Faculty Bibliography

PURPOSE: To correlate clinical features, imaging and pathologic findings in recurrent Solitary Fibrous Tumor of the orbit (SFT) in order to predict long-term behavior. METHODS: Clinical features, imaging and pathologic findings of three patients with biopsy proven SFT are reported. Demographic and clinical features were recorded at presentation and at each consultation; imaging was performed as a diagnostic tool and for follow-up. A biopsy was performed at presentation and subsequently when symptoms worsened. Pathology specimens were reviewed retrospectively to corroborate diagnosis. Intraoperative and histopathologic features were recorded. A correlation was made between clinical, imaging and pathologic results to identify outcome predictors of recurrence, locally aggressive behavior and malignant transformation. RESULTS: All cases presented recurrent tumors with locally aggressive behavior over time. All were women in the fifth decade of life. Tumors induced proptosis, swelling of the lids and eye displacement at presentation and were diagnosed as other types of collagen-rich tumors before CD34 immunohistochemistry was available. Mean follow-up was 26.6 years (range 12-37). Relevant findings for all cases included a heterogeneous, irregular tumor containing cystoid spaces filled with mucoid material diffusely enhancing with imaging techniques. Intraoperative findings included a gelatinous matrix within the center of the tumor mass, which was not present at primary resection. Histopathology could not detect specific cellular patterns or immunological markers related to these changes. CONCLUSIONS: Recurrence and locally aggressive behavior was better predicted by imaging and surgical findings rather than histopathological characteristics. Cystoid degeneration in recurrent tumors may suggest malignant transformation over time.

PURPOSE: Outcome evaluation in ocular adnexal lymphoma (OAL) is based on clinical assessment and conventional volumetric changes in tumor size. The purpose of this retrospective study was to compare if changes in apparent diffusion coefficient (ADC) tumor values obtained by diffusion-weighted MRI corresponded to changes in enhancing tumor volume in the evaluation of early treatment response or failure in patients with OAL. METHODS: A retrospective case series analysis of conventional contrast-enhanced orbital MRI and diffusion-weighted sequences was performed on 8 pathologically confirmed OAL tumors before and after therapy. Mean ADC values and normalized ADC ratios were obtained using a region-of-interest analysis method on enhancing OAL lesions; tumor volumes were calculated using a manual segmentation method. Changes in tumor volume, mean ADC tumor values, and normalized ADC ratios were compared before and after therapy using a Wilcoxon rank-sum test. RESULTS: Overall, a significant difference was found in mean ADC values and normalized ADC ratios within OAL tumors before and after therapy (p < 0.05), irrespective of the type of therapy administered. There was a trend toward decreased mean enhancing tumor volume after therapy (p = 0.161). An increase in ADC values and a decrease in enhancing tumor volume after therapy correlated with a positive treatment response in 7 of 8 tumors; a decrease in ADC values and an increase in enhancing tumor volume after therapy correlated with a negative treatment response in 1 of 8 tumors. CONCLUSIONS: Tracking changes in tumor ADC values after various treatment regimens for OAL may be useful in predicting early treatment response or failure and can provide complementary information that corresponds to conventional volume changes in tumor size. Further validation of these preliminary results in larger prospective randomized trials is needed.

A benign orbital perineurioma was discovered just posterior to the lacrimal sac during surgery for recurrent bacterial dacryocystitis in an 83-year-old woman. The tumor was circumscribed but nonencapsulated and located exterior to the lacrimal sac mucosa. It was composed of cytologically bland spindle cells organized in graceful, elongated, and twisted bundles reminiscent of a storiform pattern. Immunohistochemical staining displayed a diagnostic pattern of Claudin-1, GLUT-1, and epithelial membrane antigen positivity. Malignant transformation is virtually unknown, even with incomplete excision. The differential diagnosis includes neurofibroma, schwannoma, leiomyoma, solitary fibrous tumor (CD34 positive), and low-grade fibromyxoid sarcoma

PURPOSE: To determine intraobserver and interobserver reliability of 3 clinical techniques for measuring ocular ductions in patients with thyroid eye disease and to compare these with an established method using a Goldmann perimeter. Our secondary goals were to compare the clinical methods for test duration, ease of learning and performance, and whether these differed between novices and experts. DESIGN: Multicenter, prospective, comparative trial. PARTICIPANTS: We recruited 38 patients with thyroid orbitopathy and reduced ocular motility from 7 academic centers. METHODS: At each center, 2 novices and 2 experts measured the ocular ductions of each eye of each patient in 4 directions (0 degrees , 90 degrees , 180 degrees , and 270 degrees ) using 3 techniques (best guess [BG], light reflex [LR], and a ruler measuring [RU] technique). Test duration and clinicians' feedback were recorded. A subjective and objective measurement for ocular ductions using a Goldmann perimeter was performed for each subject by a trained technician. The 3 clinical measurements and the perimetry measurements were performed twice, separated by >/=1 hour. MAIN OUTCOME MEASURES: We measured the intraobserver and interobserver reliability of the 3 clinical techniques and intraobserver reliability of Goldmann perimeter. Clinical testing reliability was compared between experts and novices. We also examined test duration and clinician feedback. RESULTS: The LR technique had significantly better intraobserver and interobserver repeatability compared with the BG or RU clinical measurements and statistically was equivalent to the gold-standard perimetric technique. Reliability was constant regardless of the amount of restriction in ocular movement. There was no difference between reliability values for experts and novices. The LR and BG techniques were significantly faster than the RU or perimetry techniques and were considered easiest to learn and perform, but clinicians had most confidence in the LR technique results. CONCLUSIONS: The LR technique for measuring ocular ductions in thyroid orbitopathy is more reliable than other clinical techniques and as reliable as the established technique using the perimeter. However, unlike the latter method, it is easier to learn and perform by both novices and experts, is significantly faster, and can be performed by the clinician without machinery or a trained technician. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any of the materials discussed in this article

