Noninfectious Corneal Disorders

This chapter discusses noninfectious corneal disorders, caused by an immune inflammation such as phlyctenular keratoconjunctivitis, marginal staphylococcal keratitis, peripheral ulcerative keratitis, Mooren's ulcer, interstitial keratitis, rosacea keratitis, etc. Mooren's ulcer, which is considered one of the most severe diseases, is presented with serial photos of follow-ups within 3 years with different complications, management of the condition, and results. A severe case of rosacea keratitis associated with anterior uveitis is included in this chapter too. Thygeson's superficial punctate keratitis case is shown as well, but the disease is very rare in Middle Eastern populations. The material in this chapter also includes filamentary, neurotrophic, and exposure keratitis. A rare disorder like anesthetic abuse keratopathy, caused by topical excess use of anesthetic drops, is presented with before and after treatment photos to illustrate that the condition, if treated appropriately, can leave minimal scarring.

A Rare Case of Atypical Cogan’s Syndrome Presenting as Encephalitis

Cogan’s syndrome (CS) is a rare autoimmune vasculitis of unknown aetiology characterised by non-syphilitic interstitial keratitis, audio-vestibular symptoms, sometimes systemic symptoms and multi-organ involvement. Atypical CS has other ocular features such as scleritis, episcelritis, retinitis and optic neuritis. Diagnosis of CS is purely clinical without a confirmatory test. Hereby, we report a case of atypical CS presenting with features of encephalitis who was treated successfully with intravenous pulse methylprednisolone with cyclophosphamide. It is important to consider Cogan’s syndrome in the differential diagnosis of encephalitis with ocular and vestibular symptoms in young patients, as high morbidity and mortality rates are effectively lowered by early immunosuppressive treatment.

Microsporidia-induced stromal keratitis: a new cause of presumed immune stromal (interstitial) keratitis

Background and objectiveTo describe the clinical features, diagnosis and management of immune stromal keratitis/interstitial keratitis (IK) associated with microsporidial epithelial keratitis.MethodsBetween October 2020 and January 2021, medical records of IK patients microbiologically proven as microsporidia from samples collected from corneal epithelium on smear examination, and/ or molecular analysis were reviewed. Demography, clinical profile and treatment were analysed. Real-time PCR (RT-PCR) for adenovirus (ADV), Epstein-Barr virus (EBV), herpes simplex virus (HSV) and varicella-zoster virus (VZV) was done.ResultsTwenty of 152 (13%) microbiologically proven cases of microsporidial keratitis were diagnosed as IK during the study period, the mean age and duration of symptoms were 35.7±11.4 years and 46.3±27.7 days, respectively. Half had predisposing risk factors, like trauma; and 30% had prior recurrences. One-fourth of patients were using antivirals on presentation. Characteristic presentations included disciform keratitis(n=12), incomplete/complete ring(n=5), and combination(n=3), along with variable subepithelial infiltrates (n=14). All cases had stromal oedema, with an intact epithelium and fine pigment dusting on endothelium. Corneal epithelial scrapings had scanty microsporidia spores in smears of 17/20 (85%), and pan-microsporidial DNA was identified in 14/20 (70%), with Vittaforma corneae by sequencing in 11/20 (55%). Other viruses detected were ADV (14,70%), VZV (2,10%), EBV (1,5%) and HSV (1,5%). Rapid resolution of inflammation and oedema within 2 weeks of starting steroids was seen in all cases.ConclusionMicrosporidia epithelial keratitis induced stromal inflammatory keratitis; is distinguished from microsporidial keratoconjunctivitis and stromal keratitis, by characteristic clinical features, and response to topical steroids.

