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.

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.

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.

Lateral Rectus Muscle Entrapment in Complex Orbital Fracture in a Child.

A 6-year-old boy presented with a lateral rectus muscle entrapment in a complex orbital fracture sustained during bicycle riding. He was diagnosed with a skull-base fracture with pneumocephalus and zygomatic and inferior orbital wall fractures at another hospital. The zygomatic fracture and pneumocephalus were treated conservatively. Although a detailed ocular motility examination could not be performed due to pain and inability to open the eyelid, the inferior wall fracture was surgically repaired. Postoperatively, persistent extraocular movement limitation prompted a referral to our clinic. Orbital CT imaging demonstrated a fracture at the greater wing of the sphenoid with a hypoattenuated area in the adjacent middle cranial fossa. Orbital MRI taken thereafter revealed lateral rectus and orbital fat prolapse into the middle cranial fossa, indicating entrapment of these structures through the lateral wall fracture. The incarcerated lateral rectus was released using an endoscopic transorbital approach via an upper lid crease, resulting in the recovery of extraocular movement.

Efficacy of tocilizumab in patients with moderate-to-severe corticosteroid-resistant thyroid eye disease: a prospective study.

PURPOSE: To evaluate the clinical outcomes of intravenous tocilizumab (TCZ) injection in patients with moderate-to-severe active thyroid eye disease (TED). METHODS: Patients with active and moderate-to-severe TED who did not respond to conventional therapies were treated with TCZ from June 2019 to January 2021. The medical records of the patients were evaluated before the treatment. We analyzed patient demographics, including the duration of Graves' disease and TED, and assessed subjective symptoms, such as diplopia and ocular movement, clinical activity score (CAS), modified NOSPECS score, and exophthalmos before treatment and at 4, 8, 12, and 16 weeks after the first drug injection. Blood tests, including TSH Rc Ab and TS Ab, were performed before treatment and 24 weeks after the first injection. And orbital computed tomography (CT) was performed and Barrett's Index was calculated at baseline and after completion of all injections. RESULTS: Nineteen completed the scheduled treatment. There were no significant side effects, other than herpes zoster in one case and headache and dermatitis in another. Clinical symptoms before and 16 weeks after the treatment showed mean CAS decreased by 2.4 points, mean modified NOSPECS score decreased by 3.7 points, and mean exophthalmos decreased by 0.4 mm. Diplopia and extraocular muscle limitation improved in ten and remained stationary in five of the 15 patients, who presented with extraocular motility abnormalities. Six of 11 patients who underwent orbit CT showed improvement in muscle size. The mean TSH Rc Ab decreased by 7.5 IU/L and TS-Ab decreased by 162.9%. CONCLUSION: TCZ can treat active moderate-to-severe TED, showing high drug compliance and reasonable response to inflammation and extraocular motility abnormality.

Early response to intravenous methylprednisolone therapy for restrictive myopathy in patients with thyroid eye disease.

PURPOSE: To report the therapeutic efficacy of intravenous methylprednisolone (IVMP) in patients with restrictive myopathy caused by thyroid eye disease (TED). METHODS: The present prospective uncontrolled study comprised 28 patients with TED and restrictive myopathy who presented with diplopia that had developed within 6 months before their visit. All patients were treated with IVMP for 12 weeks. Deviation angle, limitation of extraocular muscle (EOM) movement, binocular single vision score, Hess score, clinical activity score (CAS), modified NOSPECS score, exophthalmometric value, and the size of EOMs on computed tomography were evaluated. The patients were divided into two groups: those whose deviation angle had decreased or remained unchanged 6 months after treatment (group 1; n = 17) and those whose deviation angle had increased in that time (group 2; n = 11). RESULTS: The mean CAS of the whole cohort significantly decreased from baseline to 1 month and 3 months after treatment (P = 0.03 and P = 0.02, respectively). The mean deviation angle significantly increased from baseline to 1, 3, and 6 months (P = 0.01, P < 0.01, and P < 0.01, respectively). The deviation angle decreased in 10 (36%), remained constant in seven (25%), and increased in 11 (39%) of the 28 patients. When groups 1 and 2 were compared, no single variable was identified as a cause of deviation angle deterioration (P > 0.05). CONCLUSIONS: When treating patients with TED who have restrictive myopathy, physicians should be aware that some patients show worsening of the strabismus angle despite inflammation control with IVMP therapy. Uncontrolled fibrosis can result in motility deterioration.

