A Case of Idiopathic Orbital Inflammation With Elevated Anti-Mumps Immunoglobulin M Antibody.

A 21-year-old female presented with periocular swelling, diplopia, and painful ophthalmoplegia in the OS. Orbital magnetic resonance imaging revealed an enhanced soft tissue mass involving the left medial rectus muscle. Laboratory test results revealed leukocytosis, elevated reactive C-reactive protein, and positive serum levels of anti-mumps immunoglobulin M (IgM) antibody without systemic manifestations of mumps infection. The clinical course was refractory, and the patient showed a poor response to high-dose steroids. An incisional biopsy revealed stromal fibrosis with focal lymphoid aggregates, indicating sclerosing inflammation. Myopathy of the medial rectus progressed to superior, inferior, and lateral recti involvement of the left orbit. Immunosuppressive agents, including steroids, were administered for 22 months after disease onset. The mumps IgM antibody level was positive for over 5 months and became negative upon testing performed 1 year after the first visit.

Correlation Between Radiologic and Histopathologic Features of Orbital Epidermoid and Dermoid Cysts.

PURPOSE: Epidermoid cysts (EC) and dermoid cysts (DC) typically appear as well-circumscribed lesions on CT. This study aimed to clarify the radiologic and histopathologic characteristics of orbital EC and DC and to determine the correlations between them. METHODS: The medical records of 69 patients who underwent surgery for orbital DC or EC at Samsung Medical Center between January 2001 and August 2016 were retrospectively reviewed. The size and location of the cysts, rim enhancement, homogeneity of contents, presence of hemorrhagic or calcific components, radiodensity of contents, and extent of bony remodeling were evaluated using CT. Additionally, the cyst lining and contents were examined histopathologically. RESULTS: Among patients with orbital cysts, EC and DC were diagnosed in 10 (14.5%) and 59 (85.5%) patients, respectively. Further, 50.0% of EC and 79.7% of DC were located in the superotemporal quadrant of the orbit. On orbital CT, the average radiodensity of EC and DC was 18.9 ± 56.2 and -67.9 ± 63.3 HU, respectively. The cystic contents were more frequently homogeneous than heterogeneous in both EC and DC; however, the radiodensity of cysts differed significantly, which may be attributed to sebaceous gland activity. Focal bony notching, bone remodeling under pressure, and bony changes from dumbbell-shaped cysts were observed more frequently in DC than in EC. CONCLUSIONS: Radiological and histopathological features are correlated in orbital EC and DC. Therefore, orbital EC and DC can be preoperatively differentiated using CT, based on the average radiodensity and bony remodeling.

Orbital Liposarcoma Progression From a Well-Differentiated to a Dedifferentiated Type in a Young Patient.

A young male patient presented with unilateral proptosis of the left eye. Laboratory tests and an initial computed tomography scan of the orbit were compatible with euthyroid orbitopathy. He subsequently underwent decompression surgery for unfavorable cosmesis and corneal exposure symptoms with good results, but returned 1 year later for recurrence of proptosis. There was a superomedial fatty density extraconal mass on computed tomography scan. A biopsy done a year later confirmed the diagnosis of well-differentiated liposarcoma from the previously noted mass and a dedifferentiated component in the newly appeared mass in the intraconal space. Orbital exenteration with radial forearm free flap was performed followed by orbital radiotherapy. No disease recurrence or metastasis was seen on follow-ups for 7 years since undergoing treatment.

Orbito-cranial schwannoma-a multicentre experience.

