Faculty Bibliography

Purpose/UNASSIGNED:Few studies have evaluated the methodology by which radiation therapy (RT) for thyroid eye disease and compressive optic neuropathy is performed. The objective of this study was to retrospectively review our experience from a radiation planning standpoint and to determine whether current treatment methods provide adequate dose to target and collateral structures. Methods/UNASSIGNED:A retrospective review of 52 patients (104 orbits) with bilateral thyroid eye disease and compressive optic neuropathy treated with RT (20 Gy in 10 fractions) at our institution. RT plans were analyzed for target volumes and doses. Visual fields, color plates, and visual acuity were assessed pretreatment and at last available follow-up post RT. A standardized, anatomic contour of the retro-orbital space was applied to these retrospective plans to determine dose to the entire space, rather than the self-selected target structure. Results/UNASSIGNED:= .62), based on these dose differences. When beam placement was retrospectively adjusted to include a space of 0.5 cm between the lens and the anterior beam edge, there was a 39.4% and 20.3% decrease in max and mean dose to the lens. Conclusions/UNASSIGNED:Without a standardized protocol for contouring in thyroid eye disease, target delineation was found to be rather varied, even among the same practitioner. Differences in dose to the anatomic retro-orbital space did not affect outcomes in the follow-up period. Although precise contouring of the retro-orbital space may be of little clinical consequence overall, a >0.5 cm space from the lens may significantly reduce or delay cataractogenesis.

Indolent lymphomas have the potential to transform into more aggressive phenotypes. This phenomenon is best exemplified by the transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma into diffuse large B-cell lymphoma. However, it is less common to find multiple small cell lymphomas in the orbit, particularly mantle cell lymphoma. We present a case of an extranodal marginal zone lymphoma arising in the orbit that acquired a subclonal t(11;14) (q13;32) CCND1-IGH translocation, giving rise to a subclone of mantle cell lymphoma. The management of this tumor was targeted towards the more aggressive mantle cell subclone. The tumor exhibited an incomplete response to rituximab alone. To the best of the authors' knowledge, this represents the first such case to be described irrespective of site of origin.

Purpose: We describe a novel surgical approach for bilateral orbital roof decompression using a frontal osteoplastic flap without frontal sinus obliteration. This technique utilizes a combined external and endonasal endoscopic approach for wide exposure to the orbital roof bilaterally. We demonstrate this technique for the resection of a massive frontal fibrous dysplasia lesion in a healthy male with bilateral orbital roof involvement. The endonasal endoscopic portion of the technique includes a Draf III frontal sinusotomy (endoscopic modified Lothrop procedure) which precludes the need for frontal sinus obliteration, restores normal frontal sinus function, and allows for postoperative endoscopic surveillance.Methods: Report of novel surgical technique with video demonstration.Results: This technique for orbital roof decompression allows for removal of a frontal lesion, wide decompression of the bilateral orbital roof, and post-operative endonasal endoscopic surveillance of the cavity. The patient in whom we demonstrate this technique had complete resolution of his orbital symptoms and minimal residual fibrous dysplasia postoperatively.Conclusion: Bilateral orbital roof decompression for frontal lesions can be performed safely and effectively with a frontal osteoplastic flap without frontal sinus obliteration, restoring normal orbital and sinus function.

Purpose: The majority of pediatric patients with optic nerve gliomas (ONG) remain asymptomatic; however, a subset of patients suffer rapid, irreversible visual loss. The purpose of this study was to determine anatomic differences of ONG noted either by imaging or by intra-operative observation between patients with gradual visual dysfunction and those with rapid visual loss.Methods: A retrospective review was performed in patients with visual loss secondary to ONG. The clinical records, pathology, and radiographic images were reviewed for all patients. The degree of folding or plication of the optic nerve (ON) was determined by analyzing the most acute angle present in the course of the ON.Outcome measures: The primary outcome measure was the angle of plication or folding of the ON and the relationship of this to the course of visual dysfunction.Results: Six patients with ONG were included in the study. A structural difference in the ON was identified in four patients with rapid vision loss compared with two patients with more gradual visual dysfunction. In patients with rapid progressive visual loss, the ONG had a 90-degree or more acute plication of the ON. Those with more gradual visual loss had more obtuse bends in the ONG.Conclusions: We have identified that the intrinsic structure of the ONG may contribute to the rare but devastatingly rapid progression of visual dysfunction in some patients. Recognizing these changes may guide clinicians to intervene prior to the development of irreversible visual loss.

