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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., (© 2020 John Wiley & Sons Ltd.)

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.

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.

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., ((c) 2005 Wiley-Liss, Inc.)

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.

Purpose: The conventional treatment of Nocardia keratitis is with topical sulfonamides. Recently, topical trimethoprim and sulfamethoxazole (Bactrim) has been suggested as treatment. This study compares the in vitro efficacy against Nocardia asteroides of Bactrim and various ratios of trimethoprim and a sulfonamide., Methods: Antibiotic disks were soaked with various ratios of trimethoprim and sulfacetamide sodium. They contained trimethoprim alone, sulfacetamide sodium alone, and both trimethoprim and sulfacetamide sodium at ratios of 1:40, 1:20, and 1:5. Disks containing Bactrim were also prepared. Each disk was placed on blood agar plates streaked with N. asteroides. The plates were incubated at 37 degrees C for 72 hours and then examined., Results: Trimethoprim alone showed minimal effect. Sulfacetamide sodium alone had a clearance zone of 12 mm. The plates of trimethoprim and sulfacetamide sodium at ratios of 1:40, 1:20, and 1:5 had clearance zones of 14 mm, 17 mm, and 27 mm, respectively. Bactrim had a clearance zone of 70 mm., Conclusion: Trimethoprim or sulfacetamide sodium alone is not as effective as both drugs together. As the ratio of the two drugs was changed, potency differed against Nocardia organisms. Bactrim was the most effective antibiotic against Nocardia organisms. It should be the recommended agent for the treatment of Nocardia keratitis.

Three children were born with partial corneal opacity and anterior segment anomalies but no cataract (Peter's anomaly type 1). In each affected eye, the corneal scar was off center and encroached on the visual axis. Glaucoma (if present) was controlled medically or surgically, after which an optical iridectomy was performed in each eye (in lieu of a penetrating keratoplasty). After surgery, in all patients the pupil extended beyond the corneal opacity and the corneal opacity decreased slightly. All could fixate and follow around the opacity. Optical iridectomy should be considered in selected cases of congenital corneal opacities.

Background and Objective: To determine whether ensured graft acceptance through the use of corneal autografts yields a better outcome than when standard homografts are used., Patients and Methods: Four patients who needed a corneal transplant in one eye and who had a blind fellow eye underwent a corneal autograft in the eye with corneal pathology., Results: Three of the four patients had clear grafts on long-term follow-up, and no patient experienced immunologic graft rejection. However, two of the four patients had large amounts of astigmatism postoperatively., Conclusion: Autografts provided protection against immunologic graft rejection. However, because corneal autografts yielded unpredictable amounts of astigmatism, their outcomes are not necessarily better than those of homografts.

Purpose: To describe a new manifestation of Peters' anomaly., Methods: We managed four infants with an unusual form of Peters' anomaly. One eye of each patient had a thickened and scarred cornea, mimicking a corneal staphyloma, protruding anteriorly from the corneal plane. The other eye of each patient ranged from normal to having severe ocular anomalies. A corneal transplant was performed in each case., Results: Follow-up ranged from 1 to 3 years. Three eyes maintained graft clarity for at least 1 year. Each of these eyes developed vision. Two of these eyes developed glaucoma. The one eye with graft failure developed an inoperable retinal detachment. The histopathology of each corneal button showed changes consistent with Peters' anomaly., Conclusions: These corneas demonstrated characteristics of both Peters' anomaly and congenital anterior staphyloma. Despite their severe anomalies, surgery successfully restored a more normal cosmetic appearance in all four eyes and vision in three eyes.

