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.

Peters anomaly: A 5-year experience.

BACKGROUND: Peters anomaly is a rare, congenital eye malformation characterized by an opaque cornea and blurred vision. Central corneal opacification can lead to delayed progression of visual development caused by defects in Descemet membrane and the posterior stroma. These children require several anesthetics for multiple eye examinations under anesthesia and corneal transplantation. AIMS: We sought to review the anesthetic management of patients with Peters anomaly for ophthalmologic procedures at Westchester Medical Center, a major referral center for Peters anomaly. METHODS: A retrospective chart review was completed which included pediatric patients who underwent ophthalmologic procedures related to Peters anomaly from 2013-2018. RESULTS: The charts of 35 patients with Peters anomaly were reviewed: 14 patients with Peters anomaly Type I, 10 patients with Peters anomaly Type II, and 11 patients with Peters plus syndrome. Thirty patients required three procedures on average, two examinations under anesthesia pre- and post-transplant, and anesthesia for the corneal transplant itself. The youngest patient encountered for examination under anesthesia was 39-week postconceptual age. Anesthetic time for examination under anesthesia averaged 31 minutes using a laryngeal mask airway while corneal transplant averaged 104 minutes utilizing endotracheal intubation. Postanesthesia care unit stay averaged 51 minutes following examination under anesthesia and 65 minutes after corneal transplant. All examinations under anesthesia were successfully completed without adverse events with the use of a laryngeal mask airway. This case series includes two patients with Goldenhar syndrome and Al-Gazali syndrome accompanying Peters anomaly. CONCLUSION: Although limited by its retrospective nature, this case series describes the cardiac and systemic implications of patients undergoing anesthesia with Peters anomaly. Our experience indicates that general anesthesia and airway manipulation are tolerated with minor postoperative concerns in these infants. Pediatric patients with Peters anomaly require multiple anesthetics for repeated ophthalmologic interventions. The laryngeal mask airway can be routinely utilized in infants less than 3 months of age for an eye examination under anesthesia with no airway complications noted. Perioperative providers should be aware of the multisystemic implications in patients with Peters plus syndrome.

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.

Dysregulated heme oxygenase-ferritin system in pterygium pathogenesis.

PURPOSE: Cyclooxygenase (COX)-, lipoxygenase (LOX)-, and cytochrome P450 monooxygenase (CYP)-derived eicosanoids have been implicated in ocular surface inflammation and neovascularization. These eicosanoids are subjected to regulation by enzymes, such as heme oxygenases (HOs) and ferritin. METHODS: Quantitative polymerase chain reaction and lipidomics based on liquid chromatography-tandem mass spectrometry were performed on pterygia from patients undergoing surgical pterygium excision. Control tissues consisted of donor corneas. In addition, lipidomics based on liquid chromatography-tandem mass spectrometry was performed on tears collected from patients before the surgery. RESULTS: Messenger RNA (mRNA) expression of HO-2, the constitutive HO isoform, was upregulated by 40% in pterygia compared with control tissue, whereas the mRNA level of the inducible form, HO-1, was downregulated by more than 50%. Levels of CYP4B1 mRNA showed an approximate 2-fold increase in pterygia compared with control. Lipidomic analysis of tissues indicated a moderate elevation in Prostaglandin E2 and thromboxane B2 levels in pterygia compared with control. Among the LOX-derived metabolites, the antiinflammatory-hydroxyeicosatetraenoic acid (15-HETE) levels were significantly reduced in pterygia (79.3 ± 48.11 pg/mg protein) compared with control (586.2 ± 213.5 pg/mg protein), whereas the proinflammatory LOX- and CYP4B1-derived 12-HETE levels were 10-fold higher in pterygia (2768 ± 832.3 pg/mg protein) compared with control (231.4 ± 87.35 pg/mg protein). Prostaglandin E2 and HETEs were also present in tears from patients with pterygium but were not detected in tears from healthy volunteers. The mRNA expression levels of both light and heavy chain ferritin were 60% and 30% lower, respectively, in pterygia compared with control. CONCLUSIONS: We believe that a dysfunctional HO-ferritin system leads to increased levels of proinflammatory mediators, thus contributing to the inflammation characteristic of pterygia.

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.

