Three-year outcomes of alcohol delamination of corneal epithelium for recurrent corneal erosions of traumatic etiology.

PURPOSE: Alcohol delamination of the corneal epithelium (ADCE) is a therapeutic option for patients with recurrent corneal erosion (RCE) who do not respond to nonsurgical management of lubricants and extended wear contact lens. The aim of the study is to report on three-year efficacy and safety of ADCE for RCE of traumatic etiology. METHODS: This is a retrospective review of consecutive patients who underwent ADCE for traumatic RCE in a local hospital between January 2010 and January 2020. The outcomes at a 3-year follow-up review are included. Nonsurgical therapy used; intra- and postoperative complications were recorded. ADCE was only offered to those who remained symptomatic despite maximum topical lubrications and/or extended wear contact lens. Success was defined as the absence of recurrence of corneal erosion. RESULTS: Twenty-six eyes of 26 patients with RCE caused by trauma underwent ADCE. The mean age of patients was 39 years. The follow-up period was a minimum of 36 months. Three eyes (11.5%) had recurrence of corneal erosion after ADCE at the 3-year follow-up. Recurrence was noted at months 2, 23, and 36 postoperatively in these patients. All patients reported significant improvement in symptoms associated with recurrent erosion. Ten eyes (38.5%) stopped all topical lubricants postoperatively. No intra- or postoperative complications were noted in our study. CONCLUSION: This study documents the long-term safety and efficacy of alcohol delamination of corneal epithelium at 3 years for patients with RCE of traumatic origin.

Retinal features in Mulvihill-Smith syndrome.

BACKGROUND: Mulvihill-Smith syndrome is a rare progeroid syndrome of sporadic nature. Previously reported ophthalmological findings include astigmatism, myopia, endothelial dystrophy, keratoconus, cataract, band keratopathy, meibomian gland dysfunction, dry eye disease, amblyopia, and allergic conjunctivitis. MATERIALS AND METHODS: The proband, a 25-year-old male subject diagnosed with Mulvihill-Smith syndrome in childhood developed retinal changes with onset of adulthood. The retinal changes were monitored for progression with fundus photography, electrodiagnostic tests, and spectral domain optical coherence tomography. RESULTS: The fundus examination revealed grossly normal looking retina with dull foveal reflex. The optical coherence tomography scan of the retina revealed diffuse thickening, schisis, and folding of retinal layers in both eyes. The structural changes in retina were progressive with wrinkling of inner retinal layers and loss of foveal contour as observed over 3 years. The electrodiagnostic tests revealed normal photoreceptor-retinal pigment epithelial interface. CONCLUSIONS: This is the first report of retinal features in Mulvihill-Smith syndrome. These ocular changes coincided with other systemic changes with the onset of adulthood. These changes may indicate the natural history of retinal features in this progeria syndrome with short life span. The detailed analysis and progression of structural changes in retina is possible with optical coherence tomography.

Validity of ophthalmology surgical competency assessment rubric for strabismus surgery in resident training.

The Accreditation Council for Graduate Medical Education (ACGME) requires US residency programs to assess ophthalmology residents for competency in 6 core areas. Ophthalmic surgical skills are currently part of the ACGME "Patient Care" competency, although some have advocated for a seventh competency, "Surgical Skills." The Ophthalmology Surgical Competency Assessment Rubric for Strabismus Surgery in Resident Training ( OSCAR: Strabismus) tool was designed to aid in the assessment of surgical skills using procedure specific behavioral anchors. The present study evaluated inter-rater agreement of the OSCAR: Strabismus tool in the assessment of resident performance. OSCAR: Strabismus evaluations of resident surgical strabismus cases were performed by a multinational group of faculty strabismus surgeons. Cronbach α statistical analysis of the completed evaluations revealed high inter-rater agreement, indicating the OSCAR: Strabismus is a reliable tool to facilitate assessment of resident strabismus surgical skills.

Concentric macular rings sign in patients with foveal hypoplasia.