Hyaluronic acid gel injection for dysthyroid upper eyelid retraction in Asian patients.

PURPOSE: To evaluate the role of hyaluronic acid (HA) filler injection in the management of upper eyelid retraction (UER) in thyroid eye disease (TED). METHODS: Retrospective interventional series of all patients with TED who had undergone HA injection, from February 2011 to April 2013 by a single surgeon (Y-DK) in a tertiary medical institution. Outcome measures: Mean margin reflex distance 1 (MRD1) pre- and post-filler. RESULTS: There was a total of 13 patients and 17 eyes included in the study. The injection dosage for 15 eyes was 0.5 ml, one patient had 0.35cc on the right eye and 0.65cc on the left eye. Mean MRD1 at presentation was 6.21 mm. There was a mean decrease in MRD1 of 2.01 mm (p < .001) at 1 month post filler, 2.06 mm (p < .001) at 6 months post filler and 2.61 mm (p < .001) at 1 year post filler. There was no correlation between pre-filler MRD1 and change in MRD1 at various time points post-filler, nor any correlation between pre-filler upper scleral show (USS) and change in USS post-filler. There was also no correlation found between clinical activity score (CAS) and change in MRD1, as well as duration of thyroid eye disease (TED) and change in MRD1. Complications included mild upper lid lumpiness on downgaze (n = 4, 23.5%) which improved but persisted with time. There were no untoward intravascular or vision-threatening complications. CONCLUSION: Transconjunctival HA injection is an effective treatment option for TED-related UER in both active and inactive TED patients in an East Asian population, with a potentially long-lasting effect.

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.

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.

Occurrence of ocular injury and orbital fracture in orbital blunt trauma patients at tertiary care center emergency room.

PURPOSE: To analyze the prevalence and association of ocular injury and orbital fracture in orbital trauma patients METHODS: Patients with periocular trauma who visited the emergency room at the referral center from 2014 to 2016 were screened. Patients examined by ophthalmologists and evaluated by CT scan were included. Patients' age, gender, cause of trauma, and injury patterns were retrieved. The location of the fracture and morphologic parameters were reviewed. The patients were divided into groups based on the presence of orbital fracture and/or the presence of ocular injury and clinical data were compared. RESULTS: Two hundred patients were included and 158 presented with fracture. Ocular injuries occurred in 129 of 158 (81.6%) in the fracture group, and in 40 of 42 (95.2%) in the no fracture group; ocular injuries were found more often in the no fracture group (p = 0.031). Open globe injuries occurred in 5 of 158 (3.2%) in the fracture group and in 6 of 42 (14.3%) in the no fracture group; open globe injuries were found more often in the no fracture group (p = 0.012). Patients with ocular injuries showed shorter depth of the orbit (41.9 vs. 44.1 mm; p = 0.003) compared to the patients without ocular injuries. Logistic regression revealed that short orbit was associated with the presence of ocular injury (p = 0.004). CONCLUSION: The incidence of ocular injuries was significantly higher in patients without orbital fracture than in those with fractures of the orbit. The orbital fracture may play a protective role against ocular injury by providing a decompressive effect on the orbital tissue.

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 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.

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.

Fibromyxoma of the Orbit.

Fibromyxomas are rare tumors that are not commonly seen in the orbit. The authors present a case of orbital fibromyxoma. A 42-year-old male presented with proptosis of the right eye that began 1 month prior. Magnetic resonance imaging revealed the presence of an orbital mass in the right eye, with low signal intensity on T1-weighted images and heterogeneous high-signal intensity on T2-weighted images. The patient underwent excisional biopsy of the orbital tumor through a Krönlein approach. The tumor originated from the inferior rectus muscle. Histopathologic analysis demonstrated scattered spindle cells, with both fibrous and myxoid stroma. Immunohistochemical staining was positive for Vimentin and negative for S-100 protein and CD34. The tumor was diagnosed as orbital fibromyxoma. The patient showed no evidence of recurrence over 18 months of follow-up after operation.