OBJECTIVES: To describe the features, management approaches, and outcomes of orbito-cranial schwannomas. METHODS: Retrospective review of ten patients with orbito-cranial schwannomas managed in six orbital services over 22 years. Data collected included demographics, presenting features, neuroimaging characteristics, histology, management approach, complications, and outcomes. RESULTS: Mean age of the patients was 41.4 ± 19.9 years, and 6 (60%) were females. The majority presented with proptosis (90%), limited extraocular motility (80%), eyelid swelling (60%), and optic neuropathy (60%). Most lesions (80%) involved the entire anterior-posterior span of the orbit, with both intra- and extraconal involvement. All tumours involved the orbital apex, the superior orbital fissure, and extended at least to the cavernous sinus. Surgical resection was performed for all. Seven (70%) of the tumours were completely or subtotally resected combining an intracapsular approach by an orbital-neurosurgical collaboration, with no recurrence on postoperative follow-up (6-186 months). Three underwent tumour debulking. Of these, two remained stable on follow-up (6-34 months) and one showed progression of the residual tumour over 9 years (cellular schwannoma on histology) necessitating stereotactic radiotherapy (SRT) for local control. Adjuncts to the orbito-cranial resection included perioperative frozen section (n = 5), endoscopic transorbital approach (n = 2), and image-guided navigation (n = 1). Post-surgical adjuvant SRT was used in three subjects. CONCLUSIONS: These results highlight the possibility of successful surgical control in complex orbito-cranial schwannomas. A combined neurosurgical/orbital approach with consideration of an intracapsular resection is recommended. Recurrence may not occur with subtotal excision and observation may be reasonable. Adjunctive SRT for progression or residual tumour can be considered.

Combined endoscopic endonasal and transorbital multiportal approach for complex skull base lesions involving multiple compartments.

PURPOSE: This study defines the specific areas that connect the surgical corridors of the endoscopic endonasal (EEA) and transorbital approach (TOA) to identify adequate clinical applications and perspectives of this combined multiportal approach. METHODS: Consecutive patients who underwent combined EEA and TOA procedures for various pathologies involving multiple compartments of the skull base were enrolled. RESULTS: A total of eight patients (2 chondrosarcomas, 2 meningiomas, 2 schwannomas, 1 glioma, and 1 traumatic optic neuropathy) were included between August 2016 and April 2021. The cavernous sinus (CS) was targeted as the connection area of the combined approach in four patients with tumors infiltrating the middle cranial fossa (MCF) and central skull base through the CS. For two patients with MCF tumors extending into the infratemporal fossa (ITF), the horizontal portion of the greater sphenoid wing and the foramen ovale were utilized as the connection area. In the remaining 2 patients, connection was achieved through the optic canal (OC). Gross total and near total resection was achieved in 5 patients with tumors, and circumferential removal of bone composing the OC was performed in one patient with traumatic compressive optic neuropathy. Postoperative complications included one cardiac arrest due to underlying cardiovascular disease and one case of oculomotor nerve palsy. CONCLUSIONS: The combined EEA and TOA procedure is a useful strategy for complex lesions involving multiple compartments of the skull base. Herein, we identified the specific areas connecting the two surgical approaches, allowing a common path for EEA and TOA procedures.

Endoscopic endonasal and transorbital approaches to petrous apex lesions.

OBJECTIVE: The petrous apex (PA) is one of the most challenging areas in skull base surgery because it is surrounded by numerous critical neurovascular structures. The authors analyzed the clinical outcomes of patients who underwent endoscopic endonasal approach (EEA) and transorbital approach (TOA) procedures for lesions involving PA to determine the perspectives and proper applications of these two approaches. METHODS: The authors included patients younger than 80 years with lesions involving PA who were treated between May 2015 and December 2019 and had regular follow-up MR images available for analysis. Patients with meningioma involving petroclival regions were excluded. The authors classified PA into three regions: superior to the petrous segment of the internal carotid artery (p-ICA) (zone 1); posterior to p-ICA (zone 2); and inferior to p-ICA (zone 3). Demographic data, preoperative clinical and radiological findings, surgical outcomes, and morbidities were reviewed. RESULTS: A total of 19 patients with lesions involving PA were included. Ten patients had malignant tumor (chondrosarcoma, chordoma, and osteosarcoma), and 6 had benign tumor (schwannoma, Cushing's disease, teratoma, etc.). Three patients had PA cephalocele (PAC). Thirteen patients underwent EEA, and 5 underwent TOA. Simultaneous combined EEA and TOA was performed on 1 patient. Thirteen of 16 patients (81.3%) had gross- or near-total resection. Tumors within PA were completely resected from 13 of 16 patients using a view limited to only the PA. Complete obliteration of PAC was achieved in all patients. Postoperative complications included 2 cases of CSF leak, 1 case of injury to ICA, 1 fatality due to sudden herniation of the brainstem, and 1 case of postoperative diplopia. CONCLUSIONS: EEA is a versatile surgical approach for lesions involving all three zones of PA. Clival tumor spreading to PA in a medial-to-lateral direction is a good indication for EEA. TOA provided a direct surgical corridor to the superior portion of PA (zone 1). Patients with disease with cystic nature are good candidates for TOA. TOA may be a reasonable alternative surgical treatment for select pathologies involving PA.