IMPORTANCE: We describe a sign that can be used as a rapid and noninvasive adjunct to aid in the diagnosis of foveal hypoplasia. OBJECTIVE: To describe a concentric macular rings sign found on infrared reflectance (IRR) images in patients with foveal hypoplasia. DESIGN, SETTING, AND PATIENTS: We studied 13 patients with foveal hypoplasia (7 with ocular albinism [OA], 5 with oculocutaneous albinism [OCA], and 1 with aniridia) at a tertiary ophthalmology center with access to electrodiagnostic services from February 18, 2009, through April 9, 2013. MAIN OUTCOMES AND MEASURES: All patients and an age-matched control participant underwent a complete clinical examination, electroretinography (full field and pattern), visual evoked potentials, fundus autofluorescence IRR, and optical coherence tomography (OCT). One patient with OA and the control participant also underwent scanning laser polarimetry with variable corneal compensation (GDx VCC). RESULTS: Thirteen patients (6 girls and 7 boys), with a mean age of 5.8 years (range, 3-11 years), were included in the study. Seven patients were diagnosed as having OA and had minimal clinical signs (fine nystagmus in 2 patients and subtle iris transillumination in 5 patients). Five patients with OCA and 1 with aniridia were also included. In 12 patients, OA and OCA were confirmed with 5-channel visual evoked potentials (optic nerve misrouting). Whenever OCT was performed, foveal hypoplasia was indicated by the lack of foveal dip. The macula lacked the foveal attenuation normally seen with fundus autofluorescence, and a concentric macular rings reflex was seen with IRR in all 13 patients and with GDx VCC in 1 patient. A normal bowtie reflex was seen with IRR and GDx VCC in the age-matched control participant. CONCLUSIONS AND RELEVANCE: Our findings suggest that concentric macular rings seen on IRR or GDx VCC can occur in patients with foveal hypoplasia and can therefore aid in the diagnosis, especially in patients with minimal clinical signs (mild OA) or in cases in which OCT cannot be performed (young patients or patients with high-amplitude nystagmus).

Retained Descemet's membrane following penetrating keratoplasty for Fuchs' endothelial dystrophy: a case report of a post-operative complication.

PURPOSE: To report a case of retained Descemet's membrane following penetrating keratoplasty in a patient suffering from Fuchs' endothelial corneal dystrophy. The use of confocal microscopy, histopathological tissue analysis, and treatment options are discussed. METHODS: Case report of an 85-year-old man with a past ophthalmic history of atrophic macular degeneration, underwent a penetrating keratoplasty for Fuchs' endothelial corneal dystrophy. Postoperative review revealed a retained retrocorneal membrane within the anterior chamber. Further surgery was performed to excise the membrane, with a subjective and objective postoperative improvement in visual acuity and without subsequent complications of the corneal graft. RESULTS: Histopathological assessment confirmed the clinical suspicion of a retained Descemet's membrane, marrying with the initial histology from the corneal button excised during the penetrating keratoplasty, which showed only a very thin Descemet's layer. CONCLUSION: Retention of the Descemet's membrane following penetrating keratoplasty is a rare but potential complication of this surgery, particularly in cases of Fuchs' endothelial corneal dystrophy due to the thickened and abnormal histological nature of the endothelium and high index of suspicion is required.

The short- and long-term effects on the visual system of children following exposure to maternal substance misuse in pregnancy.

