Citations

Purpose: This study assesses intersurgeon variability in proptosis reduction after orbital decompression for thyroid eye disease. Methods: This multicenter retrospective study included patients with thyroid eye disease who underwent orbital decompression from 1 of 7 surgeons at 7 different institutions between January 2002 and December 2018. Data were included if a single decompression technique was performed on ≥10 patients by ≥2 surgeons. The primary outcome was postoperative change in proptosis with emphasis on comparison among surgeons utilizing similar surgical techniques. Statistical analysis was performed with χ 2 and ANOVA testing, and a multivariable logistic regression model was generated. Results: Six hundred thirty-three orbits that underwent orbital decompression were included. Five different decompression techniques were analyzed: medial wall (n = 29), fat + lateral wall (n = 113), medial wall + floor (n = 123), fat + lateral wall + medial wall (n = 140), and fat + lateral wall + medial wall + floor (n = 228), without significant difference in proptosis reduction among surgeons. Surgeons did not demonstrate significant differences in outcomes at different time points compared with each other or themselves. Outcomes when comparing endoscopic versus open medial wall decompression varied among surgery types. Multivariate modeling revealed a statistically significant association between postoperative change in proptosis with preoperative proptosis ( p < 0.001). Conclusions: Postoperative change in proptosis did not differ significantly between surgeons utilizing similar orbital decompression techniques for patients with thyroid eye disease. This study may strengthen the statistical validity of multicenter clinical trials assessing orbital decompression outcomes performed by surgeons employing uniform surgical techniques, thereby advancing our understanding of optimal surgical management strategies for thyroid eye disease.

e23554 Background: Follicular dendritic cell sarcoma (FDCS) is a low-grade neoplasm arising from follicular dendritic cells. Its diagnosis is challenging due to its extreme rarity, and the optimal management remains unknown. Methods: We conducted a retrospective study of FDCS patients (pts) seen at Mayo Clinic, MN since 2018. Time to event analyses were done from the time of diagnosis. Results: Ten pts with FDCS were identified (Table). The median age was 60 years (range, 38-76) and 5 were males. Abdominal pain was the most common presenting symptom (30%) followed by constitutional symptoms, shortness of breath, and vision change. In 3 pts, the diagnosis was incidental. The 2 most common primary locations were abdomen/pelvis (50%) and mediastinum (30%). Six pts (60%) presented with advanced disease and 3 (30%) had Castleman’s disease. Most (60%) had advanced disease. Six pts had Epstein-Barr virus in situ hybridization and all tested negative. Seven pts had next generation sequencing and 5 (83%) had genetic alterations detected with CDKN2A/B alterations being most frequent (3 pts, all with advanced disease). The median follow up was 4.3 years (range, 0.2-13.3). In the 4 patients with localized disease, 3 underwent complete surgical excision resulting in complete remissions (CRs). The one who received radiation had a partial remission (PR). The median time to progression after 1st line treatment was 4 months (all with advanced disease). All 3 pts who died had advanced disease and 2 had CDKN2A/B alterations. Conclusions: In FDCS, localized diseases managed by surgical excision with or without radiation can achieve long term remissions. Advanced diseases generally do not respond to multimodality therapy including immunotherapy but can have disease stabilization with variable survival. CDKN2A/B mutations were common and potentially associated with a worse outcome. Better understanding of the impact of the FDCS mutational landscape on prognosis may provide novel approaches to therapy. [Table: see text]

