Susac syndrome: a case report with initial presentation of incomplete bitemporal hemianopia.

Susac syndrome (SS) is a rare microangiopathy affecting the precapillary arterioles of the brain, inner ear, and retina. We present a novel case of SS, presenting as acute incomplete bitemporal field loss in addition to temporally spaced neurological and vestibulocochlear symptoms. A 39-year-old female was referred to the ophthalmology clinic with acute incomplete bitemporal hemianopia and worsening hemicrania. History revealed progressive hearing loss, subjective short-term memory impairment, and vertigo temporally spaced over the preceding 12 months. Magnetic resonance brain revealed multiple small colosal lesions and liner 'spoke' lesions. Fundus fluorescein angiography revealed multiple branch retinal artery occlusions in the right eye. Audiometry confirmed bilateral sensorineural hearing loss. Treatment included intravenous corticosteroids and rituximab. This case highlights the importance of early consideration and evaluation of SS in individuals presenting with atypical ocular disturbances, where no clear cause can be elicited, in order to limit the sequelae of disease.

Outcomes of hyperbaric oxygen treatment for central and branch retinal artery occlusion at a major Australian referral hospital.

Introduction: This study analysed the treatment outcomes of patients that received hyperbaric oxygen treatment (HBOT) for retinal artery occlusion (RAO) at the Royal Brisbane and Women's Hospital in Brisbane, Australia between 2015 and 2021. Methods: Retrospective study from patient records including 22 eyes from 22 patients that received HBOT for either central RAO (17 patients) or branch RAO (five patients). Patients received the Royal Brisbane and Women's Hospital RAO protocol for their HBOT. Analysis included best corrected visual acuity pre- and post-treatment, subjective improvements, side effects and patient risk factors were also recorded. Results: Improvement in best corrected visual acuity was LogMAR -0.2 for central RAO on average with 8/17 (47%) experiencing objective improvement, 5/17 (29%) experienced no change and 4/22 (24%) experienced a reduction in best corrected visual acuity. Subjective improvement (colour perception or visual fields) was reported in an additional 4/17 patients, resulting in 12/17 (71%) reporting improvement either in visual acuity or subjectively. There was no improvement in the best corrected visual acuity of any of the five patients suffering from branch RAO. Cardiovascular risk factors present in the cohort included hypertension, hypercholesterolaemia, previous cardiovascular events, cardiac disease and smoking. Limited side effects were experienced by this patient cohort with no recorded irreversible side effects. Conclusions: Hyperbaric oxygen treatment appears a safe, beneficial treatment for central RAO. No benefit was demonstrated in branch RAO although numbers were small. Increased awareness of HBOT for RAO resulting in streamlined referrals and transfers and greater uptake of this intervention may further improve patient outcomes.

Recurrent post-operative endophthalmitis caused by Sphingomonas paucimobilis despite vitrectomy - a case and review of the literature.

Over the past 20 years, scattered reports have emerged about a low virulence, gram negative bacillus, Sphingomonas paucimobilis, causing unpredictable clinical presentations of endophthalmitis. Previous reports have characterised the organism as being resistant to aggressive treatment and prone to recurrence up to several months later, with few warning signs of any residual infection. We report a case of a 75 year-old male who returned 10 days after a left eye cataract surgery with an atypical, indolent endophthalmitis. He was treated with broad-spectrum intravitreal antibiotics and vitrectomy, and despite initial improvement, the patient suffered a recurrence after 2 weeks, necessitating successive rounds of intravitreal antibiotics. While our patient was able to achieve an excellent final visual acuity of 6/9, there are several cases in the literature reporting similar cases with much worse visual outcomes. Further research is required to elucidate early warning signs that may indicate a recurrence of S. paucimobilis infection, and the underlying mechanism by which it is resistant to standard endophthalmitis therapy. Alongside this case, we review and summarise the literature on postoperative endophthalmitis involving this organism.

Trampoline Use and Retinal Detachment in Stickler Syndrome.

Stickler syndrome is one of the most common inherited causes of retinal detachment in childhood. We present the case of a 6-year-old boy with Stickler syndrome who developed a retinal detachment in his better seeing eye after prolonged trampoline use. We suggest that trampolining should be avoided in all patients at increased risk of retinal detachment, especially in Stickler syndrome, and in those with other risk factors including high myopia and previous retinal detachments.

360-Degree laser retinopexy in primary vitrectomy for rhegmatogenous retinal detachment: factors associated with its use and impact on surgical outcomes.