Purpose: We describe a novel technique for endonasal endoscopic orbital surgery. Previously described strategies for minimally invasive orbital surgery include mobilization of the medial rectus muscle and cryosurgical retraction of the pathologic lesion. Herein we describe endoscopic trans-septal suture retraction of an orbital cavernous hemangioma to facilitate intra-orbital dissection from an external approach.Methods: A 36-year-old male presented with blurred vision in his left eye for several years, along with several months of progressive left proptosis and decreasing visual acuity. Imaging revealed a 2.5 cm orbital mass consistent with a cavernous hemangioma. The patient underwent a combined transconjunctival and endoscopic approach for resection.Results: Following left medial orbital wall and floor decompression, endoscopic trans-septal suture retraction facilitated dissection of the lesion from intraorbital structures. A complete extracapsular resection of the lesion was achieved. The inferomedial orbit was reconstructed with a synthetic implant.Conclusion: Trans-septal suture retraction is an effective adjunctive technique for endonasal endoscopic orbital tumor surgery. This technique facilitates safe retraction and dissection of soft tissue lesions from critical intraorbital structures.

A 22-year-old Hispanic man with sickle cell trait presented with blurred vision, double vision, and pain with OD movement. MRI demonstrated an extra-axial mass centered around the temporal bone with extension into the middle cranial fossa and lateral aspect of the extra-conal right orbit, and mass effect on the lateral rectus muscle. Biopsy of the lesion was consistent with renal medullary carcinoma. CT chest/abdomen/pelvis confirmed a primary tumor in the right kidney. No additional metastases were found. Renal medullary carcinoma is a rare, highly aggressive malignancy, which almost exclusively affects young men of African descent with sickle cell trait or sickle cell disease. The authors present the second confirmed case of renal medullary carcinoma metastatic to the orbit, with ocular symptoms prior the typical presenting symptoms of flank pain and hematuria.

A 22-year-old female presented for evaluation of five years of progressive left exophthalmos and intermittent blurred vision. She had previously received laser treatment for peripheral retinal neovascularization and had undergone lip reconstruction for a left-sided congenital vascular facial malformation. Magnetic resonance imaging demonstrated diffuse enlargement of the left extraocular and temporalis muscles, with prominent vessels in the temporalis muscle and intraconal fat. Left fundoscopic examination revealed grossly enlarged, tortuous retinal vessels extending from the optic disc to the peripheral retina and an abnormal network of capillaries. On the basis of these findings, a diagnosis of retinoencephalofacial angiomatosis was established. Retinoencephalofacial angiomatosis is a rare, non-hereditary disorder associated with ipsilateral retinal, brain, and facial arteriovenous malformations. This is the first report, to the authors' knowledge, of retinoencephalofacial angiomatosis presenting with exophthalmos secondary to extraocular muscle enlargement.

PURPOSE/OBJECTIVE:To compare extraocular muscle volumes in thyroid eye disease patients with and without compressive optic neuropathy. METHODS:A retrospective review of 44 orbital CT scans (28 orbits without compressive disease and 16 orbits with compressive optic neuropathy) was conducted. The extraocular muscle volumes, summated soft tissue volumes, and optic nerve volumes were calculated at a section in the posterior 1/3 of the orbit. The visual fields of the orbits with compressive optic neuropathy were analyzed. RESULTS:The mean combined extraocular muscle/summated soft tissue volume ratio and the mean superior rectus-levator complex/summated soft tissue volume ratio were greater in those with compressive optic neuropathy than in those without compressive optic neuropathy (p = 0.02, 0.008, respectively). The ratio of the mean inferior, medial, or lateral rectus/summated soft tissue volume did not differ significantly between patients with or without compressive optic neuropathy (p values of 0.315, 0.615, and 0.254, respectively). Visual field analysis of the compressive optic neuropathy group demonstrated that 58% of the orbits with visual field defects had inferior field defects. CONCLUSIONS:When measured at a section near the orbital apex, the mean combined muscle/summated soft tissue volume ratio and the mean superior rectus-levator complex/summated soft tissue volume ratio are greater in those with compressive disease than those without. This suggests that the specific enlargement of the superior rectus-levator complex makes a significant contribution to thyroid eye disease-compressive optic neuropathy and may explain the inferior visual field deficits classically found in this group of patients.