Purpose: The purpose of this study was to characterize intradiploic dermoid and epidermoid orbital cysts to determine any differences in clinical, radiographic, or surgical features. Methods: A retrospective review was performed of patients presenting with intradiplopic dermoid or epidermoid cysts. Additionally, a complete review of the literature was performed to identify cases of intradiplopic orbital dermoid and epidermoid cysts. Data collected included age, sex, presenting symptoms, location of intradiplopic cyst, ophthalmic findings, treatment, and follow-up. Clinical features of dermoid versus epidermoid cyst were compared. Additionally, machine-learning algorithms were developed to predict histopathology based on clinical features. Results: There were 55 cases of orbital intradiploic cysts, 49 from literature review and six from our cohort. Approximately 31% had dermoid and 69% had epidermoid histopathology. Average age of patients with dermoid cysts was significantly lesser than that of patients with epidermoid cysts (23 vs. 35 years, respectively; P = 0.048). There was no difference between sex predilection, presenting symptoms, radiographic findings, or surgical treatment of dermoids and epidermoids. The majority of patients (64%) underwent craniotomy for surgical removal. Machine-learning algorithms KStar and Neural Network were able to distinguish dermoid from epidermoid with accuracies of 76.3% and 69%, respectively. Conclusion: Orbital intradiploic cysts are more commonly epidermoid in origin. Dermoid cysts presented in younger patients; however, there were no other significant differences in features including ophthalmic or radiographic findings. Despite similar features, machine learning was able to identify dermoid versus epidermoid with good accuracy. Future studies may examine the role of machine learning for clinical guidance as well as new surgical options for intervention.

Purpose: To identify risk factors for the development of new-onset, postoperative diplopia following orbital decompression surgery based on patient demographics, clinical exam characteristics, radiographic parameters, and surgical techniques. Methods: We conducted a multi-center retrospective chart review of patients who underwent orbital decompression for thyroid eye disease (TED). Patient demographics, including age, gender, smoking history, preoperative exophthalmometry, clinical activity score (CAS), use of peribulbar and/or systemic steroids, and type of orbital decompression were reviewed. Postoperative diplopia was determined at a minimum of 3 months postoperatively and before any further surgeries. Cross-sectional area ratios of each extraocular muscle to orbit and total fat to orbit were calculated from coronal imaging in a standard fashion. All measurements were carried out using PACS imaging software. Multivariable logistic regression modeling was performed using Stata 14.2 (StataCorp, College Station, TX). Results: A total of 331 patients without preoperative diplopia were identified. At 3 months postoperatively, 249 patients had no diplopia whereas 82 patients developed diplopia. The average postoperative follow-up was 22 months (range 3–156) months. Significant preoperative clinical risk factors for postoperative diplopia included older age at surgery, proptosis, use of peribulbar or systemic steroids, elevated clinical activity score, and presence of preoperative compressive optic neuropathy. Imaging findings of enlarged cross-sectional areas of each rectus muscle to the overall orbital area also conferred a significant risk of postoperative diplopia. Regarding surgical factors, postoperative diplopia was more common among those undergoing medial wall decompression, bilateral orbital surgery, and balanced decompression, whereas endoscopic medial wall decompression was found to be relatively protective. Conclusions: This study identifies risk factors associated with the development of diplopia following orbital decompression using multivariable data. This study demonstrates that several characteristics including age, clinical activity score, the cross-sectional muscle to orbit ratios, in addition to the type of orbital decompression surgery, are predictive factors for the development of new-onset postoperative diplopia.

Purpose: Thyroid eye disease (TED) or Graves’ orbitopathy starts with an active inflammatory stage (active disease) followed by resolution of inflammation and progression to a fibrotic, inactive stage. In our practice, we have encountered cases that have not had active disease despite presence of fibrotic sequelae and disease progression. We aim to delineate the clinical characteristic of this unique group of patients. Methods: We conducted a single-center (Mayo Clinic Rochester) retrospective chart review of TED patients who throughout the course of their disease had only evidence for inactive TED, defined as clinical activity score <3. Results: Median age in the cohort (n = 19) was 54 years (IQR 47–61). 58% had a prior diagnosis of Graves’ disease (GD) before referral. 80% (n = 15) were euthyroid at the time of TED onset (median thyroid-stimulating hormone 1.7 mIU/L). The most common finding was diplopia (100%, n = 19) followed by proptosis (63%, n = 12). Interestingly the disease was asymmetric in 42% of cases. Overall median clinical activity score on presentation was 1 (IQR 0-1). Severity wise, 85% (n = 16) of patients were classified as moderate-to-severe during follow up. Orbital decompression was performed in only 1 case, while extraocular muscle surgery was performed in 13 cases. Conclusions: Quiet TED is a subgroup of TED patients that defies the classic disease paradigm. It presents primarily with diplopia and proptosis. Further evaluation of this group might identify useful insights in TED pathophysiology and help optimize therapeutic choices.

