Faculty Bibliography

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.

PURPOSE/OBJECTIVE:To evaluate the association of risk of obstructive sleep apnea (OSA) and thyroid eye disease-compressive optic neuropathy (TED-CON). METHODS:The authors performed a retrospective observational study. All TED patients evaluated by the principal investigator over the past 5 years were screened with the snoring tired observed pressure (STOP)-Bang questionnaire to assess their risk for OSA. Patients were grouped into 2 age-matched cohorts based on the presence (TED-CON) or absence of CON. The groups were then compared using chi-square analysis for binary variables and Student t test for continuous variables. RESULTS:Three hundred twenty-six patients were identified to have TED. Of those, 109 were enrolled in the study. The prevalence of high risk of OSA was significantly higher in the TED-CON (59.2%) when compared with the noncompressive TED group (32.8%; p = 0.006). CONCLUSIONS:Obstructive sleep apnea and TED have each been independently associated with elevated serologic and tissue inflammatory mediators. The systemic inflammation associated with OSA has been implicated in the pathogenesis of disease states aggravated by untreated OSA. Effective treatment of OSA decreases the levels of circulating inflammatory mediators. Currently, smoking is the only known modifiable risk factor in TED. There is evidence to implicate the pathologic elevation of inflammatory cytokines in the mechanism of smoking on TED. This preliminary investigation reveals a significantly greater prevalence of OSA risk factors among patients with TED-CON, suggesting that this may be another modifiable risk factor associated with TED. Based on screening with the STOP-Bang questionnaire, there is a higher percentage of patients at high risk of OSA with TED with CON than with TED without CON.

PURPOSE/OBJECTIVE:This study compares the age-related clinical features of thyroid eye disease-compressive optic neuropathy (TED-CON) to those with noncompressive disease (TED-NC). METHODS:A retrospective case series review of 165 orbits from 121 patients with TED-CON were compared with an age- and gender-matched cohort of 88 orbits from 44 patients with TED-NC with institutional review board approval. Clinical features including exophthalmos, dyschromatopsia, restricted ocular motility, visual acuity, and mean deviation on 24-2 Humphrey Visual Field were compared in 5 age groups. A previously validated formula, the Columbia TED-CON Diagnostic formula, used to mathematically predict the presence or absence of compressive optic neuropathy was applied, and the sensitivity and specificity of the formula was measured in each age group. RESULTS:Exophthalmos, dyschromatopsia, restricted ocular motility, and mean deviation on 24-2 Humphrey Visual Field vary significantly across age groups in patients with TED-CON. Conversely, only visual acuity and dyschromatopsia vary significantly across age groups in patients with TED-NC. There was a significant difference between the TED-CON and the TED-NC groups when comparing the 2 groups by decade. The Columbia TED-CON Diagnostic formula had high sensitivity and specificity (74-90%) in all age groups. CONCLUSIONS:This is the first study to date demonstrating the age-related variability of clinical characteristics in a large series of patients with either TED-CON or TED-NC. The clinical phenotype of TED-CON varies significantly by decade when examining exophthalmos, dyschromatopsia, ocular motility restriction, and mean deviation on 24-2 Humphrey Visual Field. These clinical characteristics are also significantly different when compared with a population of patients with TED-NC.

Adult xanthogranulomatous disease of the orbit is a rare and incompletely understood entity. It can be limited to the orbit or be associated with systemic disease. While routine systemic surveillance is the standard of care for patients with orbital xanthogranulomatous disease, there is no universally accepted protocol for managing periorbital and orbital involvement. The authors report a case of adult orbital xanthogranuloma, without systemic disease, who, after 10 years of observation, demonstrated sustained radiographic regression of the lesions. To the authors' knowledge, this represents one of the first reports of spontaneous regression of untreated orbital xanthogranuloma, and supports observation of lesions that do not compromise ocular function. We present our case in the context of a major review of various treatment strategies described in the literature, including surgical resection, radiotherapy, plasmapheresis, corticosteroids, multiple immunomodulatory medications.

Buphthalmos can create treatment challenges for socket rehabilitation following enucleation due to compression atrophy and mechanical stretching of adnexal tissues. The authors report a case demonstrating a surgical technique for simultaneous volume augmentation of the anophthalmic socket, expansion of the conjunctival fornix, and repair of lower eyelid retraction in a patient with history of buphthalmos and a previously placed 22 mm orbital implant at the time of enucleation. The authors utilized a single-stage, bipedicle dermis fat graft. Postoperatively, the patient demonstrated improvement in lower eyelid position, lower eyelid volume, improved prosthesis fit, improved three-dimensional projection of the ocular prosthesis, and an improvement in the superior sulcus deformity. Bipedicle dermis fat grafts are an option for surgical rehabilitation of the anophthalmic socket when orbital volume deficiency, conjunctival fornix contracture, and eyelid retraction are present and an adequately sized orbital implant has previously been placed.