Lowering of the High Eyelid Crease in Revision Upper Eyelid Surgery.

BACKGROUND: High placement of the lid crease is a common complication in upper eyelid surgery. Correction of the high crease by revision surgery is challenging and has not been well-reported. METHODS: This is a retrospective interventional study of patients who underwent revision eyelid surgery with lowering of the lid crease from 2008 to 2016 at a tertiary institution. Main outcome measures were pretarsal show (PTS) distance, lid crease symmetry, margin reflex distance 1 (MRD1), and lid height symmetry. Lid crease symmetry was graded as symmetrical, mild asymmetry (PTS difference ≤ 0.5 mm), moderate asymmetry (PTS difference > 0.5 mm but ≤ 1 mm), or obvious asymmetry (PTS difference > 1 mm). Lid height symmetry was graded as symmetrical, mild asymmetry (MRD1 difference ≤ 1 mm), moderate asymmetry (MRD1 difference > 1 mm but ≤ 2 mm), or obvious asymmetry (MRD1 difference > 2 mm). RESULTS: There were a total of 69 patients and 100 eyes. The majority (n = 42, 60.9%) of patients were females. The mean age was 38.3 ± 17.3 years, and mean follow-up was 16 months. Mean PTS decreased from 3.1 mm pre-surgery to 2.0 mm 2 years post-surgery. The proportion of patients with moderate or severe lid crease asymmetry decreased from 81.1% pre-surgery to 6.7% 2 years post-surgery. The mean MRD1 difference decreased from 1.54 mm pre-surgery to 0.23 mm 1 year post-surgery. The proportion of patients with moderate or severe lid height asymmetry improved from 64.5% preoperatively to 4.5% 1 year postoperatively. CONCLUSION: Revision eyelid surgery to correct a high crease is a challenging procedure. We present a technique that is effective in correcting the high lid crease, while simultaneously improving the lid height and lid crease symmetry. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these evidence-based medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Conservatively Treated Orbital Blowout Fractures: Spontaneous Radiologic Improvement.

PURPOSE: To determine if conservatively treated blowout fractures of the orbit undergo spontaneous improvement based on radiologic findings. DESIGN: Prospective, noncomparative series. PARTICIPANTS: Patients with conservatively treated orbital blowout fractures in a single tertiary institution from 2012 through 2016 with initial and follow-up computed tomography (CT) scans. METHODS: Comparison of initial and follow-up CT to assess for smoothening of bony contour, joining of bony edges, reduction in herniation of orbital contents, and new bone formation. Orbital and fracture volumes were calculated using a 3-dimensional reconstruction software program (3D Workstation; TeraRecon, Foster City, CA). MAIN OUTCOME MEASURES: Change in bony contour, new bone formation, and decrease in orbital and fracture volumes. RESULTS: Our study comprised 41 patients and 44 orbits, with 38 unilateral and 3 bilateral cases. Most were men (65.9%; n = 27), and the mean age was 34.3±13.5 years. The mean time from injury to follow-up scan was 4.6 months (range, 1-15 months). All orbits showed changes in bony contour from initial to follow up CT, including smoothening of the orbital contour (88.6%), joining of bony edges (90.9%), and reduction in herniation of orbital contents (65.9%). Most of the orbits (n = 41; 93.2%) showed features of neobone formation. Of the 44 orbits, 91.4% showed a decrease in orbital volume, whereas 94.3% showed a decrease in fracture volume. The reduction in volume was statistically significant for both orbital (from 23.7±4.0 to 21.8±3.9 ml) as well as fracture (from 1.2±0.8 to 0.7±0.6 ml) volumes from initial to follow-up scans, respectively (P < 0.001). CONCLUSIONS: A large proportion of patients showed improvement in radiologic findings despite being treated conservatively. This highlights the spontaneous improvement that can occur in untreated blowout fractures not just clinically, but radiologically, in terms of soft tissue and bony findings.

