Spontaneous hyphema from iris microhemangioma in Eisenmenger syndrome.

Purpose: We describe a patient with Eisenmenger syndrome and spontaneous hyphema from iris microhemangioma, two rare entities with a plausible pathophysiological connection. Observations: A 56-year-old Caucasian female with a background of cyanotic congenital heart disease complicated by Eisenmenger syndrome presented with non-traumatic hyphema and blurred vision. Multiple vascular tufts consistent with iris microhemangiomas were observed around the pupil margins bilaterally, with no iris or retinal neovascularization. In the affected eye, there was active bleeding from one lesion at 12 o'clock generating a macrohyphema. Additional findings included prominent episcleral injection and retinal venous tortuosity in both eyes. The active microhemorrhage and hyphema resolved with local medical management. Conclusions and importance: Chronic hypoxemia and erythrocytosis are known to induce dilation of the retinal and episcleral blood vessels in Eisenmenger syndrome. Corresponding dilation of iris stromal vessels may contribute to the formation and prominence of iris microhemangiomas.

Epidemiology of severe ocular trauma following the implementation of alcohol restrictions in Far North Queensland.

IMPORTANCE: Indigenous communities of Far North Queensland (FNQ) have one of the highest incidences of alcohol-related ocular trauma globally. BACKGROUND: To review the epidemiology of closed- and open-globe trauma admitted to Cairns Hospital from FNQ health districts following the implementation of alcohol restrictions in Indigenous communities. DESIGN: Retrospective study of cases from January 2014 to December 2018. PARTICIPANTS: A total of 142 cases identified from ICD-10 clinical-coding data. METHODS: Records were reviewed to determine demographics, clinical details and outcomes. MAIN OUTCOME MEASURES: Annual incidence by demography and ethnicity (Indigenous vs non-indigenous). RESULTS: Estimated annual incidence was 10.4/100 000 population (open-globe: 3.6/100 000, closed-globe: 6.8/100 000 population). Incidence rate ratio was 2.8× higher in Indigenous (22.8/100 000 population) compared to non-indigenous populations. Injury from assault was 8.2× higher in the Indigenous population. Alcohol was involved in 76% of assaults. Paediatric injuries comprised 20.4% of the cohort, with Indigenous children over-represented (44.8% of children). Visual acuity (VA) at presentation ranged from -0.08 logMAR to no-perception of light (NPL), with 41.8% poorer than +1.00 logMAR. Final VA ranged from -0.18 logMAR to NPL. Mean final VA was significantly better for closed- than open-globe injuries (+0.43 vs +1.01 logMAR). Ruptures had the poorest outcomes (mean +1.65 logMAR). CONCLUSIONS AND RELEVANCE: The overall incidence of severe ocular trauma in FNQ has decreased compared to that reported from 1995 to 2002. The extremely high incidence observed in the Indigenous communities of Cape York has decreased dramatically since the introduction of Alcohol Management Plans. Nevertheless, the Indigenous population still experiences significantly higher rates of severe ocular trauma, particularly from assault.

Purpureocillium lilacinum keratitis: a case series and review of the literature.

OBJECTIVE: To describe the clinical features and risk factors of and optimal antifungal therapy for Purpureocillium lilacinum keratitis. DESIGN: Retrospective case series in a quaternary referral hospital setting. METHODS: Comprehensive chart review of patients diagnosed with P. lilacinum keratitis in the past 10 years. PARTICIPANTS: Four patients were identified. All were aged 60 years or greater, with none having prior ocular trauma. Two had significant potential environmental exposure risks, and 3 were using systemic immunosuppressants for scleritis. RESULTS: All cases received empirical treatment that included topical corticosteroids. Three were treated with combined oral, topical, and intracameral voriconazole, but developed endophthalmitis necessitating surgery with poor outcomes, including enucleation in 2. One case received combined oral and topical voriconazole with terbinafine, and maintained visual acuity without the need for surgery. All P. lilacinum isolates were susceptible to voriconazole. CONCLUSIONS: P. lilacinum keratitis is rare, with the major risk factor being immunosuppression. There may be no history of ocular trauma. Microbiological diagnosis and antifungal susceptibility testing is essential. Combination synergistic antifungal therapy with topical voriconazole and oral terbinafine, with addition of systemic voriconazole if needed, results in the best outcome.

Phase II study of irinotecan in combination with temozolomide (TEMIRI) in children with recurrent or refractory medulloblastoma: a joint ITCC and SIOPE brain tumor study.

BACKGROUND: This multicenter phase II study investigated temozolomide + irinotecan (TEMIRI) treatment in children with relapsed or refractory medulloblastoma. METHODS: Patients received temozolomide 100-125 mg/m(2)/day (days 1-5) and irinotecan 10 mg/m(2)/day (days 1-5 and 8-12) every 3 weeks. The primary endpoint was tumor response within the first 4 cycles confirmed ≥4 weeks and assessed by an external response review committee (ERRC). In a 2-stage Optimum Simon design, ≥6 responses in the first 15 evaluable patients were required within the first 4 cycles for continued enrollment; a total of 19 responses from the first 46 evaluable patients was considered successful. RESULTS: Sixty-six patients were treated. Seven responses were recorded during stage 1 and 15 in the first 46 ERRC evaluated patients (2 complete responses and 13 partial responses). The objective response rate during the first 4 cycles was 32.6% (95% confidence interval [CI], 19.5%-48.0%). Median duration of response was 27.0 weeks (7.7-44.1 wk). In 63 patients evaluated by local investigators, the objective response rate was 33.3% (95% CI, 22.0%-46.3%), and 68.3% (95% CI, 55.3%-79.4%) experienced clinical benefit. Median survival was 16.7 months (95% CI, 13.3-19.8). The most common grade 3 treatment-related nonhematologic adverse event was diarrhea (7.6%). Grade 3/4 treatment-related hematologic adverse events included neutropenia (16.7%), thrombocytopenia (12.1%), anemia (9.1%), and lymphopenia (9%). CONCLUSIONS: The planned study primary endpoint was not met. However, its tolerability makes TEMIRI a suitable candidate chemotherapy backbone for molecularly targeted agents in future trials in this setting.

A phase II single-arm study of irinotecan in combination with temozolomide (TEMIRI) in children with newly diagnosed high grade glioma: a joint ITCC and SIOPE-brain tumour study.

A multicenter, two stage phase II study, investigated irinotecan plus temozolomide in children with newly diagnosed high grade glioma. The primary endpoint was tumor response during a two-cycle treatment window, confirmed by external review committee. Patients received oral temozolomide 100 mg/(m(2) day) (days 1-5) and intravenous irinotecan 10 mg/(m(2) day) (days 1-5 and 8-12) for two 21-day cycles (three cycles for patients exhibiting objective tumor response). Standard treatment was then administered according to local investigator choice. In total 17 patients were enrolled and treated by local investigators. However, central pathology review found three patients did not have a diagnosis of high grade glioma and another four patients did not have evaluable disease according to independent central radiological review. The primary endpoint was based on the first ten evaluable patients as determined by the external review committee. Recruitment was stopped for futility after there were no complete or partial responses during the two-cycle treatment window in the first ten evaluable patients. Five patients had stable disease, and five progressed. Data for secondary endpoints including; time to tumor progression, time to treatment failure, and overall survival is reported. The safety profile of the treatment showed the combination was tolerable with two patients (11.8 %) having grade three nausea, and one (5.9 %) experiencing a grade four neutropenia, leading to permanent discontinuation from adjuvant treatment. Irinotecan plus temozolomide, although well tolerated did not improve outcome over historical controls in this setting.