Integrated Intraoperative Optical Coherence Tomography for Pediatric Lamellar Corneal Transplant Surgery.

Integrated intraoperative anterior segment optical coherence tomography is an extremely valuable tool that can be used by corneal surgeons when performing pediatric lamellar corneal transplant surgery. This chapter will demonstrate its use in infants and children with a variety of corneal diseases. These diseases include congenital corneal opacities such as Peters' anomaly (both types I and II), surgical management of limbal dermoids, and its use in selective corneal transplant surgery such as DALK (deep anterior lamellar keratoplasty) or DSEK (Descemet stripping endothelial keratoplasty). These techniques could be used in children with anterior corneal scarring or storage disorders, such as mucopolysaccharidoses, or posterior endothelial disease, such as congenital hereditary endothelial dystrophy or posterior polymorphous dystrophy. This chapter will demonstrate the author's experience using this device during anterior segment corneal surgery.

Lyme disease presenting as persistent severe photophobia.

Long-term photophobia in children that has no obvious cause is uncommon and presents a diagnostic dilemma. It may produce significant discomfort and result in social isolation and school absence. We present the case of a 5-year-old boy who presented with chronic photophobia due to interstitial keratitis that was the result of Lyme disease.

Pediatric ocular surface disease associated with suspected abuse.

PURPOSE: To describe one of the largest case series of children whose ocular surface disease was strongly suspicious for nonaccidental injury (NAI). METHODS: This multicenter retrospective case series includes 4 patients whose presentations were concerning for anterior segment NAI. The history, examination, treatment, and outcomes of these patients is presented, along with a brief review of case reports in the literature. RESULTS: A broad spectrum of anterior segment findings was noted in our case series and in cases previously reported in the literature. NAI appears to be associated with bilateral and recurrent disease as well as improvement during hospitalization that is better than initially expected. CONCLUSIONS: Ocular surface NAI is a diagnosis of exclusion and necessitates a thorough history and examination. Clinician concern for ocular NAI should prompt examination or referral for signs of other bodily injuries, especially in young children. Siblings of patients who have received the diagnosis of NAI may also be at risk.

Anatomic and visual outcomes of corneal transplantation during infancy.

PURPOSE: To determine the effect of age at penetrating keratoplasty (PKP) on graft survival and visual outcome in children with corneal opacities transplanted during infancy. METHODS: In this two-center retrospective consecutive cohort study, the medical records of infants who underwent unilateral or bilateral PKP during the first year of life between 2004 and 2011 were reviewed retrospectively. PKP was categorized as early (age 0-90 days) or late (age 91-365 days). Main outcome measures were graft survival and vision (classified as poor, fair, or good, considering both testing method and age norms). RESULTS: A total of 62 eyes of 52 infants were included: 19 eyes underwent early PKP; 43 eyes, late PKP. Of the 62 eyes, 61 had central congenital corneal opacities; 1 was acquired. Median follow-up was 38.1 months (range, 12.2-150.5 months). Kaplan-Meier graft survival estimates were 0.92 at 1 year (95% CI, 0.81-0.96) and 0.61 at 5 years (0.44-0.74). Graft survival (early PKP, 73.7%; late PKP, 65.1% [P = 0.57]) did not differ between groups. Of the 55 eyes with recorded visual acuities, no significant difference existed in proportion with ambulatory or better vision at latest follow-up between early and late PKP (42.1% vs 55.6%; P = 0.61). CONCLUSIONS: Visual outcomes were better for PKP performed during infancy compared to results of prior reports of late PKP; however, clearing of congenital opacities in the first 3 months of life did not improve visual outcomes compared to later PKP. One-half of grafts survived >5 years. Early PKP did not worsen graft survival, but PKP may be technically easier to perform later in infancy.

Incidence of Peters anomaly and congenital corneal opacities interfering with vision in the United States.

PURPOSE: The aim of this study was to determine the incidence of Peters anomaly (PA) and congenital corneal opacities (CCO) interfering with vision in the United States. METHODS: We collected data from the Eye Bank Association of America (EBAA), the Eye-Bank for Sight Restoration (Eye-Bank) in New York City, the New York State Department of Health (NYSDH), and the Pediatric Keratoplasty Association (PKA). RESULTS: The EBAA data for 1996 and 1997 indicate that approximately 44,000 corneal transplants are performed annually. Of them, at least 128 are performed in infants, for various etiologies. A survey by the members of the PKA on 1995 showed that 65% of all grafts in infants are performed for PA. The NYSDH data from 1992 to 1997 indicate that the incidence of CCO interfering with vision was 2.2 infants per 100,000. Of those, PA accounted for 1.5 per 100,000. Eye-Bank data from 1988 to 1997 indicate that 12 children (1.1 per 100,000) received 23 transplants on 19 eyes for PA. CONCLUSIONS: Combining the data from all 4 sources indicates that approximately 1 infant corneal transplant is performed for every 24,000 live births and most of all CCO interfering with vision is due to PA. Applying the NYSDH and Eye-Bank data to a national birth rate of 4 million, we would expect approximately 88 children born annually in the United States with CCO interfering with vision, with at least 44 to 60 being due to PA. Many of these children may require more than 1 transplant.

The pediatric corneal infiltrate.

PURPOSE OF REVIEW: To highlight the most important causes of acquired corneal scarring and infiltrates in children. The lack of recognition of these disorders can lead to long-term visual loss in children. RECENT FINDINGS: There have been few articles on this topic in the recent ophthalmic literature, but all have stressed the need for early diagnosis and treatment of these diseases. SUMMARY: The most important causes of corneal scarring or infiltrates in children are keratoconus, phlyctenulosis, and herpes simplex keratitis.