Purpose: This study aimed to alert ophthalmologists as to their role in the diagnosis of rabies., Methods: A 13-year-old girl was admitted with acute encephalitis of unknown etiology. Bacterial and viral cultures and test results for Lyme disease and tuberculosis were negative. Initial cerebrospinal fluid, serum, skin, and saliva specimens were negative for rabies. A corneal impression test was performed., Results: Immunofluorescent antibody staining of the epithelial cells on the corneal impression test was positive for rabies. Subsequently, the diagnosis was confirmed by serum serologic analysis and saliva testing., Conclusion: Ophthalmologists can assist in the diagnosis of rabies by using the corneal impression test. Corneal smears should be part of the routine antemortem work-up for presumptive rabies.

LD (with its ocular manifestations) is a worldwide disorder that is increasing in frequency. It is a treatable multisystemic disease that presents in three stages of severity. It can present with unusual forms of conjunctivitis, keratitis, cranial nerve palsies, optic nerve disease, uveitis, vitreitis, and other forms of posterior segment inflammatory disease. A patient with any of these ocular manifestations should be questioned for exposure to an area endemic for LD, tick bites, skin rash, or arthritis. Such patients should undergo serological testing. If the clinical presentation is suggestive of LD, a course of oral antibiotics should be used (unless the patient gives a history of adequate therapy). Topical corticosteroids can be used for anterior segment inflammation. An antibiotic therapeutic trial can be used for posterior segment or neuroophthalmic disease. Systemic corticosteroids without concomitant antibiotics should not be used in the treatment of ocular LD. If ocular LD is discovered and treated early, response to therapy usually is satisfactory.

Crouzon's syndrome is an autosomal dominant disorder characterized by premature craniosynostosis leading to multiple ocular abnormalities and vision loss. Strabismus, optic atrophy, exposure keratitis, hypertelorism, nystagmus, and unexplained visual loss have been reported. Keratoconus has been described only once previously in a patient with other ocular abnormalities. This article reports bilateral keratoconus in a patient with Crouzon's syndrome without other ocular pathology. We emphasize the importance of early recognition and therapy of this treatable cause of decreased vision in Crouzon's syndrome.

Lyme disease (with its ocular manifestations) is a worldwide disorder that is rapidly increasing in frequency. It is a treatable, multisystemic disease that presents in three stages of severity. It can present with unusual forms of conjunctivitis, keratitis, cranial nerve palsies, optic nerve disease, uveitis, vitritis, and other forms of posterior segment inflammatory disease. A patient with any of these ocular manifestations should be questioned for exposure to an area endemic for Lyme disease, tick bites, skin rash, or arthritis. Such patients should undergo serological testing. If the clinical presentation is suggestive of Lyme disease, a course of oral antibiotics should be used (unless the patient gives a history of adequate therapy). Topical corticosteroids can be used for anterior segment inflammation. An antibiotic therapeutic trial can be used for posterior segment or neuroophthalmic disease. Systemic corticosteroids without concomitant antibiotics should not be used in the treatment of ocular Lyme disease. If ocular Lyme disease is discovered and treated early, response to therapy is usually satisfactory.

A case of miconazole corneal toxicity is reported. This was seen in a patient who had had a penetrating keratoplasty for Acanthamoeba keratitis; the patient was treated with miconazole postoperatively. The miconazole toxicity manifested itself as a row of pinpoint vesicular elevations in the corneal epithelium associated with surrounding superficial punctate keratitis. Stopping the miconazole led to the resolution of the corneal epithelial changes.

Between April 1986 and April 1989, the authors conducted a prospective study of the use of open-loop anterior chamber intraocular lenses (AC IOLs) in patients undergoing penetrating keratoplasty for pseudophakic bullous keratopathy or monocular aphakic bullous keratopathy. All patients underwent the same operation--a penetrating keratoplasty combined with insertion of an open-loop AC IOL. Pseudophakic patients had an IOL exchange at the time of surgery. Thirty-six patients have been followed an average of 15 months. Thirty-two (89%) of the grafts are clear. Preoperatively, 100% of eyes had visual acuity less than 20/200. Postoperatively, 11 eyes (31%) have visual acuity better than 20/40 and 23 eyes (64%) have visual acuity better than 20/100. The most common causes for visual acuity less than 20/200 were cystoid macular edema, glaucoma, and immunologic graft failure. Using open-loop AC IOLs in patients with pseudophakic or aphakic bullous keratopathy can give good postoperative results and functional vision.