PURPOSE: : The purpose of this study was to investigate the prevalence of a common canalicular entrance in the lacrimal sac and to investigate the anatomy of the canalicular/lacrimal sac junction with direct visualization using a novel cadaveric dissection technique. METHODS: : Preserved cadavers were dissected to allow direct visualization of the canalicular entrance(s) to the lumen of the lacrimal sac. The prevalence of a common canaliculus and the anatomical variations of the canalicular/lacrimal sac mucosal fold of tissue were recorded. RESULTS: : One hundred twenty-four lacrimal systems (95 cadavers; 43 female, 52 male) were included in the study analysis. Overall, 123 lacrimal systems demonstrated a common canaliculus entering the lacrimal sac. Only one demonstrated 2 separate orifices (right orbit; male) in the sac (0.08%; 95% confidence interval, 0.1%-4.4%). Seventy-four lacrimal systems had some variation of a canalicular/lacrimal sac mucosal fold (59.7%). The remaining 50 (40.3%) had no visible canalicular/lacrimal sac mucosal fold. CONCLUSIONS: : This study provides direct anatomical evidence that the prevalence of separate canalicular orifices in the lacrimal sac is lower than previously reported (<1%). Additionally, the presence of a valve-like structure at the canalicular/lacrimal sac junction is common. These observations can potentially play a role in evaluating and treating lacrimal system pathology

PURPOSE:: Evaluate the relationship between extraocular muscle (EOM) size, measured by computed tomography, and ocular motility in thyroid eye disease (TED). METHODS:: This case series is based on a retrospective review of the records of 54 patients (108 orbits) with TED. Main outcome measures included EOM size and degree of motility restriction. The average diameter of each rectus muscle was compared with published norms. Four subpopulations based on Age (<40 or >/=40 years) and State of thyroid eye disease (active or stable) were studied. Versions were measured by the corneal light reflex method. The trend of muscle diameter versus motility restriction was evaluated. RESULTS:: The average EOM diameter was greater than the norm in the study cohort and 4 subpopulations. The average diameter was largest in the Older and Active TED subpopulations. The inferior rectus and medial rectus were most frequently restricted in the study cohort and 4 subpopulations. The medial rectus had the strongest trend between increasing diameter and motility restriction, followed by the inferior rectus and the superior muscle group (comprised of the superior rectus and levator palpebrae superioris). However, there was a general lack of strong correlation between the diameter of the rectus muscles and their respective motility, especially in the Younger subpopulation. CONCLUSION:: EOM diameters are larger and have more restricted motility in the Older and Active TED subpopulations. Contrary to prior publications, the correlation between EOM diameters and motility was weak, especially in the Younger subpopulation. These findings suggest that the pathophysiology of EOM enlargement is different based upon the age of the patient and the activity of the orbitopathy

PURPOSE:: Treatment of large-angle strabismus in thyroid eye disease has historically suffered from low success rates. The authors report a novel technique that adds Tenon recession without conjunctival recession to standard extraocular muscle surgery. METHODS:: Twenty-six patients with thyroid eye disease-associated strabismus with preoperative deviations of >/=25 prism diopters in horizontal or vertical deviations underwent strabismus surgery. The amount of recession for a given muscle was determined by a combined analysis of the preoperative versions, deviation in primary gaze and in gaze opposite the restricted muscle, and intraoperative forced duction testing. Before conjunctival closure, the underlying Tenon layer and associated orbital tissues were recessed and allowed to retract posteriorally. The overlying conjunctiva was subsequently reapproximated to its anatomic position without tension. RESULTS:: The average preoperative measurements for horizontal and vertical deviations were 39.2 +/- 13.4 and 45.0 +/- 18.9 prism diopters, respectively. Average postoperative measurements for horizontal and vertical deviations were 3.2 +/- 6.0 and 11.1 +/- 11.9 prism diopters, respectively. Overall, 22 of 26 patients (84.6%) were within 10 prism diopters of intended correction postoperatively and achieved binocular fusion with or without the use of prisms for both distance and near vision. Four had residual deviations that required reoperation, all of which successfully produced binocular vision without the use of prisms. CONCLUSIONS:: In the authors' experience, Tenon recession improves outcomes in surgery for large-angle strabismus in thyroid eye disease