PURPOSE: To document the prevalence of ophthalmic morbidities in babies born to mothers who misused substances in pregnancy and to assess whether it changes over 5 years. DESIGN: Retrospective comparative case series. METHODS: The series included: (1) a retrospective review of 301 children born between 2000 and 2004 to mothers misusing substances during pregnancy; (2) assessment at 5-year follow-up of this cohort; and (3) comparison with 7887 age-matched controls drawn from the preschool screening cohort in the north of Scotland in the same time period. Odds ratios (OR) and 95% confidence intervals (CI) were calculated. Following data collection in both patient and control groups, the following were calculated: (1) rate of referral to the ophthalmology department; (2) prevalence of nystagmus and strabismus at presentation in the study group and at 5 years of age in both patient and control group; (3) prevalence of reduced visual acuity and lack of stereopsis. RESULTS: Ophthalmology referrals, strabismus, and nystagmus were found to be statistically significantly higher in the study group compared with the control group. In the study group, at baseline referral, 46 of 301 (15.3%) had strabismus (2.8% in control group) and 11 of 301 (3.7%) nystagmus (0.004% in control group). At 5-year follow-up, the prevalence of strabismus was 14.0% (OR 5.70, 95% CI: 4.01-8.12) and that of nystagmus was 3.3% (OR 90.34, 95% CI 24.73-330.02). A total of 42.4% of these children at age 5 had no demonstrable binocular vision and 28.2% had visual acuity of 0.3 (logMAR) or worse in 1 or both eyes. Clinic attendance at last follow-up was poor (61.9%). CONCLUSIONS: Exposure to maternal drug misuse in utero is associated with a statistically significantly higher prevalence of strabismus and nystagmus. These abnormalities tend to persist at 5 years of age and are associated with long-term visual morbidity, such as lack of binocularity and poor visual acuity. Our study highlights the need for ophthalmologic surveillance of this population and supports attendance, as nonattendance may be linked to more problems with substance misuse in parents and greater pathology in the child.

Unilateral retinal hemorrhages with subarachnoid hemorrhage in a 5-week-old infant: is this nonaccidental injury?

PURPOSE: Retinal hemorrhages are an important sign in the setting of nonaccidental injury (NAI) (abusive head injury) in young infants and form a very important part of the evidence in support of the diagnosis. The diagnosis of NAI has serious social and legal implications. Other causes of retinal hemorrhages in an infant, such as birth trauma, accidental head injury, subarachnoid hemorrhage, other less common disorders of clotting, leukemia, and infections such as endocarditis, need to be considered and ruled out in making a diagnosis of NAI. METHODS: Descriptive case report. RESULTS: A 5-week-old child presented with rapid onset of symptoms of drowsiness and hypotonia, unilateral retinal hemorrhages, and an intracranial hemorrhage in the posterior fossa. NAI was high on the list of differential diagnosis, which caused considerable anxiety in the parents. The cause of the intracranial hemorrhage only became apparent at repeat neuroimaging several weeks later. CONCLUSIONS: The case is presented to point out arteriovenous malformation as a possible cause of retinal hemorrhages in this age group where an early diagnosis of the etiology is often not possible. A diagnosis of NAI, commonly associated with a similar clinical presentation, can have serious social and legal implications.

Mycophenolate mofetil embryopathy may be dose and timing dependent.

Mycophenolate mofetil (MMF) is an immunosuppressive agent that has now been recognized as teratogenic in humans. A pattern of malformations from in utero exposure to MMF has recently been described, and includes cleft lip and palate, microtia and atresia of the external auditory canal. We present a nulliparous mother who had taken MMF for recurrent erythema multiforme for the first 5 weeks of her pregnancy, and developed a spontaneous miscarriage during the seventh week of pregnancy. For her second pregnancy, she took MMF on her own accord for four days in the seventh week after her last menstrual period. The newborn had bilateral microtia, absence of the external auditory canals, and right iris and chorioretinal coloboma, consistent with the pattern recognized as part of the MMF embryopathy phenotype. As the newborn was not exposed to other immunosuppressive agents in utero, we believe that the phenotype described to be the result of the teratogenic effect of MMF. The spontaneous miscarriage in the first pregnancy may be due to the higher dose and longer duration of MMF exposure. The second pregnancy, with MMF exposure of 4 days, proceeded to term with the resultant phenotype. We conclude that the effect and severity of the embryopathy may be dependent on the dose, timing, and duration of MMF exposure. The manufacturer and the United States Food and Drug Administration have now disseminated information regarding the teratogenic risk of MMF. Women should be fully counseled and advised about contraception during the course of treatment with MMF.

Is this really a capillary haemangioma?