We treated a farmer who had Listeria monocytogenes bacterial keratitis. Therapy with topical antibiotics was unsuccessful; it was necessary to treat the patient with topical and systemic penicillin and gentamicin. To elucidate the pathogenesis of this infection, we developed a rabbit model. Using the patient's strain of L. monocytogenes, we determined that the severity of the rabbit infection was dose-related. If we used an inoculum of more than 10(7) organisms, many of the features of the human Listeria keratitis were mimicked. We also found that treatment with either penicillin or gentamicin did not control the infection as well as using both antibiotics simultaneously, a combination which resulted in relatively rapid resolution of infection and no corneal scarring. The human and animal data indicate that L. monocytogenes can be a virulent corneal pathogen. Listeria corneal infections must be treated aggressively with both penicillin and gentamicin to prevent permanent visual loss.

A 1-month-old infant had a protuberant congenital corneal dermoid that extended into the anterior chamber. The dermoid was excised in two stages. A 12-mm lamellar keratectomy was followed three months later by a smaller (8-mm) penetrating keratoplasty. This technique minimized the complications associated with large corneal transplants and increased the chance of long-term success. The graft has remained transparent and the posterior segment appears to be normal. The infant can maintain constant fixation and recognize small objects and crawls without difficulty.

A 26-year-old woman developed bilateral peripheral uveitis. This was followed five years later by the onset of ulcerative colitis. To our knowledge this is the first report of a relationship between these two entities. A brief review of the literature is undertaken along with a description of some of the immunologic phenomena common to these two disorders. Systemic manifestations with peripheral uveitis are rarely reported, and we feel that many case of uveitis in patients with ulcerative colitis may go undetected. Therefore the true incidence of uveitis in ulcerative colitis may be higher than is realized. It is emphasized that a complete and extensive ocular examination be made in all patient with ulcerative colitis or other forms of inflammatory bowel disease. In this way, the true relationship between these disorders may be elicited and the presence or absence of a common immunologic disturbance may be determined.

Twenty-one patients admitted between 1977 and 1982 to the Medical College of Virginia were studied retrospectively for work-related eye injuries. This group comprised 7.7% of all eye-injury admissions. Patients suffered both blunt and penetrating injuries; penetrating injuries had poorer prognoses. Twenty of the 21 patients had not worn protective eyewear. The average per-patient cost related to ocular injury was $2946, and the average hospital stay was 6.4 days. In contrast, the one patient who had worn protective eyewear had ocular-related costs and hospitalization of only $350 and a one-day stay. Protective eyewear is important in lessening the severity of injury as well as decreasing the financial and psychologic burden to the patient, hospital, and employer.

A 33-year-old homosexual patient with acquired immune deficiency syndrome (AIDS) developed sudden unilateral loss of vision. Slit-lamp and funduscopic examination of the affected eye was completely within normal limits. The patient, however, had a Marcus Gunn pupil, decreased color vision, and a large central scotoma on visual field examination. This was consistent with retrobulbar optic neuritis. Laboratory investigation revealed a highly positive serum and cerebrospinal fluid venereal disease reaction level (VDRL). A diagnosis of neurosyphilis was made, and the patient was treated with ten days of intravenous penicillin therapy. Ophthalmologists should be aware that many patients with AIDS have also been exposed to syphilis. Regardless of their clinical presentation, all AIDS patients should be examined and tested for syphilis and treated as is necessary.

Light and electron microscopic studies in three cases of recurrent granular dystrophy with corneal epithelial involvement showed intracellular rod and trapezoidal crystalline granules characteristic of granular dystrophy. Small intraepithelial cytoplasmic granules were rimmed by 15-nm particles, while larger deposits were often membrane-bound. The granules were closely associated with tonofilaments. There were ultrastructural similarities to keratohyaline. Extracellular granules were often intermixed with cytoplasmic organelles of disintegrated squamous epithelial cells. There was no evidence of a subepithelial pannus. These findings suggest an epithelial origin of the granular dystrophic deposits in these recurrent cases.

