Primary Leiomyosarcoma of the Orbit and Conjunctiva: A Report of Four Cases With a Review of the Literature.

Leiomyosarcomas (LMS) are common soft tissue tumors in the body. Primary orbital and conjunctival LMS are, however, rare. Herein, we describe the diverse clinical presentations, histopathological features, and management outcomes of three cases of primary LMS of the conjunctiva and one case of primary orbital LMS. The first patient was a 40-year-old female with primary orbital LMS who developed recurrence following wide local excision. The remaining three cases were primary conjunctival LMS. All four patients underwent orbital exenteration and were disease-free at a mean follow-up period of 18.64 months. LMS is known for local recurrences and metastasis. Complete surgical excision and prompt adjuvant radiotherapy can improve the prognosis.

A rare case of lacrimal sac dirofilariasis confirmed by polymerase chain reaction.

A 69-year-old female presented with complaints of persistent watering in the left eye for the past 8 years. She underwent a left external dacryocystorhinostomy (DCR) 8 years ago. On examination, left lacrimal passage irrigation showed regurgitation of fluid from opposite punctum. She underwent a revision external DCR with bicanalicular intubation and a portion of fibrosed sac was sent for histopathological examination (HPE). HPE showed a Microfilarial worm with surrounding granulomatous inflammation. Polymerase chain reaction (PCR) on the DNA extracted from the specimen identified the species to be Dirofilaria repens. Dirofilariasis is an accidental zoonotic infection in humans. Due to its migration, the worm can affect ocular and periocular structures. There are two case reports of perilacrimal dirofilariasis. This is the first case to report a filarial worm in the lacrimal sac causing chronic inflammation which probably led to failure of DCR. Treatment of choice is the complete eradication of the worm. PCR aids in the identification of species.

Graves' Eye Disease: Clinical and Radiological Diagnosis.

Graves' disease is an autoimmune disorder in which hyperthyroidism results in various systematic symptoms, with about 30% of patients presenting with Graves' eye disease (GED). The majority of patients with GED develop mild symptoms, including eyelid retraction, exposure of the globe, superior rectus-levator muscle complex inflammation, and fat expansion, leading to exophthalmos. More severe cases can result in extraocular muscle enlargement, restricted ocular movement, eyelid and conjunctival edema, and compression of the optic nerve leading to compressive optic neuropathy (CON). GED severity can be classified using the Clinical Activity Score, European Group on Graves' Orbitopathy scale, NO SPECS Classification system, and VISA system. CT and MRI aid in the diagnosis of GED through the demonstration of orbital pathology. Several recent studies have shown that MRI findings correlate with disease severity and can be used to evaluate CON. Mild cases of GED can be self-limiting, and patients often recover spontaneously within 2-5 years. When medical treatment is required, immunomodulators or radiotherapy can be used to limit immunologic damage. Surgery may be needed to improve patient comfort, preserve the orbit, and prevent vision loss from optic nerve compression or breakdown of the cornea.

Management of Marcus Gunn jaw wink syndrome with tarsofrontalis sling vis a vis levator excision and frontalis sling: a comparative study.

PURPOSE: To compare the results between tarsofrontalis sling (TFS) and levator palpebrae superioris (LPS) excision with TFS in cases of ptosis with Marcus Gunn jaw winking syndrome (MGJWS). METHOD: A retrospective review of records of all patients undergoing either TFS (group A) or LPS excision along with TFS (group B) in cases of ptosis with MGJWS was done over the past 10 years and their results were compared. RESULTS: The study included 73 patients (75 eyes). There were 36 patients (36 eyes) in group A and 37 patients (39 eyes) in group B. There was a significant reduction in the excursion due to MGJWS from 4.7 ± 1.49 to 1.91 ± 1.04 mm in group A (p = .001) and 4.65 ± 1.34 to 1.79 ± 0.98 mm in group B (p < .05). The reduction in excursion due to MGJWS and lagophthalmos were comparable in both groups. The mean follow-up in group A was 5.62 ± 6.94 (Range: 1-24) months and group B was 19.15 ± 29.16 (Range: 1-96) months (p = .01). CONCLUSION: Both TFS and LPS excision with TFS is equally effective in dampening the eyelid excursion in MGJWS. Tarsofrontalis sling is a safe, easy and effective technique for MGJWS with lesser complication.

