Harry Petrushkin

Tuberculous orbital apex syndromes

Aims: To describe the clinical and radiological features of orbital apex syndromes caused by presumed tuberculosis (TB). Methods: A review of the discharge summaries of all patients seen in the Medical Eye Unit (MEU), St Thomas’ Hospital between 1975 and 2006 identified seven patients with a diagnosis of orbital apex syndrome or optic neuropathy attributable to TB. Case notes and radiology were reviewed retrospectively for each patient. Results: All of the patients were born outside the UK and were HIV-negative. Four presented during 2005–6. Six of the seven patients presented with a visual acuity (VA) of less than counting fingers (CF), but all achieved a VA of 6/9 or better after a median 3 weeks of treatment with antituberculous treatment and systemic corticosteroids. All patients had constitutional symptoms. Chest x ray and CSF were normal in each case, but MRI was abnormal in six. Conclusions: We report a recent cluster of tuberculous orbital apex syndromes, in the context of an increasing incidence of TB in the UK. The condition is difficult to diagnose, but the combination of high-dose corticosteroids and anti-TB medication was rapidly effective and achieved a good outcome in all cases.

Intermediate uveitis and multiple sclerosis: to scan or not to scan

The investigation and management of inflammatory eye disease is complex and relies on detailed history taking and examination. It is not therefore unexpected that many patients are over-investigated. A question that has caused much debate in the past is whether patients with intermediate uveitis (IU), particularly young Caucasian women with no neurological symptoms, should have routine neuroimaging to ‘screen’ for multiple sclerosis (MS). In this editorial, we discuss the key issues related to the clinical presentation of IU, its association with MS and the rationale for investigation; in particular, we put forward our view that neuroimaging should not be carried out as a screening tool for patients with IU. IU is the term given to inflammation, which has the vitreous as its focal site. It can be caused by a number of infections including tuberculosis (TB), syphilis, Lyme disease, toxocariasis, Whipples disease and Epstein–Barr virus. IU is also associated with a number of systemic autoimmune diseases such as sarcoidosis, inflammatory bowel disease and MS.1 Many patients will not have any underlying disease process, and in this group the term idiopathic IU or pars planitis is applied. On examination, snow banking, vitreous snowballs, peripheral retinal vascular sheathing, vitreous cells and vitreous haze can be seen.1 It is important to clearly document the presence of these signs as well as the standardisation of uveitis nomenclature grading for vitreous inflammation.2 A number of other differential diagnoses must be considered in any patient presenting with posterior segment inflammation and it is important to exclude these before treatment is started and the clinical signs masked. The main diseases that need to be excluded are sarcoidosis, TB and masquerade syndromes such as primary intraocular lymphoma. In our practice, patients who present with IU and no relevant medical history or concurrent systemic symptoms will …

Clinical Review: Familial Mediterranean Fever-An Overview of Pathogenesis, Symptoms, Ocular Manifestations, and Treatment.

Abstract Familial Mediterranean fever is an autoinflammatory multisystem disease, which most commonly affects patients from the Mediterranean basin. This review discusses the common polymorphisms in the MEFV gene as well as the role of pyrin in disease pathogenesis. Patients with familial Mediterranean fever typically develop peritonitis, pleuritis, arthritis, and fever. In addition, a number of authors have reported ophthalmic features. These case reports and series are further explored in this review. Colchicine has transformed the prognosis for patients with familial Mediterranean fever. The rationale for the use of colchicine, as well as the evidence for newer biologic agents is also covered.

Behçet's disease: do natural killer cells play a significant role?

Behçet’s disease (BD) is a complex inflammatory disease, of unknown etiology. While disease pathogenesis remains unclear, a strong relationship between BD and HLA-B*51 has been established over the last 30 years. A number of theories exist regarding the cause of BD; however, few are able to account for the increased rates of HLA-B*51 positive individuals, particularly around the Mediterranean basin and Middle-East where the prevalence is highest. This review outlines current immunogenetic data on BD and the immunoregulatory role natural killer cells may play. It also describes the interaction of the killer immunoglobulin-like receptor – KIR3DL1 with its ligand Bw4, which is found on HLA-B51. Finally, CD94/NKG2D, MICA, and ERAP are outlined with regard to their potential roles in BD.

Optic disc assessment in the emergency department: a comparative study between the PanOptic and direct ophthalmoscopes.

Optic disc assessment is an essential part of the neurological examination of acutely unwell patients. This study compares the PanOptic ophthalmoscope with the direct ophthalmoscope for accuracy of diagnosis and ease of use. Patient satisfaction was also compared for the two instruments. A single-masked prospective observational study was carried out. The authors showed that the PanOptic ophthalmoscope was more sensitive (p=0.03) and specific (p=0.03) than the direct ophthalmoscope. The PanOptic ophthalmoscope was preferred by both doctors (p=0.001) and patients (p=0.04) in terms of comfort and ease of use.

Acute corneal hydrops in keratoconus: a national prospective study of incidence and management.