Cyanoacrylate adhesives are frequently used to seal small corneal perforations. Their use has been limited, however, because of concern over their toxic effect on ocular structures. We describe a post-keratoplasty patient with a wound dehiscence that a surgeon had attempted to seal with cyanoacrylate glue. The glue was accidentally injected into the anterior chamber. After surgical reconstruction of the eye, however, no permanent ocular damage was observed.

Five patients who had postoperative pseudophakic bacterial endophthalmitis were treated with topical, periocular, and systemic antibiotics. Four patients also received intravitreal antibiotics and oral corticosteroids. The causative organisms were Staphylococcus epidermidis, S. aureus, and Proteus mirabilis. Final visual acuities ranged from 6/7.5 (20/25) to no light perception. Response to treatment and final visual acuity did not appear to be related to retention or removal of the intraocular lens.

Seven adult patients (six men and one woman, ranging in age from 50 to 83 years) underwent intracapsular cataract surgery for dislocated traumatic cataracts. Six of the seven also had anterior vitrectomies. All seven patients had preoperative visual acuities of counting fingers or worse. There were no serious postoperative complications and five of the patients had postoperative visual acuities of 20/60 or better. Visual acuity improved less in the other two patients because of pre-existing but unrecognized macular disease.

We treated six patients who had recurrent episodes of nontuberculous phlyctenular keratoconjunctivitis and progressive corneal vascularization and scarring with oral tetracycline. This treatment resulted in rapid relief of symptoms and the apparent arrest of this disease. There have been no complications secondary to the use of tetracycline. To date, there has been only one recurrence in the patients treated with tetracycline which consisted of mild conjunctival hyperemia. We believe oral tetracycline is a safe and effective treatment for resistant and recurrent nontuberculous phlyctenular keratoconjunctivitis and an alternative to topical corticosteroid treatment in those patients suffering from corticosteroid-induced complications.

After undergoing uneventful cataract extractions, four patients developed an inflammatory reaction that included scleritis and peripheral corneal infiltrates and ulcers. Two of these patients also developed conjunctival ulcers. The inflammatory reaction in all four patients responded to topical corticosteroids.

We performed hemolytic assays for C1, C4, C3 and C5 on tear samples from 7 normal subjects and 10 patients with corneal ulcers. Hemolytic activities in tears were determined by 50% hemolysis of sensitized sheep red blood cells. Absent or low hemolytic activities of C1, C4 and C3 were found in normal tears. C5 was detected in tear samples from 4 of 7 normal subjects. Tear samples from patients with corneal ulcers showed elevated levels of C1, C4, C3 and C5, but wide ranges in values were found. C5 had the highest hemolytic activities in both normal tears and tears from eyes with corneal ulcers. Complement in tears may contribute to host defense in microbial corneal ulcers.

Proteins and mucosubstance of the saline extract of human ocular mucus were studied by immunological analysis. A minor study was made with human tears for comparison. Immunoelectrophoresis of proteins from these two sources consistently revealed similar characteristic gel patterns. Proteins were found as the major constituents of both samples. However, more mucosubstance was present in the saline extract of human ocular mucus than in tears. Seventeen proteins were identified in the mucus extract. Albumin, IgA, and lactoferrin appeared to be the three major proteins, while lysozyme, lactoferrin, tear prealbumin, and ocular mucoisolate were tear and ocular mucus specific. Although saline soluble mucoisolate is complex in structure, it seemed to resist electrical dissociation, producing only one major precipitation line along with a line of IgA during immunoelectrophoresis. The ocular mucoisolate accounted for about 12% of the saline extractable proteins of human ocular mucus.