Sporadic Burkitt Lymphoma Involving the Orbit - A Report of Two Cases and Review of Literature.

Burkitt lymphoma (BL) is an aggressive, rapidly growing B-cell non-Hodgkin lymphoma found predominantly in children and has three clinical subtypes. The sporadic subtype, seen in non-endemic areas, typically presents as an abdominal mass. Primary orbital involvement is rarely reported. We report two cases of sporadic orbital BL manifesting as unilateral rapidly progressive proptosis with orbit being the initial site of presentation. Following an incision biopsy, BL was confirmed on histopathology and immunohistochemistry. Both patients demonstrated a remarkable improvement with systemic chemotherapy. Burkitt lymphomas grow rapidly with the potential for vision loss. Albeit rare, clinicians should be aware of this entity as timely diagnosis and initiation with chemotherapy display a dramatic response.

Intermittent Manual Carotid Compression as a Treatment Option for Indirect Low Flow Carotid Cavernous Fistula.

PURPOSE: To evaluate the efficacy of intermittent manual carotid compression (IMCC) in the management of low-flow carotid cavernous fistulae (CCF). MATERIALS AND METHODS: Patients diagnosed with low-flow CCF and treated with IMCC over a period of 13 years were retrospectively analyzed. Data analyzed included demographic details, clinical features, type of CCF, and response to therapy. Outcomes were categorized as complete cure, partial cure, and no cure or worsening. Patients with complete and partial cure were grouped as good outcome whereas those with no cure or worsening as poor outcome. RESULTS: A total of 44 patients were advised IMCC for low-flow CCF, of whom five were lost to follow-up. Results of the remaining 39 patients were analyzed, of whom 21 (53.8%) were males. The mean age at presentation was 54.38 ± 14.54 years. The median duration between the onset of symptoms and presentation was 5.0 ± 4.10 months. The common presenting features were episcleral congestion and proptosis (89.7%), extraocular motility restriction (66.7%), and diplopia (48.7%). Most common CCF type was type D (34, 87.2%). All patients were advised to undergo IMCC. Good outcome was seen in 35 patients (89.7%, 95% CI: 80.2% to 99.2%), whereas poor outcome was seen in four (10.3%) patients. The mean duration of follow-up was 15.91 ± 21.87 months. CONCLUSION: The present study found a good outcome with IMCC in 89.7% cases of indirect low-flow CCF with no major complications. It should be considered the management of choice in patients who are systemically unfit or cannot afford endovascular embolization.

A Rare Fungal Orbital Infection in an Immunocompetent Young Male Caused by Lichtheimia corymbifera (Absidia corymbifera).

AIM: To present a case of orbital fungal infection caused by a rare fungus, Lichtheimia corymbifera (Absidia corymbifera) in an immunocompetent individual. MATERIALS AND METHODS: A retrospective case study. RESULT: A 23-year-old male presented with painful proptosis of the right eye for 3 months. Examination revealed normal vision and pupillary light reflex but restricted ocular movements in the right eye. A tender, firm mass was palpable along the inferomedial quadrant of the right orbit. He had acute worsening of proptosis with loss of light perception within 24 hours. Magnetic resonance imaging (MRI) showed a heterogeneously enhancing lesion in the right orbit. Urgent incisional biopsy revealed the growth of Absidial fungal infection. He received intravenous Amphotericin B for 2 weeks with no response. Repeat MRI revealed an extension of the infection up to the cavernous sinus and intracranial optic nerve. He was managed by subtotal exenteration, socket irrigation with Amphotericin B, and intravenous Amphotericin B. CONCLUSION: Invasive orbital fungal infection, though rare, should be considered a differential diagnosis in immunocompetent patients with fulminant proptosis and vision loss.

Peripunctal Mass Lesions: Clinicopathological Analysis and Surgical Outcomes-A Multicentric Series.