Clinical Outcomes of IgG4-Related Ophthalmic Disease and Idiopathic Sclerosing Orbital Inflammation.

PURPOSE: To compare the clinical features, treatment outcomes, and prognoses of immunoglobulin G4-related ophthalmic disease (IgG4-ROD) and idiopathic sclerosing orbital inflammation (ISOI). METHODS: This retrospective case series included 40 and 22 biopsy-proven cases of IgG4-ROD and ISOI, respectively. The authors reviewed medical charts and images to determine the clinical presentation, involvement pattern, and treatment outcomes. The main outcome measures were differences in disease manifestation, treatment modalities and efficacy, medication-free remission rate, and predictive factors for remission. RESULTS: The median ages were 51 (range 31-72) and 39 (range 9-73) years in the IgG4-ROD and ISOI groups, respectively. The most frequently involved sites were the lacrimal gland (39 cases, 98%) in IgG4-ROD patients and the extraconal region (15 cases, 68%) in ISOI patients. No significant difference was observed in the initial treatment response between the groups. Medication-free remission was achieved in 22 (55%) IgG4-ROD patients and 10 (45%) ISOI patients; none of the IgG4-ROD patients showed permanent ocular dysfunction, while half of the ISOI patients had permanent visual loss or limited ocular motilities. Multivariate analysis indicated that extraocular muscle enlargement (odds ratio, 0.11; 95% confidence interval, 0.01-0.98) and IgG4:IgG ratio on histopathology (odds ratio, 0.08; 95% confidence interval, 0.01-0.86) were negatively associated with medication-free remission in IgG4-ROD patients. CONCLUSIONS: Although IgG4-ROD and ISOI share common histological findings of mass-forming sclerosis, IgG4-ROD frequently has a more indolent clinical course, whereas ISOI often demonstrates a more aggressive clinical course, resulting in ocular dysfunction even after remission. Different pathogeneses may account for the different disease characteristics.

Surgical Risk Prediction for Nasolacrimal Duct Obstruction in Radioactive Iodine-Treated Thyroid Cancer: A Nationwide Cohort Study.

Background: To estimate the incident risk of surgical intervention for nasolacrimal duct obstruction after radioactive iodine (RAI) treatment and elucidate the high-risk group of patients with thyroid cancer (TC). Methods: This is a nationwide, population-based cohort study using data from the Korean National Health Claims database. Among the entire South Korean population aged between 20 and 80 years from 2007 to 2019, all patients with TC were included. Among all TC patients, a cohort was established by classifying it into the RAI and non-RAI groups according to whether RAI treatment was performed during the recruitment period. A log-rank analysis was used to evaluate the risk of nasolacrimal duct obstruction surgery in the RAI group compared with that in the non-RAI group. The Cox proportional hazards regression analysis was performed to calculate the adjusted hazard ratio (HR). The analysis was performed stratified by the age group, sex, frequency of RAI treatment, and cumulative dose of 131I. Results: Among 55,371 TC patients, who received RAI treatment, 515 (0.93%) underwent nasolacrimal duct obstruction surgeries, and among 56,950 TC patients, who did not receive RAI treatment, 72 (0.13%) underwent the surgeries. The average timing of incident nasolacrimal duct obstruction surgery was 3.8 ± 2.1 years after the first exposure to RAI. The incidence rate of nasolacrimal duct obstruction surgery was 104.04/100,000 person-years in the RAI group. There was a significantly higher risk of nasolacrimal duct obstruction surgery (HR 7.50 [confidence interval, CI: 5.86-9.60], p < 0.001) in the RAI group than in the non-RAI group. The risks significantly increased in the high-dose group (HR 14.15 [CI: 10.88-18.39]) and those younger than 50 years (HR 15.54 [CI: 9.76-24.72]). Conclusion: We quantified the risk of RAI-induced nasolacrimal duct obstruction surgery through a long-term follow-up of 9 years, which may contribute to estimating the general health care burden related to RAI treatment for TC patients in a practical setting. Great attention should be paid to its risk in patients younger than 50 years, especially in those treated with high doses.

