Defining Success in Infant Penetrating Keratoplasty for Developmental Corneal Opacities Anagha Medsinge, M.D. 1 Lynne Speedwell, F.C.Optom., M.Sc.2 Ken K. Nischal, M.D., F.R.C.Ophth.1, 2

ABSTRACT Background and Purpose: Neonatal corneal opacities (NCO) are one of the common causes of visual impairment in infants. We present the two infants with penetrating keratoplasty (PKP) for unilateral NCO with different visual and graft outcomes, and illustrate the importance of timing of surgery, importance of visual rehabilitation, and the lack of necessity of a clear graft to achieve reasonable visual function in infants with NCO. Patients and Methods: Two infants with unilateral NCO (Peters anomaly) underwent PKP at age of 5.5 weeks (Case One) and 16 weeks (Case Two). Postoperative optical correction, amblyopia therapy, visual and graft outcomes were recorded. Results: At the last follow-up (9.5 years in both the cases), Case One achieved a best-corrected visual acuity (BCVA) of 20 / 80 with –22 D of contact lens. The graft had a small clear zone centrally but otherwise was opacified to some extent. Case Two achieved a BCVA of 20 / 125 at 30 cms eccentrically with a clear graft. Case Two was uncooperative for amblyopia therapy and optical treatment. Conclusion: For a successful visual outcome in NCO, early PKP, aggressive amblyopia therapy, optical correction, and commitment from the parents for longterm follow-up and demanding treatment are required.

From the 1Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania; however, the principal work was carried out at the 2Great Ormond Street Hospital for Children, London, United Kingdom. Requests for reprints should be addressed to: Ken K. Nischal, M.D., F.R.C.Ophth., Department of Ophthalmology, Children’s Hospital of Pittsburgh of UPMC, 4401 Penn Avenue, FP 5105, Pittsburgh, PA 15224; e-mail: nischalkk@ upmc.edu

INTRODUCTION Anterior segment developmental anomalies (ASDA) are known to be often associated with poor visual prognosis. 1-4 Historically, the outcome of penetrating keratoplasty (PKP) in children has been reported as being poor.5-7 The term “out-

© 2014 Board of Regents of the University of Wisconsin System, American Orthoptic Journal, Volume 64, 2014, ISSN 0065-955X, E-ISSN 1553-4448

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come” has had different meanings to different authors. There are fifteen published series of PKP in neonatal corneal developmental opacity (e.g., Peters anomaly, sclerocornea); of these, two documented outcome mainly in terms of visual acuity,1, 8 two reports state only graft survival as the outcome of PKP9, 10 and in eleven series, both the issues (visual acuity and graft survival / graft clarity) are described as outcomes. 2-4, 11-18 However, very few of these reports have defined what they mean by graft success or clear grafts,3, 10, 11 and rarely a definition of failure has been given.11 Furthermore, as our surgical techniques have improved and our understanding of the nature of ASDA has improved, so have perceived outcomes.11, 12 We have recently proposed a new classification for NCO that suggest that causes of NCO secondary to iridocorneal adhesions should do well in terms of graft survival.19 This has been borne out by other authors.12 Other authors have published good results in children with unilateral ASDA, also with iridocorneal adhesions.18 We present two cases of unilateral NCO secondary to iridocorneal adhesions (also known as Peters anomaly type 1) who had unilateral PKP. Both cases were operated and fitted with contact lenses and all follow-up was carried out at a pediatric teaching hospital in London, England. We want to illustrate the importance of timing of surgery, the importance of visual rehabilitation, and the lack of necessity of a clear graft to achieve reasonable visual function.

postoperatively, the cycloplegic refraction was –17.00 / –3.00 D x 80° in the right eye. She was treated with a contact lens, which she tolerated well. Patching was started initially 1-2 hours daily, increasing slowly to 3 hours / day. She developed latent nystagmus and patching was eventually increased to 4 days per week.21 She developed neovascularization of the graft, which was cauterized, but was left with marked opacification of the graft. Patching was eventually stopped 8.5 years postoperative. Intraocular pressure and posterior segment examination was normal in both the eyes. Visual acuity with –22 D CL in the right eye was 20/80 and it was 20/36 with +5.0/–2.50 x 70° CL in the left eye. At follow-up, 9.5 years after surgery (at age of 9 years 9 months), the graft had a small (3 mm) clear zone centrally, but otherwise was opacified to some extent with new vessels (Figure 1). CASE TWO An Afro-Caribbean female was born after an uneventful pregnancy. She was diagnosed with unilateral NCO secondary to iridocorneal adhesions in the left eye. Examination of the right eye was normal. After her parents were counseled, she was scheduled for left PKP as soon as possible, but the first available date where appropriate corneal material was available was at 16 weeks of age. Surgery was postponed for 1 week as the child was unwell on the day of initial surgery date. Again the technique and postoperative management were as previously described.20 Postoperatively, 1 hour / day patching was initiated in the right eye. Two months postoperative, she had a refractive error of –1.50 D. The graft remained