BACKGROUND: To determine patient and surgical factors associated with the use of 360-degree laser retinopexy during primary pars plana vitrectomy (PPV) ± scleral buckle (SB) for rhegmatogenous retinal detachment (RRD) and its impact on surgical outcomes. METHODS: Patients who underwent PPV ± SB for repair of non-complex RRD at a single centre were included in this retrospective study. The primary outcome was single surgery anatomical success (SSAS). Secondary outcomes included visual acuity, epiretinal membrane formation, the presence of cystoid macular oedema, tonic pupil and corneal epithelial defects. Multiple logistic regression and multivariate regression was used. RESULTS: The study included 192 cases, of which 130 received 360-degree laser. Worse preoperative logMAR visual acuity (P = 0.009), male sex (P = 0.060), higher PVR grades, supplemental SB (P = 0.0468) and silicone oil/C3F8 tamponade (P < 0.0001) were associated with 360-degree laser use. No significant associations between 360-degree laser and SSAS (P = 0.079), final logMAR visual acuity (P = 0.0623), ERM development (P = 0.8208), postoperative CMO (P = 0.5946), tonic pupil (P > 0.9999) or corneal epithelial defects (P = N/A) were found. CONCLUSIONS: 360-degree laser retinopexy during primary PPV ± SB for RRD was associated with more complex cases and more extensive operations. Even when accounting for this, there was no difference in surgical outcomes or complication rates.

Extrafacial Granuloma Faciale: A Case Report and Brief Review.

Granuloma faciale (GF) is a rare, inflammatory, cutaneous disorder of unknown aetiology. It presents clinically as one or several well-circumscribed violaceous papules, plaques, and nodules almost exclusively confined to the facial region. Rarely, extrafacial lesions can occur, most often on sun-exposed sites. We report a case of extrafacial GF in a 63-year-old male with indolent lymphoma, who presented with plaques involving the right preauricular region and left posterior axilla. The clinical and histopathological findings were consistent with GF. Our case highlights the importance of performing skin biopsies in patients with persistent erythematous plaques and nodules, particularly to exclude important malignant and granulomatous differential diagnoses.

Ultrawide-field fundus photography of the first reported case of gyrate atrophy from Australia.

Gyrate atrophy of the choroid and retina is a rare chorioretinal dystrophy inherited in an autosomal recessive pattern. We describe the first documented case of gyrate atrophy from Australia in a 56-year-old woman with a history of previous diagnosis of retinitis pigmentosa and worsening night vision in her right eye over several years. She was myopic and bilaterally pseudophakic, and fundus examination revealed pale optic discs and extensive peripheral chorioretinal atrophy exposing bare sclera bilaterally with only small islands of normal-appearing retina at each posterior pole. Visual field testing showed grossly constricted fields, blood testing showed hyperornithinemia, and further questioning revealed consanguinity between the patient's parents. We then used the patient's typical retinal findings of gyrate atrophy to demonstrate the potential use of ultrawide-field fundus photography and angiography in diagnosis and monitoring response in future treatment.

Microbiological isolates and antibiotic sensitivities in culture-proven endophthalmitis: a 15-year review.

BACKGROUND: To describe the microbiological spectrum and antibiotic sensitivities of organisms causing culture-proven endophthalmitis in Queensland, Australia, and to compare results with similar studies from other parts of Australia and other countries. METHODS: A retrospective, multicentre, non-comparative, consecutive case series. Public hospital microbiology records from culture-positive endophthalmitis cases were reviewed over 15 years from June 1998 to June 2013. Outcome measures were type of endophthalmitis, vitreous isolates cultured and antibiotic sensitivities. RESULTS: 205 cases of culture-proven endophthalmitis were identified with a total of 229 isolates cultured. The most common organisms isolated were Staphylococcus epidermidis in 23.1%, Coagulase-negative Staphylococcus in 12.7%, Streptococcus viridans group in 10.0%, Candida species in 6.1%, fungal mold species in 5.7%. For gram-positive organisms, sensitivities were vancomycin 100%, cephazolin 79% and penicillin 47%. For gram-negative organisms, sensitivities were ceftazidime 100%, amikacin 100%, ciprofloxacin 100% and gentamicin 95.5%. For fungal isolates, sensitivities were voriconazole 93%, ketoconazole 89%, caspofungin 70% and amphotericin B 58%. CONCLUSIONS: The microbiological spectrum and antibiotic sensitivities of endophthalmitis cases in Queensland, Australia, is similar to the spectrum of organisms causing endophthalmitis in other parts of Australia, North America and Europe. Empirical intravitreal vancomycin, ceftazidime and voriconazole are the most appropriate empirical antibiotics for suspected infective endophthalmitis.

Pseudallescheria endophthalmitis: four cases over 15 years in Queensland, Australia, and a review of the literature.