Misdiagnosis of hydrophilic acrylic intraocular lens optic opacification: report of 8 cases with the MemoryLens.

PURPOSE: To report 8 patients with misdiagnosis of MemoryLens intraocular lens (IOL) late postoperative calcification and the resulting potentially avoidable procedures leading to secondary complications. DESIGN: Retrospective interventional case series. PARTICIPANTS: Eight patients with an opacified hydrophilic acrylic MemoryLens. METHODS: Eight cases of MemoryLens IOL opacification that were originally misdiagnosed were reviewed. The following parameters were noted: baseline patient characteristics, time frame of IOL opacification, original misdiagnosis, subsequent surgical procedures, and postexplantation outcomes and complications. Two explanted lenses from this series were available for laboratory analyses. They underwent gross and light microscopic evaluation and scanning electron microscopy with energy-dispersive x-ray spectroscopy (EDS). MAIN OUTCOME MEASURES: Patient parameters noted above. The IOLs were examined for distribution, structure, and composition of the deposits causing opacification of their optic components. RESULTS: All 8 patients' IOLs were implanted between August 1999 and March 2000. Complaints of decreased visual function occurred 13 to 58 months after implantation. Half of the patients were initially diagnosed as having posterior capsular opacification and underwent neodymium:yttrium-aluminum-garnet laser capsulotomy. The other half were initially diagnosed with some form of vitreous involvement and underwent vitrectomies. Six of the 8 patients underwent subsequent lens exchanges, with 3 of them having postexchange complications including endophthalmitis, cystoid macular edema, and retinal detachment. Scanning electron microscopy with EDS confirmed the presence of calcified deposits on the surface of the 2 explants available for analysis. The deposits also stained positive for calcium. CONCLUSIONS: Despite previous reports, misdiagnosis of MemoryLens IOL calcification leading to optic opacification still occurs. Not recognizing this process can lead to potentially avoidable surgical procedures and increased risk of complications after repeated interventions.

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.

Microphthalmia with linear skin defects (MLS) syndrome: clinical, cytogenetic, and molecular characterization of 11 cases.

The microphthalmia with linear skin defects (MLS) syndrome (MIM 309801) is a severe and rare developmental disorder, which is inherited as an X-linked dominant trait with male lethality. In the vast majority of patients, this syndrome is associated with terminal deletion of the Xp22.3 region. Thirty-five cases have been described to date in the literature since the first description of the syndrome in the early 1990s. We now report on the clinical, cytogenetic, and molecular characterization of 11 patients, 7 of whom have not been described previously. Seven of these patients have chromosomal abnormalities of the short arm of the X-chromosome, which were characterized and defined by fluorescence in situ hybridization (FISH) analysis. Intriguingly, one of the patients displays an interstitial Xp22.3 deletion, which to the best of our knowledge is the first reported for this condition. Finally we report on the identification and molecular characterization of four cases with clinical features of MLS but apparently normal karyotypes, verified by FISH analysis using genomic clones spanning the MLS minimal critical region, and with genome-wide analysis using a 1 Mb resolution BAC microarray. These patients made it possible to undertake mutation screening of candidate genes and may prove critical for the identification of the gene responsible for this challenging and intriguing genetic disease.

Corneal surgery for severe phacoemulsification burns.

OBJECTIVE: To evaluate the outcome of corneal surgery for the treatment of severe corneal phacoemulsification burns. DESIGN: Interventional case series. PARTICIPANTS: Four patients with severe intraoperative phacoemulsification burns. INTERVENTION: One patient underwent penetrating keratoplasty and 3 underwent lamellar keratoplasties for the treatment of their corneal burns. MAIN OUTCOME MEASURES: Preoperative and postoperative visual acuities, preoperative and postoperative corneal topographies, and clinical observation. RESULTS: The visual acuity (VA) after the phacoemulsification burn was counting fingers in all patients. One patient had a penetrating keratoplasty, whereas the other 3 had lamellar grafts. After the corneal surgery, the corrected postoperative VA was 20/30 to 20/50 in all patients, with astigmatism ranging from 2.5 to 5.5 diopters. CONCLUSION: Severe phacoemulsification burns can be safely treated with corneal surgery, with acceptable postoperative visual acuities.