Long-term visual prognosis in children after corneal transplant surgery for Peters anomaly type I.

PURPOSE: To evaluate the long-term visual prognosis in children with corneal transplant surgery for Peters anomaly type I. DESIGN: Retrospective review of interventional case series. METHODS: Twenty-four children treated in a university-based practice were divided into two groups for analysis: a younger preverbal group and an older group of children three years of age or older. Children underwent corneal transplantation surgery (penetrating keratoplasty [PKP]) for Peters anomaly type I as infants (age range, two to 18 months). Visual acuity using Snellen or Allen charts and glaucoma and other complications were tabulated. RESULTS: Twenty-four patients had Peters anomaly; 16 had unilateral disease, eight had bilateral disease. Thirty eyes underwent PKP. Average age at PKP was five months. The mean follow-up from PKP to the most recent visit was 78.9 months. Fifteen eyes (50%) were treated for glaucoma. Five transplants (17%) had graft rejection episodes; two of these failed and were regrafted. Six eyes (20%) required cataract surgery. One eye had a retinal detachment. Currently, 27 eyes (90%) have clear grafts. In the younger group of children, five of six grafts are clear (83%). In the older group of 24 eyes of verbal children, seven eyes (29%) have visual acuity ranging from 20/20 to 20/50, six (25%) have visual acuity ranging from 20/60 to 20/100, nine (38%) have visual acuity ranging from 20/200 to counting fingers, and two eyes (8%) have visual acuity of hand movements. In this group, nine of 12 eyes without glaucoma had visual acuity better than 20/100; only four of 11 eyes with glaucoma were better than 20/100. CONCLUSIONS: Many children with PKP for Peters anomaly type I can experience good or functional vision in their operated eye. Children with glaucoma have a poorer visual prognosis.

Efficacy of nonsimultaneous bilateral LASIK after nonsimultaneous bilateral penetrating keratoplasty.

PURPOSE: To show if nonsimultaneous bilateral laser in situ keratomileusis (LASIK) is a safe and effective procedure for patients with bilateral penetrating keratoplasty (PKP). METHODS: Five patients (10 eyes), with keratoconus, underwent PKP separately in each eye. After an average PKP follow-up of 45.8 months per eye, (range, 19-92 months), each eye underwent 1-stage LASIK using the Visx Star S3 laser to correct its residual refractive error. We used the cycloplegic refraction as the target for the LASIK surgery. Each patient had his or her eyes done separately 3 weeks apart. RESULTS: Pre-LASIK myopia averaged -3.93 +/- 2.9 D, ranging from -10.00 to +0.75 D. Pre-LASIK astigmatism averaged -3.25 +/- 0.80 D, ranging from -1.75 to -4.50 D. Mean pre-LASIK keratometry was 45.4 +/- 2.6, ranging from 42.2 to 50.5. Uncorrected visual acuity pre-LASIK averaged 20/220, ranging from 20/60 to 20/400. Best-corrected spectacle visual acuity (BCSVA) pre-LASIK averaged 20/22, ranging from 20/20 to 20/30. Nine eyes had no complications. One eye had a flap buttonhole during LASIK, and surgery was aborted. No vision was lost in this eye. Mean follow-up after LASIK in the 9 eyes was 17 +/- 15.2 months, (range, 4-56 months). Mean post-LASIK ametropia in these 9 eyes was +0.25 +/- 0.45 D, ranging from -0.50 to +0.75 D. Average post-LASIK astigmatism was -0.33 +/- 0.38 D, ranging from 0 to -0.75 D. In the 9 treated eyes, uncorrected vision post-LASIK averaged 20/25, ranging from 20/20 to 20/30. BCSVA post-LASIK averaged 20/21, ranging from 20/20 to 20/25. No lines of visual acuity were lost in any of the eyes. CONCLUSION: Young patients who have had bilateral PKP, with good postoperative vision and low levels of myopia, astigmatism, and minimal wound override, are good candidates for bilateral nonsimultaneous LASIK. Further studies can now be done on the performance of bilateral simultaneous LASIK in patients who have had corneal transplant surgery in both of their eyes.

Visual and refractive results of combined PTK/PRK in patients with corneal surface disease and refractive errors.

PURPOSE: To investigate the changes in symptoms, refraction, and visual acuity (VA) in patients with corneal surface disease and refractive errors who had phototherapeutic keratectomy (PTK) combined with photorefractive keratectomy (PRK). SETTING: University-based referral practice. METHODS: Patients with myopia or astigmatism and map-dot-fingerprint dystrophy or recurrent erosions were treated. The corneal epithelium was removed with a 64 blade, and laser was performed using a Visx Star S3 laser. RESULTS: Nineteen eyes of 14 patients were treated. Preoperatively, mean myopia was -3.76 diopters (D) (range -7.50 to -0.75 D), mean astigmatism was +0.96 D (range 0.00 to +2.25 D), and mean UCVA was 20/400. At 3 months, mean myopia was -0.53 D (range -1.75 to +0.75 D) (P<.001), mean astigmatism was +0.58 D (range 0.00 to +1.25 D) (P = .05), and mean uncorrected VA was 20/23 (P<.001). At 6 months, mean myopia was -0.31 D (range -1.00 to +0.75 D) (P<.001), mean astigmatism was +0.56 D (range 0.00 to +1.25 D) (P = .05), and mean uncorrected VA was 20/23 (P<.001). The change in astigmatism was confirmed by vector analysis. Only 1 patient lost 1 line of acuity, and all patients were asymptomatic. CONCLUSION: Patients with myopia and astigmatism and symptomatic epithelial basement membrane disorders who had PTK/PRK had resolution of their symptoms and nearly achieved emmetropia. Therefore, this procedure is safe and effective for patients with corneal surface disease and myopia.