Neurosyphilis Presenting with Optic Neuritis

The incidence of syphilis has greatly reduced in the severity of affected individuals due to the early treatment with antibiotics. However, due to the increase in the prevalence of human immunodeficiency virus (HIV) infection, it has also caused a concurrent rise in the number of neurosyphilis patients. Most common ocular manifestations include uveitis, interstitial keratitis, and vasculitis. A healthy 28-years old man presented with a progressive blurring of vision in the right eye for one month. It was his second episode. The first episode occurred 4 months back which resolved spontaneously without treatment. Vision in the right eye was 6/18. The pupil was dilated and the relative afferent pupillary defect was positive. Fundus examination showed a hyperemic swollen right optic disc with blurred margins and no macular fan or star. Vision, anterior segment, and fundus were normal in the left eye. After the investigations, he was diagnosed as a case of neurosyphilis with optic neuritis. He was treated with intravenous penicillin for two weeks followed by oral penicillin for three weeks. Vision in the right eye improved to 6/6; the optic disc swelling resolved with clear margins. He maintained good vision during the follow-up of six months. Early referral to an ophthalmologist upon suspicion of syphilis, detailed evaluation, and immediate treatment is mandatory to prevent permanent vision loss in these patients.

O30 Case report of Cogan syndrome in a child with audiovestibular dysfunction and ocular inflammation

Case report - Introduction Cogan syndrome is a very rare disorder of probable autoimmune vasculitic origin, first described in 1934. The age of onset seems to range from 3 to 50 years with an average age of disease onset at 29 years. It is characterised by audiovestibular dysfunction and ocular inflammation. The cause remains unknown and the epidemiology of the disease is purely based on case reporting. As of 2015, there were fewer than 250 case reports. Interestingly, it seems extremely rare in Arabic and Middle Eastern countries in which a new case has been recently diagnosed and is being reported in this abstract. Case report - Case description A 14-year-old girl of Arabic origin first presented to the ENT specialist with a 3-week history of balance problems, left tinnitus and sensorineural hearing loss in both ears worse on the left. She first received treatment for vestibular neuritis with no improvement. Two months later, she complained of bilateral red eyes and excessive lacrimation when diffuse interstitial keratitis was diagnosed by the ophthalmologist. An array of blood tests including a full autoimmune disease was arranged and a rheumatology opinion was sought. The patient had a normal inflammatory response and her screen for antinuclear antibodies, extractable nuclear antigen, rheumatoid factor and antineutrophil cytoplasmic antibodies were all negative. A full virology screen was also negative. Having failed to respond to all supportive treatment modalities, a trial of a tapering 2-week course of prednisolone starting at 25mg daily was instigated. The patient’s symptoms improved by more than 50% on glucocorticoids subsequent to which the diagnosis of Cogan syndrome was anticipated. As the patient’s symptoms relapsed when glucocorticoids were stopped, she was recommenced on prednisolone 25mg in line with azathioprine 100mg daily. The patient’s hearing, which was almost lost in the left ear, improved significantly by more than 70% initially, and then to normal hearing as stated by the patient supported by special ENT testing. Her balance problems and vertigo also improved remarkably. Her tinnitus, which was the last symptom to resolve, almost went away over the subsequent 2 months. The patient had already come off glucocorticoids 6 months down the line, and stayed on azathioprine 75-100mg daily for almost a year after which gradual weaning was trialled and then eventually succeeded. She has now been off azathioprine for 6 months without experiencing any disease relapse. Case report - Discussion Cogan syndrome can be difficult to diagnose particularly in children. The diagnosis is essentially clinical depending on the presence of audio-vestibular symptoms and interstitial keratitis along with a prompt response to immunosuppressive medication. In this patient, the consistent clinical picture supported by the positive response to glucocorticoids especially in the absence of an alternative diagnosis would have made the diagnosis of Cogan syndrome likely. Given the rarity of this disorder, there are no classification criteria as yet hence the ongoing need for case reporting. Moreover, there is no clear guidance on the duration of immunosuppressive treatment of this condition. Case report - Key learning points It is pivotal in managing conditions with a suspected autoimmune origin to have a multidisciplinary approach involving other relevant specialties in a holistic approach. The prompt response to glucocorticoid therapy still remains the key in securing the diagnosis of a number of conditions in clinical practice especially in the absence of diagnostic criteria and/ or supportive investigations such as serological and tissue-based testing. Case reporting has a crucial role in enhancing awareness of rare medical conditions.