PURPOSE: To describe the clinical features and outcomes of patients with culture-proven Pseudallescheria endophthalmitis in the context of previously published cases. METHODS: A multicenter, retrospective, consecutive case series was undertaken. Pathology records were reviewed for all patients with Pseudallescheria endophthalmitis on vitreous/aqueous cultures from June 1998 to June 2013 throughout Queensland, Australia. RESULTS: Three females and 1 male aged 60 to 78 years were identified. All patients had endogenous endophthalmitis, had an underlying malignancy, and were from South East Queensland. Vitreous cultures identified Scedosporium auranticum in two cases and Scedosporium prolificans and Scedosporium apiospermum in one case each. All isolates were sensitive to voriconazole. Enucleation was required in two patients. Two patients had final best-corrected visual acuities of perception of light and no perception of light, respectively. CONCLUSION: Endophthalmitis caused by species of the P. boydii complex is a devastating condition with poor visual outcomes. It is a condition that is difficult to treat because of its variable antifungal agent resistance.

Exudative vasculopathy in a child with Leber congenital amaurosis.

Leber congenital amaurosis is a severe retinal dystrophy that causes blindness or severe visual impairment, usually before the age of 1 year. We present the case of a 13-year-old girl with Leber congenital amaurosis who developed an exudative vasculopathy. She was successfully treated with cryotherapy and argon green laser. To our knowledge, only 4 cases of this condition in patients with Leber congenital amaurosis have been reported previously. This phenotype may be related to c.2991+1655A>G (p.Cys998X) mutations in the CEP290 gene.

Toxic optic neuropathy in the setting of docetaxel chemotherapy: a case report.

BACKGROUND: To describe the first reported case of toxic optic neuropathy secondary to docetaxel (Taxotere®) chemotherapy. CASE PRESENTATION: A 53-year-old female presented with predominantly unilateral visual loss, but extensive bilateral visual field defects and bilateral optic nerve head swelling 2 weeks after first dose of docetaxel (Taxotere®) and trastuzumab (Herceptin®) chemotherapy for a left sided node-positive, HER2 positive breast cancer. Extensive investigation ruled out other causes of optic neuropathy. She was treated with high dose corticosteroids intravenously for 1 week then a tapering oral dose over 8 weeks. Visual field defects gradually resolved and visual acuity improved. Docetaxel chemotherapy was discontinued but targeted therapy with trastuzumab continued without further complication. CONCLUSION: Docetaxel can cause a toxic optic neuropathy possibly due to an ischemic or neurotoxic mechanism at the optic nerve head. With cessation of docetaxel therapy and treatment with systemic corticosteroids, visual recovery can occur without significant residual visual deficit.

A case of conjunctival mucoepidermoid carcinoma in Australia.

Conjunctival mucoepidermoid carcinoma is a very rare but highly aggressive conjunctival neoplasm with 42 previously reported cases. We report the case of a 56-year-old male with a left ocular surface squamous neoplasm, which was subsequently treated with excision and autoconjunctival graft. Histopathology of the operative specimen reported a low-grade conjunctival mucoepidermoid carcinoma, and the patient was then treated with an adjunctive course of mitomycin C. On review 10 months after lesion excision, there was no recurrence, and the patient was otherwise well. Due to its rare incidence, difficult clinical diagnosis, and accompanying poor prognosis, conjunctival mucoepidermoid carcinoma should always be considered in the differential diagnosis of conjunctival neoplasms, and full histopathologic examination, including mucin-staining techniques, of all suspicious conjunctival biopsies should occur.

Candida dubliniensis endophthalmitis: five cases over 15 years.

BACKGROUND: Recent studies have shown that the recently identified organism Candida dubliniensis is less pathogenic than the more common Candida albicans. Due to its rare nature, C. dubliniensis has been previously reported as the causative organism in endophthalmitis in only three cases. We undertook a multicenter, retrospective, consecutive case series to describe the clinical features and outcomes of patients with culture-proven C. dubliniensis endophthalmitis. Medical records were reviewed for all patients with C. dubliniensis endophthalmitis on vitreous/aqueous cultures from June 1998 to June 2013 from all public hospitals throughout Queensland, Australia. RESULTS: Six eyes from five patients were identified - four males and one female aged from 21 to 55 years (mean 37 years). Four patients were intravenous drug users and four patients had hepatitis C. All five patients were treated with systemic antifungal therapy and intravitreal antifungal injections, and all required vitrectomy. Two eyes developed retinal detachment over the course of the endophthalmitis. Five eyes had visual outcomes of 20/60 or better, and one eye had a poor outcome with final visual acuity of hand movements only. There was no associated mortality, and no infected eyes required enucleation or evisceration. CONCLUSIONS: C. dubliniensis endophthalmitis is a rare condition which occurs mainly in intravenous drug users and can occur in both HIV-positive and HIV-negative patients. Unlike C. albicans endophthalmitis, C. dubliniensis endophthalmitis has reasonable visual outcomes and does not appear to be associated with high mortality.