Ocular characteristics in 10 children with long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiency: a cross‐sectional study with long‐term follow‐up

Purpose: To present long‐term ocular complications and electroretinographic (ERG) findings in children with long‐chain 3‐hydroxyacyl‐CoA dehydrogenase (LCHAD) deficiency – a life‐threatening metabolic disease – and the relation to age at diagnosis, treatment and other clinical parameters. Methods: Ten children with LCHAD deficiency underwent repeated ophthalmological evaluations including ERG. Results: All 10 children developed chorioretinal pathology. Regardless of age at diagnosis, initiation of treatment and age at examination, inter‐individual differences were present. Profound chorioretinal atrophy, severe visual impairment and progressive myopia had developed in two teenagers. Milder chorioretinopathy with or without subnormal visual acuity was present in all other children. ERG was pathological in seven children. The chorioretinopathy often started in the peripapillary or perimacular areas. In one patient, unilateral visual impairment was associated with fibrosis. Conclusion: Early diagnosis and adequate therapy might delay but not prevent the progression of retinal complications. Late diagnosis with severe symptoms at diagnosis, neonatal hypoglycaemia and frequent decompensations may increase the progression rate of the chorioretinopathy. LCHAD deficiency, a potentially lethal disease, is sometimes difficult to diagnose. Unusual chorioretinal findings should alert the ophthalmologist to the long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiency, especially if there is a history of neonatal hypoglycaemia or failure to thrive.     

Posterior capsular opacification in patients with pseudoexfoliation syndrome: a long‐term perspective

Purpose: To compare posterior capsular opacification in patients with and without pseudoexfoliation syndrome 6–7 years following surgery. Methods: Forty‐four eyes of 44 patients with pseudoexfoliation syndrome who underwent cataract surgery with phacoemulsification in 2001 and 2002 were available for follow‐up in 2008. These patients were compared to 86 age‐ and gender‐matched patients (86 eyes) without pseudoexfoliation syndrome who had surgery during the same time period. Posterior capsule opacification was assessed using digital retroillumination photography. The images were analysed with a software program (POCOman ) to determine the extent and severity of the capsular opacification. Results: Percentage and severity of posterior capsular opacification within the central 4.0 and 1.3 mm of the optical zone were compared in eyes with and without pseudoexfoliation syndrome. Neither of these results were statistically significant. Before the re‐examination in 2008, neodymium:yttrium‐aluminium‐garnet laser posterior capsulotomy had been conducted in 16% (n = 7) of eyes with pseudoexfoliation syndrome, as well as in 16% (n = 14) of eyes without pseudoexfoliation syndrome. Conclusion: Our study indicates that, with phacoemulsification, development of long‐term posterior capsular opacification is not increased in patients with pseudoexfoliation syndrome after uncomplicated cataract surgery.     

Efficacy of transconjunctival excision of orbital fat prolapse: a long‐term follow‐up study

Purpose: To describe the long‐term efficacy of transconjunctival excision of subconjunctival orbital fat prolapse. Methods: Retrospective study of consecutive cases of orbital fat prolapse treated with transconjunctival resection between December 2002 and December 2011. Results: Thirty‐two eyes of 23 patients (19 males and four females) were included. The lesion was unilateral in 14 and bilateral in nine cases. It was located superotemporally in a majority of cases. Excision was performed by opening the conjunctiva and excising the prolapsing orbital fat. The conjunctival wound was closed with 1–2 interrupted sutures. With an average follow‐up of 29 months (range 4–108), a recurrence was seen in three cases. The average time to recurrence was 46 months (range 40–52 months). Conclusion: Transconjunctival excision is a simple, safe and effective primary surgical procedure to treat subconjunctival fat prolapse. In our series, the recurrence rate was 9%, but no additional surgery was required. As the average time to recurrence was longer than our average follow‐up, more patients may develop a recurrence in future.     

Intracameral mydriatics in phacoemulsification cataract surgery – a 6‐year follow‐up

Purpose: To evaluate the long‐term safety of intracameral mydriatics (ICM) in phacoemulsification cataract surgery compared with conventional topical mydriatics (TM). Methods: A total of 45 patients were examined 6 years after phacoemulsification cataract surgery. The patients had previously participated in a prospective randomized double‐blind study including 60 patients, operated with either ICM or TM. The follow‐up included best‐corrected visual acuity (BCVA), intraocular pressure (IOP), grade of posterior capsule opacification (PCO), YAG laser capsulotomy rate, pupil size, corneal thickness and endothelial morphology. Results: No differences in postoperative BCVA, IOP, pupil size, PCO or YAG rate were observed between the groups. Endothelial cell loss, endothelial morphology and corneal thickness were also equivalent. Conclusions: Intracameral mydriatics is a safe alternative to topical mydriatics in phacoemulsification cataract surgery with no long‐term disadvantages at 6‐year follow‐up.     

Longterm follow‐up of children with traumatic optic nerve avulsion

Purpose: We report the longterm follow‐up of children with optic nerve avulsion (ONA) caused by traumatic events. The remarkable differences in courses and outcomes may elucidate the spectrum of ONA‐associated symptoms and injuries. Methods: During the last 15 years, three children with ONA were referred to our department. These cases are presented with special attention to their longterm follow‐up. Results: Two patients suffered from complete ONA after head injury. The third patient presented with partial ONA caused by a bicycle accident. Longterm follow‐up varied between 7 and 15 years. In the first patient, a pale swollen retina without any visible retinal vasculature was observed early in the course of follow‐up. The retina later completely detached. In the second patient, extended fibroglial scarring occurred and an extremely large epiretinal membrane formed and was finally released spontaneously into the vitreous. The third patient developed only mild fibroglial scarring and retinal pigment epithelium hyperplasia. The optic nerve head in this patient came to resemble a morning glory disc. Conclusions: Optic nerve avulsion can adopt different courses and outcomes in different patients. Final visual outcome seems to depend on the degree of visual acuity immediately after injury. Substantial intraocular architecture changes can occur as a result of ONA.     

