an IOL can be placed.49 About half of the cases have a good visual acuity outcome, but development of amblyopia is common. Common modes of trauma in the US are paintballs, BBs, and pens and pencils.50 In contrast, in rural India, bow and arrow injuries are common.47 Perhaps unsurprisingly, the majority of these injuries occur in boys.

Bilateral vs Unilateral Surgery In children with bilateral cataracts, there is some controversy about performing surgery on both eyes under the same anesthesia, or scheduling surgery on two separate days.51 Usually, bilateral cases are done on two separate days to prevent the small chance of bilateral endophthalmitis (or bilateral TASS) if the solutions or equipment are contaminated. Others argue, however, that the small risk of bilateral endophthalmitis needs to be weighed against the risk of two general anesthesia events in a very young child, and the risk of deprivation amblyopia if the second surgery is delayed for any reason. In a developing country, the incidence of early endophthalmitis (O.16%) was found to be similar to the risk of anesthetic related death (0.11%).52 If bilateral cases are performed on the same day, a fully new set-up should be used, including re-scrub and gowning of surgeons and assistants between eyes. The total cost is estimated to be 20% lower for simultaneous surgery.53

General Anesthesia Risks There has been concern recently about the risks of general anesthesia on cognitive development in young children.54 Animals subjected to prolonged periods of high-dose inhalational anesthestics at very young ages have neuronal apoptosis. Some retrospective studies show higher risks of developmental or learning disabilities in children exposed to anesthesia.55,56 The SmartTots consortium is dedicated to further research on safe anesthetics, and has issued a consensus statement that the research results are mixed and, in the absence of conclusive evidence, it would be unethical to withhold anesthesia or sedation

for necessary surgical procedures. In each case, the risks of not treating the underlying condition need to be weighed against the potential risks of anesthesia.57

CONCLUSION Cataract surgery in children poses different risks and complications than in adults. In adults, cataract surgery can be performed with topical or local anesthesia, is quick, usually uncomplicated, and allows early restoration of vision without extensive follow-up. In children, however, general anesthesia is required, ocular anatomy and cataract morphology require alternative techniques and management, need for secondary surgery is common, and visual rehabilitation after cataract surgery is a long process that requires parental dedication. Despite these challenges, good outcomes for all ages are possible, and the consequence of not operating is usually much greater.

DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

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