Visual field and orbital computed tomography correlation in dysthyroid optic neuropathy due to thyroid eye disease.

Purpose: The pathogenesis of dysthyroid optic neuropathy (DON) in thyroid eye disease (TED) is thought to be compression of the apical optic nerve by hypertrophied extraocular muscles. We correlated worsening DON to the area occupied by extraocular muscles.Methods: Records of adults with TED DON evaluated from 1/1/2013 to 1/1/2018 were retrospectively reviewed. Each patient's visual field with the worst mean deviation (MD) was selected. Orbit CT scans were reviewed. Reformatted oblique coronal images were created perpendicular to the optic nerve. The cross-sectional area (CSA) of the orbit and each muscle group was measured and expressed as ratios of the CSA of the orbital apex. Univariate and multivariate analysis was performed for predictors of HVF MD.Results: 34 orbits with TED DON were analyzed. On orbital CT, the superior muscle complex occupied 15% of the apex (range 6-26%), inferior 18% (range 6-33%), lateral 10% (range 4-18%), medial 17% (range 8-27%), and all combined 61% (range 28-80%). Increasing total muscle area and superior complex area correlated with worsening MD. In multivariate linear regression, the superior muscle complex remained a significant predictor of MD (p = 0.01) over total muscle area (p = 0.25).Conclusions: Enlargement of extraocular muscles is common in TED, but DON occurs in only 6%. Our findings demonstrate that as DON worsens, as quantified by visual field MD, the superior muscle complex crowds the apex. This is consistent with the typical inferior visual field findings seen in TED DON. Hypertrophy of the superior rectus and levator palpabrae superioris complex may be predictive of worsening DON.

Atypical Case of Rosai-Dorfman Disease of the Lacrimal Gland with Adjacent Bone Erosion.

BACKGROUND/AIMS: Rosai-Dorfman disease (RDD) is a rare, self-limited disorder of unknown etiology that affects children and young adults worldwide and typically manifests as chronic, painless cervical lymphadenopathy. Orbital involvement is very rare and may be an isolated extranodal manifestation or associated with concurrent systemic disease. Adjacent bone involvement is most exceptional, and secondary optic neuropathy has never been reported. METHODS: This is a case report with review of the literature. RESULTS: We present a 32-year-old man who, over a 3-month period, developed worsening vision, headache, and vertical diplopia. On examination, there was decreased vision with dyschromatopsia, proptosis, and hypotropia of the left eye. CT scan of the orbits revealed a soft tissue mass inseparable from the lacrimal gland with adjacent bone erosion. Histopathologic evaluation revealed a diffuse infiltrate of histiocytes, lymphocytes, plasma cells, and neutrophils with peripolesis and emperipolesis (tunneling of lymphocytes and plasma cells in the histiocytes' cytoplasm without destruction), consistent with RDD. Resolution of symptoms as well as of the optic neuropathy was achieved with oral corticosteroids. CONCLUSION: RDD is an important diagnosis that must be considered in the differential diagnosis of an orbital mass.

Acquired Obliteration of the Proximal Lacrimal Drainage System.

PURPOSE: We present a series of patients with acquired obliteration of one or more components of the proximal lacrimal drainage system, including puncta and canaliculi. This finding was noted with and without other obstructions or stenoses of the lacrimal drainage system. Review of these patients in search of possible risk factors for this condition and histopathology of two patients are presented. METHODS: Medical records of patients treated at the Massachusetts Eye and Ear Infirmary between 2010 and 2016 with diagnosis codes of lacrimal punctum stenosis, stenosis of unspecified lacrimal punctum, epiphora due to insufficient drainage, and epiphora not otherwise specified were retrospectively reviewed. Patients were excluded if there was a history of congenital punctal agenesis, trauma, or any other identifiable etiology of lacrimal system obstruction. RESULTS: Twelve patients were identified with obliterated puncta. Most patients were female (n = 11, 91.7%) and Caucasian (n = 9, 75%). The mean age was 61 years (median 62, range 22-90 years). Clinical findings ranged from one involved punctum to all four puncta, and associated ipsilateral canalicular obstruction was noted in 4 patients (33.3%). The inferior puncta were the more frequently affected structures (61.5%) compared to the superior puncta (38.5%). Nine patients (75%) reported use of topical ophthalmic medications prior to onset of symptoms. The most frequent class of medication implicated was topical antibiotics; however, patients often used a combination of medications. In 2 cases, pathological specimens of the canaliculi revealed absence of a canalicular lumen. CONCLUSIONS: Although various combinations of lacrimal system stenoses and obstructions have been described, acquired punctal obliteration is an under-recognized entity. These cases differ from typical punctal stenosis in that the puncta are completely obliterated, and no indication of a punctal opening is clinically detectable. In this study, 3 patients had previously documented normal lacrimal irrigation. The ipsilateral canaliculi were also found to be obstructed in a third of patients. Two patients had complete absence of a canalicular lumen on histopathology. The predominance of inferior punctum involvement, patient history of symptom onset after ipsilateral medication use, and known prior patent irrigation in several cases support the hypothesis that these cases represent a severe yet insidious idiosyncratic reaction to topical medication.

Effect of resection of an orbital arteriovenous malformation on central venous pressure.

We report the first utilization of intraoperative central venous pressure (CVP) monitoring in the resection of an orbital arteriovenous malformation. A 24-year-old woman with a history of a left orbital mass who had previously undergone resection of a cranio-orbital arteriovenous malformation presented with gradual recurrence in the left orbit. She visited the emergency department with sudden vision loss, which resolved over several hours. This transient vision loss was thought to be due to a steal phenomenon from the ophthalmic artery due to the residual vascular malformation. Further surgical resection was undertaken. A preoperative angiogram identified residual feeding vessels, and the larger vessels were embolized. At the start of the procedure, her CVP was elevated (29 mm Hg), as measured by a central venous line. The remaining feeding vessels were surgically ligated, and an intraoperative arteriogram confirmed their successful ablation. At the conclusion of the procedure, the CVP had decreased to 9 mm Hg.