Prognostic factors of restrictive myopathy in thyroid eye disease.

To investigate the prognostic factors of extraocular muscle restriction in patients with thyroid eye disease (TED), 65 patients with TED and restrictive myopathy were evaluated. Demographics, clinical activity score (CAS), smoking status, thyroid disease status, thyroid hormone status, thyroid autoantibody status, orbital computed tomography (CT) scan at initial presentation, and treatment regimens were assessed. The movements of the most severely affected extraocular muscles were categorized into five grades. The patients were divided into the improved and the not-improved group based on the improvement in the limitation of the extraocular muscle excursion (LOM) throughout the follow-up, and the groups were compared using clinical factors. The mean LOM significantly improved from 2.3 ± 1.1 to 1.7 ± 1.2 after 1 year of follow-up. The excursion of the most restricted muscle improved in 32 patients but not in 33 patients during the follow-up. The initial concentration of the thyroid-stimulating antibody (TSAb) was significantly lower in the improved (229.3 ± 114.1) than in the not-improved group (345.0 ± 178.6) (P = 0.02) Age, sex, smoking status, CAS, thyroid status, and muscle thickness on the CT scan did not significantly differ in the groups. This study showed that the initial concentration of TSAb is a factor affecting the recovery of restrictive myopathy.

Clinical characteristics and treatment outcomes of diffuse large B-cell lymphoma involving the eye and ocular adnexa in East Asia.

PURPOSE: To investigate the clinical features and outcomes of diffuse large B-cell lymphoma (DLBCL) involving the eye and ocular adnexa in an East Asian population and emphasize the importance of prompt biopsy and treatment in unusually progressive ocular adnexal DLBCL for better visual outcomes. METHODS: Retrospective case series of 38 patients diagnosed with DLBCL involving the eye and ocular adnexa between 1995 and 2018 at a single tertiary institution. RESULTS: There were 38 patients (22 men, 16 women), 22 (57.9%) of which had ocular adnexal lymphoma (OAL) and 16 (42.1%), vitreoretinal lymphoma (VRL). The OAL group showed higher rates of unilateral involvement (p = 0.01) and localized disease presentation (p = 0.01). Thirteen (59.1%) of 22 OAL patients were primary diseases and 9 (40.9%) were secondary. Between primary and secondary OAL groups, there was no statistical difference in clinical characteristics such as age, gender and laterality (p = 0.47, p = 0.19 and p = 0.66, respectively). All secondary OAL cases were either disseminated or relapsed by definition and only two cases (15%) of primary OAL showed disseminated presentation (p < 0.01). Six (37.3%) of 22 OAL patients showed a rapid decrease in visual acuity within 15 days and the visual recovery was irreversible in 3 of them. CONCLUSION: OA-DLBCL has a grave prognosis and its visual outcomes largely depend on the timing of diagnosis and treatment. When a high-grade lymphoma is suspected, prompt biopsy and treatment should be performed to ensure optimal visual, clinical and survival outcomes.

Lagophthalmos after congenital ptosis surgery: comparison between maximal levator resection and frontalis sling operation.