Abstract Objective This study was aimed to illustrate the features and complexities of nonspecific orbital inflammation via discussion of two representative cases. Design Present study is a retrospective case review. Setting The study was conducted at a tertiary care medical center. Participants Two patients with nonspecific orbital inflammation were participants of this retrospective study. Main Outcome Measures Outcome of the study was disease-free patients and off all medications. Results At follow-up, both patients are disease free and off all medications. Conclusion Surgery plays a diagnostic and therapeutic role. While the clinical subtype is important for differential diagnosis and symptomatic treatment, the histologic subtype is similarly important. For inflammatory dacryoadenitis, surgery can be therapeutic. For extensive granulomatosis with polyangiitis, debulking surgery may allow better penetration of medications, especially rituximab.

Purpose: Well-differentiated neuroendocrine or carcinoid tumors are found most commonly in the gastrointestinal tract. When metastatic to the orbit, they tend to have a propensity for the extraocular muscles. The purpose of this study was to better understand the diversity in presentation of orbital carcinoid disease and to determine predictors for survival. Methods: In this observational cross-sectional cohort study, data from 8 tertiary orbital practices were compiled. Demographic, clinical, pathologic, American Joint Committee on Cancer stage and grade, imaging, and management data were extracted for all the patients. Descriptive statistics were calculated. Subgroups were compared utilizing analysis of variance analyses and Kaplan–Meier curves. Time to progression and disease-specific and overall mortality were calculated. Comparisons were performed for the following a priori pairs: unknown versus known primary tumor, single versus multiple extraocular muscle involvement, unilateral versus bilateral orbital disease, extraocular muscle versus other orbital involvement, and excisional versus incisional surgery. Results: A total of 28 patients with carcinoid tumors of the orbit were identified. Of these, 57.1% of patients were female, the mean age at diagnosis of the primary tumor was 58.8 years and the mean age at diagnosis of orbital disease was 62.6 years. At primary presentation, all patients were American Joint Committee on Cancer stage III or IV and 21.4% demonstrated carcinoid syndrome. Muscle involvement was noted in 78.6% of patients, and of these, 72% were noted to have single muscle disease. Eight patients had no primary tumor identified; 3 of these 8 demonstrated disseminated disease at the time of diagnosis. The overall 5-year survival rate was 81.8% from diagnosis of primary tumor and 50% from diagnosis of orbital disease. Subgroup analysis revealed that patients with unilateral orbital disease when compared with bilateral orbital disease had a longer progression-free survival and time to death from all causes ( p = 0.025). Patients with disease localized to the orbit at presentation had longer time to death than those with disseminated disease. Treatment with surgery, radiation, or octreotide did not appear to affect survival. Patients managed with systemic chemotherapy had a shorter time of survival than the rest of the group. All other subgroup comparisons were not found to be statistically significant. Conclusions: Neuroendocrine tumors of the orbit represent a wide spectrum of disease, with some cases being part of disseminated disease, while others being localized presentations. This heterogeneity may be responsible for the slightly higher overall survival in these patients than others with metastatic carcinoid tumors in other locations.

Purpose: To demonstrate the utility of an ultrasonic aspirator (Sonopet, Stryker Corporation; Kalamazoo, MI) for debulking firm, soft tissue masses of the orbit. Methods: Case series. The ultrasonic aspirator was used to debulk firm, soft tissue masses in 3 cases. The initial patient had a large orbitofacial mass extending to the inferior and lateral orbital apex secondary to IgG4 disease. The second patient had a lacrimal sac adenocarcinoma extending to the medial orbital apex. The third patient had a large orbital mass extending to the apex secondary to granulomatosis with polyangiitis. Results: The ultrasonic aspirator facilitated debulking of infiltrative firm soft tissue masses of the orbit. The device’s ability to emulsify, irrigate, and aspirate, along with its small footprint, facilitated precise sculpting and debulking to an extent which would have been difficult otherwise due to location. Conclusions: The ultrasonic aspirator allows precise sculpting of infiltrative firm soft tissue masses in the orbit and is particularly useful in cases with challenging anatomical access.