PurposeTo determine incidence and management of acute corneal hydrops in the UK.MethodsWe used the BOSU report card system to survey cases of acute corneal hydrops in patients with keratoconus that occurred in the UK between November 2009 and December 2010. Ophthalmologists who reported a case were sent an initial questionnaire, with a follow-up questionnaire after 6 months. We collected information on the demographics, complications, changes in visual acuity, and management. The 2011 National Census was used as a source for population and ethnicity in the UK.ResultsThere were 73 incident cases of acute corneal hydrops, with a response to the initial questionnaire for 64 (88%) patients and follow-up data at 6 months for 57 (78%) patients. For the 64 confirmed cases the median (interquartile range) age of onset was 31.9 (23.2, 41.3) years and 48 (75%) of the cases occurred in males. A total of 42 (66%) patients were white, 14 (22%) were South Asian, and 7 (11%) were black. The proportion of South Asian and black patients with acute corneal hydrops was significantly higher than in the general population (P<0.001). The minimum estimated annual incidence of acute corneal hydrops in patients with keratoconus was estimated to be 1.43 (1.10, 1.83) per 1000. At 6 months following acute corneal hydrops a decision to proceed with keratoplasty had been made for 12 (20.3%) patients.ConclusionsThis is the first population-based estimate of the incidence of acute corneal hydrops in keratoconus.

Gamma Delta (γδ) T Cells and Their Involvement in Behçet’s Disease

Behçets disease (BD) is a multisystem inflammatory disorder characterized by orogenital ulcerations, ocular manifestations, arthritis, and vasculitis. The disease follows a relapsing-remitting course and its pathogenesis is unknown. Genetic predisposition and immune-dysregulation involving gamma delta (γδ) T cells are reported to have a role. γδ T cells are atypical T cells, which represent a small proportion of total lymphocytes. They have features of both innate and adaptive immunity and express characteristics of conventional T cells, natural killer cells, and myeloid antigen presenting cells. These unconventional T cells are found in the inflammatory BD lesions and have been suggested to be responsible for inducing and/or maintaining the proinflammatory environment characteristic of the disease. Over the last 20 years there has been much interest in the role of γδ T cells in BD. We review the literature and discuss the roles that γδ T cells may play in BD pathogenesis.

Botulinum Toxin for the Treatment of Early-Onset Esotropia in Children With Cerebral Palsy

To the Editors: It is well established that children with cerebral palsy have an increased incidence of ocular misalignment with unpredictable surgical outcomes.1 We describe three children with periventricular leukomalacia who were treated in our unit with botulinum toxin for correction of their early-onset esotropia. The three children in our case series all presented between 4 and 10 months of age with a moderateto large-angle non-accomodative esotropia. Both surgical and non-surgical options were discussed with the childrens’ parents, and botulinum toxin was the preferred treatment option. All children underwent general anesthesia and had 0.1 mL of 2.5 units Botox (Allergan, Irvine, CA) injected into their medial recti, with a 90-second pause between the injection and withdrawal of the needle. The results appear in the table. All children achieved an improvement in their ocular alignment following the injections. One child remained stable after just one injection at 10 diopters (D) base out. One child required two injections before stabilizing at 2 D base out with gross stereopsis and the final child required three injections before stabilizing at 20 D base out with intermittent stereopsis. None of the children in our series developed ptosis or amblyopia as a result of the injection. The psychosocial effects of strabismus surgery are well recognized and children with strabismus often encounter significant negative social bias.2,3 However, there is a high incidence of unpredictable outcomes following strabismus surgery in this group. Previous studies have indicated overcorrection after using the standard surgical dosage of bimedial medial rectus recessions as a primary treatment for their esotropia or undercorrection with altered normograms.1 It is therefore difficult to adequately counsel parents with regard to how many operations may be needed and the degree of correction achieved. Multiple surgeries also cause scarring, discomfort, redness, and a mechanical restriction of eye movements. There have been several recent studies showing a good response to botulinum toxin in normal children with mild to moderate early-onset esotropia.4,5 Our results match these findings and we believe that botulinum toxin is an excellent alternative to surgery in children with cerebral palsy that should be considered as a viable option in the management of early-onset esotropia in children with periventricular leukomalacia.

Intralesional steroid for orbital manifestations of Rosai–Dorfman disease

This work was kindly funded by the Frost TFC Charitable Trust and the Royal College of Ophthalmologists Fellowship.

The Use of Topical Non-steroidal Anti-inflammatory Drugs for Uveitic Cystoid Macular Edema.

ABSTRACT The use of topical non-steroidal anti-inflammatory drugs in the management of uveitic cystoid macular edema is controversial. In this letter, we review the literature and report our results from a retrospective case series of 281 patients taking topical nepafenac 0.1% for uveitic cystoid macular edema between 2010 and 2016. Over a 4-month period, there was a modest improvement in best-corrected visual acuity (0.66 ± 3.7 logMAR) and central macular thickness (69.8 ± 132.5 μm). Patients with idiopathic anterior uveitis and HLA-B*27-associated anterior uveitis recovered more vision while taking topical nepafenac 0.1%, than those with other uveitides, however, this was not statistically significant. There is a need for controlled prospective studies to evaluate the efficacy of topical non-steroidal anti-inflammatory drugs in the management of uveitic cystoid macular edema. At present, clinicians have little evidence on which to base practice.