Periocular tumours in newborn babies are not uncommon and their diagnosis and management can be challenging. Capillary haemangioma is the most common of them and superficial ones are easy to recognise. Deep-seated (orbital) lesions can mimic various other orbital mass lesions - dermoid cysts, cellulitis, lymphangioma and the more serious rhabdomyosarcoma and neuroblastoma. A careful elicitation of history, physical examination, and appropriate orbital imaging (ultrasound for superficial ones and magnetic resonance imaging/angiography for deep-seated lesions) helps in the diagnosis. This is a brief report of a very large vascular lesion involving the lower lid with very atypical clinical features. The approach to diagnosis and successful treatment and the histological features are discussed.

Myectomy versus anterior transposition for inferior oblique overaction.

BACKGROUND: Inferior oblique overaction can be either secondary (as a sequela of ipsilateral superior oblique palsy) or primary (commonly associated with horizontal strabismus). Superior oblique underaction often coexists with both primary and secondary inferior oblique overaction. This retrospective case series compares the efficacy of inferior oblique myectomy versus anterior transposition in improving inferior oblique overaction and superior oblique underaction in eyes with either primary or secondary inferior oblique overaction. METHODS: One hundred twenty eyes of 81 patients were included in this retrospective case series, of which 20 had anterior transposition of the inferior oblique and 100 eyes underwent myectomy. Inferior oblique myectomy was compared with inferior oblique anterior transposition in improving inferior oblique overaction and superior oblique underaction in each diagnostic subgroup. Postoperative outcome was qualitatively and quantitatively assessed. Fisher's exact test was used to compare the outcomes. The quantitative improvement of function in terms of inferior oblique overaction and superior oblique underaction was analyzed by regression analysis. RESULTS: When postoperative inferior oblique overaction was considered, there was no statistically significant difference between myectomy and anterior transposition in both primary and secondary inferior oblique overaction. Myectomy was superior to anterior transposition in improving superior oblique underaction in both primary inferior oblique overaction (OR = 0.14; 95% CI, 0.015-1.45; p = 0.056) and secondary inferior oblique overaction (OR = 0; 95% CI, 0-0.027; p < 0.001). The quantitative improvement of function showed a significant difference between procedures for superior oblique underaction (t-test; p = 0.005; 95% CI, 0.25-1.3) but not inferior oblique overaction (t-test; p = 0.8; 95% CI, -0.67-0.54). CONCLUSIONS: This study demonstrates both inferior oblique myectomy and inferior oblique anterior transposition to be effective in correcting primary and secondary inferior oblique overaction. Myectomy is more effective in improving superior oblique underaction associated with both primary and secondary inferior oblique overaction. On this basis, we feel that inferior oblique myectomy has some advantage over anterior transposition in treating combined inferior oblique overaction and superior oblique underaction and can be considered the procedure of choice.

Comparative estimation of anterior chamber depth by ultrasonography, Orbscan II, and IOLMaster.

PURPOSE: To assess the degree of agreement of anterior chamber depth (ACD) measurements by 2 optical devices (Orbscan II and IOLMaster) and compare them with contact ultrasound A-scan. SETTING: Department of Ophthalmology at Hull Royal Infirmary, England. methods: In this prospective study of 81 eyes of 41 consecutive patients, ACD estimation was done by 3 methods-scanning slit topography (Orbscan II), partial coherence interferometry (IOLMaster), and contact ultrasound A-scan in that order. The same observer performed all measurements. Repeated measures Analysis of Variance (ANOVA) was used to analyze difference between ACD measurements by the 3 methods. Difference in measurements between methods was assessed using the paired t test. RESULTS: The mean ACD (+/-SD) by the 3 methods was 3.32 mm (0.60), 3.33 mm (0.61), and 2.87 mm (0.55), respectively. There was a statistically significant difference between measurements recorded by the 3 methods (P<.01). Mean contact A-scan measurements were 0.40 mm and 0.43 mm lower than by Orbscan II and IOLMaster respectively (P<.01). CONCLUSION: Applanation ultrasound gives consistently lower measurements for ACD compared to Orbscan II and IOLMaster. Although a high degree of agreement between Orbscan II and IOLMaster was noted, further studies are needed to assess the interchangeability of measurements in clinical practice.