MULTIMODAL IMAGING OF MACULAR CHOROIDAL MACROVESSEL: A REPORT OF TWO CASES.

PURPOSE: To report two cases of macular choroidal macrovessel with description of multimodal imaging and review of published cases. METHODS: Medical and imaging records were retrospectively reviewed. A literature review was performed to identify other cases of macular choroidal macrovessel published between 1990 and 2018. RESULTS: There were 2 patients referred for evaluation of a potential choroidal tumor, including a 55-year-old white woman with no visual symptoms and a 68-year-old white woman with blurred vision. Funduscopic examination in each case revealed a focal area of choroidal elevation in the temporal foveal area with a single, dilated, slightly tortuous choroidal vessel, estimated to measure 250 µm and 300 µm, respectively, at widest girth, and extending temporally toward the equator with a tapering width. Optical coherence tomography showed an optically hollow choroidal lesion elevating the retinal pigment epithelium with overlying ellipsoid zone mottling. Both cases demonstrate shallow subretinal fluid, sparing the foveola. Fluorescein angiography showed faint staining along the course of the vessel in both cases. Indocyanine green angiography demonstrated clear visualization of the ectatic vessel with early filling and late staining without leakage. Both patients retained visual acuity of 20/25 in the affected eye and did not require treatment. CONCLUSION: Macular choroidal macrovessel is a rare vascular anomaly presenting as a single, dilated, tortuous choroidal vessel, originating in the temporal foveal region and extending temporally to the equator. This finding can simulate a choroidal neoplasm or parasitic track. Optical coherence tomography and indocyanine green angiography have diagnostic value demonstrating the vascular nature of this lesion and delineating the vascular course.

Endoscopic endonasal resection of orbital schwannoma assisted with small-incision medial orbitotomy: case series and surgical technique.

PURPOSE: To describe a surgical approach for the resection of schwannomas occurring in the medial aspect of the orbit and to review a series of patients who underwent this novel technique. METHODS: This retrospective, non-comparative case series presents the surgical technique and outcomes of patients who underwent removal of a medial orbital schwannoma via an endoscopic endonasal approach combined with a small-incision medial orbitotomy by a team of two surgeons (BSB and SKF). Patient demographics, pre- and post-operative clinical examination findings, visual field testing, and radiographic studies were reviewed. Operative reports were reviewed for technical details and complications. RESULTS: The patients included a 12 year-old male, 73 year-old female and 8 year-old male. Indications for surgery included: decreased visual acuity, diplopia, proptosis and Humphrey visual field (HVF) deficit, in the presence of a medial orbital biopsy-proven schwannoma. The surgical approach in all three patients was primarily endoscopic endonasal. Additionally, two had transcaruncular orbitotomies and one had a small-incision medial lid crease orbitotomy to assist with lateral tumor dissection. Tumor resection was complete in one case and near-total in two cases. There were no intra-operative surgical complications. Average resected specimen volume was 3.41 cm3 ± 2.20. All patients had post-operative improvement in visual acuity (VA) and proptosis. Post-operative follow-up intervals were 27.5 months, 12.3 months and 3.5 months, respectively. CONCLUSION: Resection of orbital schwannomas using an endoscopic endonasal approach with small-incision medial transorbital assistance is a safe and effective option for a multidisciplinary surgical team.

Botryoid Rhabdomyosarcoma of the Conjunctiva in a Young Boy.

Primary conjunctival rhabdomyosarcoma (RMS) is a rare entity that can present with or without papillomatous features. A 5-year-old Asian boy was referred for a rapidly growing conjunctival tumor in the superior fornix of the left eye. Surgical excision yielded a 28 mm multilobulated papillomatous specimen that exhibited histopathologic and immunohistochemical features consistent with embryonal (botryoid) RMS. Molecular analysis revealed the absence of the PAX3/FOXO1 fusion gene, indicating favorable prognosis. After surgery, he was promptly treated with systemic chemotherapy and proton beam radiotherapy.