BACKGROUND/OBJECTIVES: To compare postoperative lagophthalmos after maximal levator resection (MLR) and frontalis suspension (FS) in congenital ptosis patients with poor levator function (LF). METHODS: A cross-sectional study was performed to compare postoperative outcomes in patients with preoperative LF ≤ 4 mm who had undergone MLR or FS at a single tertiary institution, and who had visited the outpatient clinic between February 2017 and August 2018. Main outcome measures were as follows: (1) Preoperative LF and margin reflex distance 1 (MRD1), (2) Postoperative MRD1, lagophthalmos and grade of superficial punctate keratopathy (SPK). RESULTS: Our study comprised 152 eyelids of 122 patients. There were 71 eyelids in the MLR group and 81 eyelids in the FS group. The MLR group had comparable mean postoperative MRD1 (2.8 ± 0.8 mm) to the FS with autogenous fascia lata (AFL) group (3.0 ± 0.7 mm), while the FS with preserved fascia lata (PFL) group had the lowest mean postoperative MRD1 (2.2 ± 1.0 mm). The PFL group had significantly less lagophthalmos (0.6 ± 1.0 mm) than the AFL (1.9 ± 1.4 mm) and maximal levator resection (1.9 ± 1.7 mm) groups. In the MLR group, there was no significant difference in postoperative surgical measurements between the LF 0-2 mm group and LF 2.5-4 mm group in terms of exposure keratopathy, degree of lagophthalmos and MRD1. CONCLUSION: MLR is an effective alternative to FS in congenital ptosis patients with poor levator function, with the risk of postoperative lagophthalmos related to postoperative MRD1 rather than preoperative LF.

Stereotactic radiosurgery for orbital cavernous venous malformation: a single center's experience for 15 years.

BACKGROUND: Stereotactic radiosurgery such as Gamma Knife radiosurgery (GKRS) has been shown to have a good treatment effect for orbital cavernous venous malformation (CVM). However, radiation-induced retinopathy or optic neuropathy is a vision-threatening complication of orbital irradiation. Predicting the post-treatment visual outcome is critical. METHODS: Clinical and radiological outcomes were investigated in 30 patients who underwent GKRS for orbital CVM between July 2005 and February 2020. Measurement of peripapillary retinal nerve fiber layer (pRNFL) thickness using optical coherence tomography (OCT) was obtained in 14 patients. RESULTS: The median clinical and radiological follow-up periods were 46.6 months (range, 15.9-105.8) and 27.5 months (range, 15.4-105.8), respectively. Twenty-eight patients underwent multisession (4 fractions) GKRS. The median cumulative marginal dose was 20 Gy (range, 16-24). Two patients underwent single-session GKRS. Marginal doses were 15 Gy and 10.5 Gy in each patient. The volume of CVM decreased in 29 (97%) patients. Visual acuity was improved in 6 (20%) patients and was stable in 22 (73%) patients. Visual field defect and exophthalmos were improved in all patients. Serial investigation of OCT showed no statistically significant difference in pRNFL thickness after GKRS. Patients with normal average pRNFL thickness showed better visual recovery than patients with thin average pRNFL thickness. CONCLUSIONS: GKRS is an effective and safe treatment option for orbital CVM. The pRNFL thickness before GKRS can be a prognostic indicator for visual recovery in orbital CVM after GKRS.

Use of endoscopic transorbital and endonasal approaches for 360° circumferential access to orbital tumors.