Macular lesion resembling adult‐onset vitelliform macular dystrophy in Kearns–Sayre syndrome with multiple mtDNA deletions

We present the case of a 48‐year‐old woman with a clinically and histopathologically confirmed Kearns–Sayre syndrome who developed a maculopathy resembling an adult‐onset vitelliform macular dystrophy in her right eye. DNA analysis identified the presence of multiple deletions in the mtDNA of the muscle sample, with the common deletion of 4977 bp the most abundant. To the best of our knowledge, there have been no previous reports of such macular lesion occurring in association with Kearns–Sayre syndrome.     

Pseudoexfoliation and Alzheimer’s disease: a population‐based 30‐year follow‐up study

Purpose: To determine the effect of pseudoexfoliation (PEX) on the development of Alzheimer’s disease (AD) in a population in which PEX is a common finding. The relationship between open‐angle glaucoma (OAG) and AD was also studied. Methods: Predictors of incident AD, including mixed and unspecified dementia, were analysed in a cohort of 679 residents 65–74 years of age, examined in a population survey in the municipality of Tierp, Sweden, 1984–1986. To expand the cohort, participants in other studies in Tierp were enrolled. In this way, the cohort embraced 1123 people, representing more than 15 700 person‐years at risk. Medical records were reviewed to identify subjects diagnosed with dementia. Those with a follow‐up time shorter than 2 years were excluded from the study. Results: By the conclusion of the study, in December 2011, 174 new cases of AD, including mixed and unspecified dementia, had been detected, 41 of whom were affected by PEX at baseline. Higher age and female gender were the only predictors identified. No association between PEX and AD was found (hazard ratio 0.98; 95% confidence interval 0.69–1.40). Newly diagnosed OAG at baseline did not increase the risk (hazard ratio 1.09; 95% confidence interval 0.69–1.74). Conclusion: Pseudoexfoliation is not a predictor of AD. No association was found between OAG and AD.     

A 3‐year follow‐up study on the risk of stroke among patients with conjunctival haemorrhage

Purpose: To the best of our knowledge, no large population‐based studies on the relationship between conjunctival haemorrhage and stroke have been conducted to date. Using a nationwide population‐based data set, this study investigated the relationship between conjunctival haemorrhage and the subsequent risk of stroke within a 3‐year period following diagnosis. Methods: We analysed data sourced from the Taiwan Longitudinal Health Insurance Database 2000. The study cohort consisted of 17 349 patients with conjunctival haemorrhage and 86 745 comparison subjects. Each patient was individually tracked for a 3‐year period from their index date to identify all those who had subsequently received a diagnosis of stroke. Results: The incidence rate of stroke was 2.44 (95% CI = 2.31–2.55) per 100 person‐years in patients with conjunctival haemorrhage and 1.63 (95% CI = 1.59–1.68) per 100 person‐years in comparison patients. After adjusting for patients’ monthly income and geographic location, as well as for hypertension, atrial fibrillation, diabetes, hyperlipidaemia and coronary heart disease, stratified Cox proportional hazards regressions revealed a statistically significant hazard ratio for stroke in patients with conjunctival haemorrhage (HR = 1.33; 95% CI = 1.24–1.42, p < 0.001). Conclusions: In this study, patients with conjunctival haemorrhage were found to be at a significant risk of stroke during a 3‐year follow‐up period after diagnosis.     

Comparison of glistenings in intraocular lenses with three different materials: 12‐year follow‐up

Purpose: To compare the degree of lens glistenings associated with three intraocular lenses (IOLs) of different materials and examine the relationship between the dioptric power of the optics and lens glistenings in a long‐term study. Setting: St. Erik Eye Hospital, Stockholm, Sweden. Methods: Forty‐six eyes of 46 patients underwent standard phacoemulsification and implantation with a heparin‐surface‐modified (HSM) polymethylmethacrylate (PMMA) IOL, a silicone IOL or a hydrophobic acrylic IOL. Evaluations of the patients and the glistenings were conducted 11.3–13.4 years postoperatively. The glistenings were examined using Scheimpflug imaging and subsequently analysed using an image analysis program. Results: The median follow‐up time was 12.2 years (range, 11.3–13.4). The hydrophobic acrylic IOL had significantly more lens glistenings than the silicone (p = 0.003) and the PMMA (p = 0.000) IOLs. The silicone IOL had significantly more lens glistenings than the PMMA lens (p = 0.048). The IOL power did not affect the degree of lens glistenings in the hydrophobic acrylic IOL group (p = 0.64). The other groups had too little lens glistenings to evaluate the relationship. Conclusion: In this long‐term follow‐up study, the hydrophobic acrylic IOL had a significantly higher degree of lens glistenings compared to the silicone and PMMA IOLs. The PMMA IOL had almost no lens glistenings. The IOL dioptric power was not significantly correlated with the degree of lens glistenings associated with the hydrophobic acrylic IOL.