Superior rectus muscle insertion injury following cosmetic upper lid blepharoplasty: a case report.

BACKGROUND: Direct damage to the superior rectus (SR) muscle insertion following upper lid blepharoplasty has not been reported. We document a rare case of vertical diplopia due to direct damage to the SR muscle insertion following cosmetic upper lid blepharoplasty. CASE PRESENTATION: We describe a case of 24-year-old woman with Asian eyelid. The patient had already undergone multiple cosmetic upper lid surgeries and complained of vertical diplopia immediately after her most recent surgery (levator resection with skin approach). Preoperatively, large-angle right hypotropia and severe upgaze limitation were present and noticeable ptosis was observed in the right eye. Intraoperatively, the SR muscle fibers were observed to be detached at the insertion site and severe fibrosis and adhesion surrounding the muscle was noted. After strabismus surgery, vertical strabismus was improved. CONCLUSIONS: This case can provide valuable insight to surgeons performing ptosis surgery and blepharoplasty, particularly in cases of reoperation. Surgeons should be careful while manipulating the levator muscle or resecting deep tissues not to affect the SR muscle.

Long-Term Outcomes of Eye-Sparing Surgery for Adenoid Cystic Carcinoma of Lacrimal Gland.

PURPOSE: This study's primary purpose is to assess the long-term outcomes of patients who have undergone eye-sparing surgery and adjuvant radiotherapy for adenoid cystic carcinoma of the lacrimal gland. METHODS: In this retrospective analysis, clinical records were reviewed of all patients diagnosed with adenoid cystic carcinoma of the lacrimal gland, at a single institution, between March 1998 and November 2012. Ten patients were identified as having undergone eye-sparing surgery and adjuvant radiotherapy for adenoid cystic carcinoma of the lacrimal gland. Preoperative radiographic findings, treatment modalities, histological results, and patient outcomes were analyzed. RESULTS: There were 6 male and 4 female patients. The patients' tumors were staged according to the 8th American Joint Committee on Cancer staging system, and were as follows: 1 patient was classified as T1aN0M0; 6 patients were classified as T2aN0M0; 1 patient was classified as T2cN0M0; 2 patients were classified as T3aN0M0. All patients had a histologically confirmed diagnosis of lacrimal gland adenoid cystic carcinoma, which was confined to the orbit, and was without extension into adjacent bone marrow or other organs. All patients underwent eye-sparing tumor excision followed by postoperative radiotherapy, with a median dose of 6000 cGy (range: 5000-6600 cGy). At the last follow up, 8 patients were alive without evidence of disease. One patient was deceased at 58 months post-surgery, due to esophageal carcinoma; this was unrelated to the lacrimal gland tumor. The final patient experienced tumor recurrence in the medial orbit 53 months post-surgery, and exenteration was performed. This patient was alive, without disease recurrence, at 90 months following exenteration. The median follow-up time was 89.5 months (range: 37-217 months). Systemic metastasis did not occur in any patient. CONCLUSIONS: Eye-sparing surgery and adjuvant radiotherapy have demonstrated favorable local control and long-term survival outcomes in patients with orbit-confined lacrimal gland adenoid cystic carcinoma. Consequently, eye-sparing surgery with adjuvant radiotherapy can be considered as a viable treatment option for orbit-confined lacrimal gland adenoid cystic carcinoma.

A Case of Lacrimal Sac Septum Presenting With Dacryocystocele.

Lacrimal sac septum is a rare structural abnormality of the lacrimal system. A 15-year-old patient presented with epiphora and a mass inferior to the left medial canthal tendon. Dacryocystography and CT-dacryocystography revealed a dilated lacrimal sac with complete obstruction. A horizontal lacrimal sac septum was found in the mid-sac region during endoscopic dacryocystorhinostomy. Histopathologic examination of the septum revealed chronic inflammation with stratified columnar epithelial cell lining consistent with the lacrimal sac.