OBJECTIVE: Orbital tumors are often surgically challenging because they require an extensive fronto-temporo-orbital zygomatic approach (FTOZ) and a multidisciplinary team approach to provide the best outcomes. Recently, minimally invasive endoscopic techniques via a transorbital superior eyelid approach (ETOA) or endoscopic endonasal approach (EEA) have been proposed as viable alternatives to transcranial approaches for orbital tumors. In this study, the authors investigated the feasibility of 360° circumferential access to orbital tumors via both ETOA and EEA. METHODS: Between April 2014 and June 2019, 16 patients with orbital tumors underwent either ETOA or EEA at the authors' institution. Based on the neuro-topographic "four-zone model" of the orbit with its tumor epicenter around the optic nerve in the coronal plane, ETOA (n = 10, 62.5%) was performed for tumors located predominantly superolateral to the nerve and EEA (n = 6, 37.5%) for those located predominantly inferomedial to the nerve. Eight patients (50%) presented with intraconal tumors and 8 (50%) with extraconal ones. The orbital tumors included orbital schwannoma (n = 6), cavernous hemangioma (n = 2), olfactory groove meningioma (n = 1), sphenoorbital meningioma (n = 1), chondrosarcoma (n = 1), trigeminal schwannoma (n = 1), metastatic osteosarcoma (n = 1), mature cystic teratoma (n = 1), sebaceous carcinoma (n = 1), and ethmoid sinus osteoma (n = 1). The clinical outcomes and details of surgical techniques were reviewed. RESULTS: Gross-total resection was achieved in 12 patients (75%), near-total resection in 3 (18.8%), and subtotal resection in 1 (6.2%). Eight (88.9%) of the 9 patients with preoperative proptosis showed improvement after surgery, and 4 (66.7%) of the 6 patients with visual symptoms demonstrated improvement. Four (40%) of the 10 patients treated with ETOA experienced partial third nerve palsy immediately after surgery (3 transient and 1 persistent). There have been no postoperative CSF leaks or infections in this series. CONCLUSIONS: Without transcranial approaches requiring temporalis muscle dissection and orbitozygomatic osteotomy, the selection of ETOA or EEA based on a concept of a four-zone model with its epicenter around the optic nerve successfully provides a minimally invasive 360° circumferential access to the entire orbit with acceptable morbidity.

Pilosebaceous Malignant Transformation of Dermoid Cyst in the Orbit.

Dermoid cysts may occur in the orbit, most commonly in the superolateral area. Malignant transformation of such lesions has been previously reported; however, most turn out to be squamous cell carcinoma. The authors' patient initially presented with mild proptosis and limitation in extraocular movements. Preliminary biopsy showed whitish amorphous material and abundant hairs filling the thin-walled cyst, consistent with dermoid cyst. The patient underwent tumor resection via lateral orbitotomy with bone window and transorbital endoscopic approach for the dural involvement. Final biopsy showed dermoid cyst with pilosebaceous malignant transformation showing p53 expression and 30% of Ki-67 index. Adjuvant radiotherapy was performed. To the best of the authors' knowledge, this is the first reported case of this type. Despite its rarity, there should always be a high index of suspicion and complete work-up for an accurate diagnosis.

Orbital Actinomyces Masquerading as Meningioma.

Orbital actinomyces is a rare diagnosis with only a few cases reported in the literature. It can be difficult to diagnose due to its slow, indolent course, and nonspecific findings on imaging and clinical examination, and frequently it can masquerade as other pathologies such as neoplasm and inflammatory disease. The authors present a case of actinomyces masquerading as meningioma with findings of hyperostosis and a superior orbital roof interosseous tract on imaging.

Use of Meshed Acellular Dermal Allograft as a Lining Material After Orbital Exenteration.

PURPOSE: To evaluate the surgical outcome and safety of acellular human dermal allograft as a new lining material to the exposed orbit after exenteration. METHODS: Retrospective case series of patients who underwent orbital exenteration followed by reconstruction with meshed-type acellular dermal allograft from 2009 to 2018 in a single tertiary institution. RESULTS: There were 14 eyes (2 right, 12 left) of 14 patients (6 men, 8 women). Mean age at operation was 69.1 ± 16.5 years. Indication for surgery was malignancy in all patients. One patient underwent subtotal exenteration, while the rest underwent total exenteration. SureDerm Meshed was used in 12 patients, AlloDerm Meshed in 1, and CGDerm Meshed in 1. Mean follow-up period was 12.1 months. Full or nearly full epithelialization occurred in 10 of 14 patients (71.4%) at 1 month and 9 of 12 patients (75.0%) at 3 months. There was delayed epithelialization in 3 patients due to poor wound care (n = 1), adjuvant radiotherapy (n = 1), and adjuvant radiotherapy followed by cerebrospinal fluid leak (n = 1). CONCLUSIONS: Meshed acellular human dermal allograft showed good success in reconstruction after orbital exenteration and may be considered as an alternative lining material to split-thickness skin graft after orbital exenteration.