Orbital decompression surgery and medical therapy for thyroid eye disease (TED) have evolved over the past 150 years and afforded the opportunity to restore pre-disease appearance and visual function. This manuscript explores the past 150 years of surgical innovation for the treatment of TED. The "Age of Surgical Heroism" spans the time from 1888 to 1979 during which the pioneers of orbital decompression developed lateral orbitotomy, transcranial decompression, paranasal sinus decompression, and transantral decompression despite an incomplete understanding of the pathophysiology of both TED and a limited ability to non-invasively assess their patients. The "Age of Surgical Refinement" dawned with the development of computed tomography and represents the years 1979-2000. During this time, the "swinging eyelid" approach for two- and three-wall decompressions was introduced, a combined orbital-extradural four wall decompression procedure was developed, fat decompression was explored, and endoscopic decompression techniques were advanced. At the beginning of the 21st century, our understanding of the orbital pathophysiology of TED evolved significantly. Clinicians recognized the age-related phenotype of TED based largely on the relative contribution of extraocular muscle enlargement vs. orbital fat expansion. The "Modern Age" of Customized Orbital Decompression features both "medical decompression" during the active phase of TED and, in the stable phase, customized surgical plans incorporating individual patients' anatomy, orbital pathology, and surgical goals that collectively maximize therapeutic benefit while minimizing therapeutic morbidity.

Superficial subcutaneous lipomas are benign, slow-growing neoplasms comprised mature adipose cells that may be surrounded by a fibrous tissue capsule. While found commonly on the body, simple lipomas are rarely described in the eyelids. Reports of two eyelid lipomas in the literature describe the anatomy to be posterior to the orbital septum. The presenting authors report the case of a simple lipoma of the eyelid found fully in the preseptal plane. Lipomas can occur anterior to the orbital septum and should be considered in the differential when presented with a painless, slow-growing eyelid mass in adults.

PURPOSE/OBJECTIVE:To determine the reliability of 3 scales for assessing soft tissue inflammatory and congestive signs associated with thyroid eye disease. METHODS:This was a multicentered prospective observational study, recruiting 55 adults with thyroid eye disease from 9 international centers. Six thyroid eye disease soft tissue features were measured; each sign graded using 3 scales (presence/absence [0-1], 3-point scale [0-2], and percentage [0-100]). Each eye was graded twice by 2 independent raters. Accuracy (fraction of agreement) was calculated between the 2 trials for each rater (intrarater reliability) and between raters for all trials (interrater reliability) to determine the most sensitive scale for each feature that maintained a threshold of agreement greater than 0.70. Trial, intrarater reliability, and interrater reliability were determined by accuracy measurement of agreement for each inflammatory/congestive feature. RESULTS:Fifty-five patients had 218 assessments for 6 thyroid eye disease metrics. The intrarater reliability for each feature was consistently better than the interrater reliabilities. Using an agreement of 0.70 or better, for the interrater tests, conjunctival and eyelid edema could be reliably measured using the 0-1 or 0-2 scale while conjunctival and eyelid redness could only be reliably measured with the binary 0-1 scale. Caruncular edema and superior conjunctival redness could not be measured reliably between 2 raters with any scale. The percentage scale had poor agreement unless slippage intervals of >20% were allowed on either side of the measurements. CONCLUSIONS:Of the specific periocular soft tissue inflammatory features measured between raters in the Clinical Activity Score and Vision, Inflammation, Strabismus, Appearance scales, edema of the eyelids and conjunctiva could reliably be measured by both 0-1 and 0-2 scales, erythema of the eyelid and bulbar conjunctiva could reliably be measured only by the 0-1 scale, and the other parameters of superior bulbar erythema and caruncular edema were not reliably measured by any scale.

A 15-month-old male was referred for biopsy of presumed rhabdomyosarcoma in the setting of rapidly progressing left-sided proptosis. Examination revealed left periorbital edema and left hypoglobus. MRI revealed a soft-tissue density mass within the left lateral retrobulbar space. Several days later, he developed acute periorbital ecchymosis and increasing edema. With high suspicion for a vascular lesion, a CT scan was performed with dynamic arterial and venous imaging. Central filling was noted in the lateral retrobulbar component with increased enhancement on delayed venous imaging in the middle cranial fossa component favoring the diagnosis of a low-flow orbital venous malformation. In the setting of spontaneous orbital hemorrhage and risk of future vision loss, the decision was made to proceed with a combined neurosurgical approach treating the intracranial component and debulking the orbital component. This case highlights the importance of thorough radiographic evaluation prior to proceeding with a surgical procedure in the appropriate clinical context.