Background:Intra-arterial administration of melphalan chemotherapy has shown promise in the treatment of retinoblastoma. This report describes our results using superselective intra-arterial melphalan in patients with newly diagnosed retinoblastoma and those who were treated for progression after systemic chemotherapy.Methods:This is a retrospective review of all retinoblastoma patients treated with intra-arterial melphalan at the University of California, San Francisco from March 2010 to August 2012. Twenty eyes (16 patients) underwent 40 intra-arterial melphalan infusions, and dose was determined by age. Patients were treated at monthly intervals and received a range of 1–5 treatments. Response to therapy, toxicity, and procedural radiation exposure was assessed.Results:All patients are alive without metastatic disease at a median follow-up of 14.5 (1–29) months. Treatment with enucleation or external beam radiation was avoided in 11/20 eyes (55%) overall [6/12 (50%) in newly diagnosed eyes and 5/8 (63%) in refractory/relapsed eyes]. Response rates (per the International Classification of Retinoblastoma) were as follows: 6/7 (86%) in groups A–C and 5/13 (38%) in groups D and E. Nonhematologic and hematologic toxicities were minimal and comparable with those in previous reports. The mean procedural radiation dose was 20.2 ± 11.9 mGy per eye per procedure.Conclusion:Superselective intra-arterial melphalan therapy is effective for less advanced eyes but further modifications to therapy are required to improve results in eyes with advanced retinoblastoma.

A 64-year-old hypertensive woman presented with complaints of a painless, progressive decrease in vision, headache, dizziness for the last month. She was a known case of diabetes mellitus on insulin therapy. On examination, best-corrected visual acuity was 6/12 in the right eye and 6/18 in the left eye. The fundus examination in both eyes revealed features of grade IV hypertensive retinopathy. Systemic examination revealed the raised blood pressure of 200/110 mm Hg. Diagnosis of pheochromocytoma was made on the basis of increased urinary norepinephrine (892.8 mg/dL) and mass in the left adrenal gland (measuring 31×28 mm) at contrast-enhanced CT. Medical management to control hypertension was done and ultimately, she underwent left laparoscopic adrenalectomy. After 10 months of surgery, the patient was asymptomatic, blood pressure was within normal limit and her vision improved to 6/6 in both the eyes. The retinal features of hypertensive retinopathy had completely disappeared.

Invasive sino-orbital aspergillosis is a rare cause of orbital apex syndrome (OAS) in immunocompetent patients and often misdiagnosed as tumour because of its aggressive nature and invasive patterns. We report a 23-year-old immunocompetent man presenting with painful progressive loss of vision, ophthalmoplegia and proptosis of the right eye suggestive of OAS. MRI with gadolinium contrast showed an enhancing heterogeneous mass filling the paranasal sinuses, extraconal space and extending up to the right orbital apex. A functional endoscopic biopsy reported as invasive sino-orbital aspergillosis. He was started on intravenous voriconazole and maximal surgical debridement was done. He gradually regained his vision to 20/30 in the right eye. A review of literature reported several such cases which were managed medically or surgically but with poor visual recovery. This case highlights the need for awareness among clinicians for early diagnosis and treatment to prevent vision loss and better survival.

No abstract