CASE ONE A female child was born after an uneventful pregnancy. She was diagnosed with asymmetrical NCO secondary to iridocorneal adhesions worse in the right eye as compared to the left. After her parents were counseled, she underwent right PKP at age of 5.5 weeks. The technique and postoperative management were as previously described.20 Patching of the left eye was initiated after surgery. Three months

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FIGURE 1: Case One at the last follow-up showing corneal graft with central clear zone (white arrow), peripheral opacification with new vessels (black arrow). (Photo courtesy of author.)

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FIGURE 2: Case Two at the last follow-up showing clear corneal graft (arrow). (Photo courtesy of author.) transparent; however, at 6 and 17 months postoperatively, there were endothelial graft rejections that were successfully treated with oral steroids. Six months postoperatively, her cycloplegic refraction was –5.00 / –4.00 x 70° in the left eye. The child was not cooperative for spectacle wear or contact lens or patching. Hence, penalization with atropine three times per week was started. Eighteen months postoperatively, her refractive error was –18.00 D, she was markedly photophobic in the operated eye, and she was still intolerant to contact lenses. Despite repeated attempts at optical wear, patching, and penalization, the child refused to cooperate. At 23 months postoperative, patching was stopped. At follow-up, 9.5 years after surgery, intraocular pressure and posterior segment examination were normal in both the eyes. The graft was clear (Figure 2). Visual acuity was 20 / 20 in the right eye and 20 / 125 at 30 cm eccentrically in the left eye with –22 D.

DISCUSSION NCO secondary to iridocorneal adhesions can prevent infants from developing vision. The degree of visual deprivation depends on the size, location, and density of the opacity. Visual deprivation early in life can affect negatively on neurobiological development of the child.22 As has been shown in unilateral congenital cataracts, there is a critical period after which visual outcomes are much poorer. This is thought to be around 6 weeks of age.23 It makes sense that surgery for stimulus depriv-

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ing unilateral corneal opacity should be similarly treated. It is interesting that the studies published thus far, even if they distinguish between unilateral and bilateral cases, rarely discuss timing of surgery for unilateral cases.1, 11 Previous studies have evaluated surgical outcomes following PKP for Peters anomaly (PA), but those reporting visual outcomes are sparse.2-8, 11-18 The poor visual outcomes in the majority of the studies were attributed to preexisting ocular abnormalities other than PA, severity of initial corneal opacity, delayed surgical intervention, serious postoperative complications, lack of consistency of optical treatment, and compliance with amblyopia therapy. Few papers emphasize the importance of amblyopia therapy1, 11-14, 18, 25 and correlation between the visual outcome and status of the corneal graft.2, 4, 11-18 Previous studies on PKP in infants for congenital corneal opacities, which have reported visual acuity data, usually report a dismal prognosis. Grafts may remain clear, but visual outcomes are frequently disappointing. In these reports generally, only one quarter to one-half of their patients had vision better than 20 / 200.1, 3, 8, 13 Out of fourteen papers, only seven emphasize the importance of amblyopia treatment (Table). Good visual outcomes in twenty-four children with Type 1 PA in a series by Zaidman et al. could be attributed to only including patients with iridocorneal adhesions, and their aggressive postoperative regimen with amblyopia therapy. They concluded that good visual outcome can be achieved in these patients with early surgery during infancy and if their amblyopia is co-managed with a pediatric ophthalmologist.12 Najjar et al. emphasized early and aggressive amblyopia therapy in bilateral PA. However, milder forms of PA benefited more with amblyopia therapy.25 In their series, only five patients received amblyopia

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Nijjar / 2006

Yang / 2009

Rezende / 2004 Basdekidou / 2011

McClellan / 2003 Chang / 2013

3.

4.

5.

7.

9.

8.

Gollamudi / 1994

Michaeli / 2005

2.

6.

Zaidman / 2007

Author / Year

1.

Sr. No.

18 / 30

22 (23)

3 (3)

14 (14)

16 (29)*

47 (72 )

25 (50)

17 (26)

24 (30)

Total no. of patients (Eyes)

20 / 400 (44%) CSM-7 (72%) 20 / 50 to 20 / 200-3 Poor fixation-1 1 / 60

20 / 20-20 / 50(29%) 20 / 60-20 / 100(25%) 20 / 200 to CF (38%) HM = 8% FF-5 Nystagmus-4 20 / 40 to 20 / 60-3

Defining success in infant penetrating keratoplasty for developmental corneal opacities.

Neonatal corneal opacities (NCO) are one of the common causes of visual impairment in infants. We present the two infants with penetrating keratoplast...
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