PURPOSE: To report the epidemiology, histopathological spectrum, and surgical outcomes of peripunctal mass lesions. METHODS: Multicentric retrospective case series involving 6 centers in India. All cases of peripunctal masses with histopathological diagnoses and minimum follow up of 3 months were included. Demographic data, clinical photographs, objective assessments of clinical improvement were assessed before and after treatment. RESULTS: A total of 50 patients were included. The mean age was 46.4 years (range: 12-76 years). The mean duration of complaints was 27.4 months (range: 1-120 months) and mean follow-up period after surgery was 15.8 months (range: 3-120 months). The most common presenting complaints were a mass lesion/cosmetic concern (82%), followed by epiphora (48%) and foreign body sensation (16%). The most common lesion was melanocytic nevus (19/50; 38%), followed by squamous papilloma (8/50; 16%), hidrocystoma (7/50; 14%), and epidermoid cyst (7/50; 14%). Three cases of malignant tumors were diagnosed: 2 cases of sebaceous gland carcinoma and 1 case of squamous cell carcinoma. In all, 21 of 50 (42%) cases underwent excision with the placement of a Mini-Monoka stent, whereas the remaining 29 cases underwent only excision. At final follow up, a healed punctal opening was visible in 46 of 50 (92%) of the cases; 2 (4%) cases had a slit-like punctum and in 1 case (2%), a stenosed punctum was visible. However, only 1 case (2%) reported epiphora at follow up. CONCLUSIONS: Peripunctal masses are largely benign and present most commonly on the lower eyelid. Melanocytic nevus is the most common peripunctal mass lesion. In the series, stent placement did not play a significant role in the functional outcome.

The spectrum and clinicopathological correlation of eyelid lesions: Twenty years' experience at a tertiary eye care center in South India.

PURPOSE: : To study the epidemiological pattern and diagnostic accuracy of histopathologically proven eyelid lesions over a period of two decades. METHODS: : A retrospective study of all histopathologically proven eyelid lesions from April 1996 to March 2016 was conducted. The lesions were broadly categorized as benign or malignant. Inflammatory and infectious lesions were included under the benign category. The percentage and diagnostic accuracy of each lesion was calculated. RESULTS: : There were a total of 994 (M = 551, F = 443) cases. The mean age of the patients was 43.5 ± 19.9 years. There were 809 (81.4%) benign and 185 (18.6%) malignant lesions. Benign lesions were commonly seen in the fourth decade, while the malignant ones in the late fifth decade. The upper lid was the most common site in both groups (n = 481, 48.4%). The commonest benign lesion was chalazion (n = 484, 59.8%). Dermal nevus (n = 94, 11.6%) was the most common benign neoplasm, while Molluscum contagiosum (n = 25, 3.09%) was the most common infectious lesion. Sebaceous gland carcinoma (SGC) (n = 103, 55.7%) was the leading malignant lesion followed by basal cell carcinoma (n = 39, 21.1%). Eleven malignant cases were misdiagnosed as benign (5.9%). Chalazion (99.1%) and SGC (65%) had the highest diagnostic accuracy, while Molluscum (40%) and squamous cell carcinoma (40%) were the most misdiagnosed lesions in the respective groups. CONCLUSION: : Benign eyelid lesions are far more common than malignant ones. Atypical and rare presentations may lead to misdiagnosis. Knowledge of epidemiological patterns and clinical features can help in achieving higher diagnostic accuracy.

Primary Ewing's sarcoma with orbit involvement: Survival and visual outcomes after eye-sparing multidisciplinary management in eight patients.

BACKGROUND: To describe the clinical presentation, treatment, and overall prognosis in eight patients with primary Ewing's sarcoma (ES) involving the orbit. METHODS: A retrospective interventional study of all biopsy-proven cases of primary ES involving the orbit was done. RESULTS: There were seven males and one female with a median age of 14 years. Imaging showed osseous involvement in all eight cases with extraorbital extension in four. Complete tumor resection was done in four, partial resection in three, and biopsy followed by sinus surgery in one. EWSR1 gene rearrangement analysis was done to confirm diagnosis. All patients received multidrug systemic chemotherapy and seven patients received adjuvant radiotherapy. Eye salvage was achieved in all patients. At a mean follow-up duration of 52.63 months, seven patients were doing well with no evidence of disease. CONCLUSIONS: ES involving the orbit is sensitive to chemotherapy and radiation. Aggressive multimodality treatment can help salvage the globe and improve overall survival.

Role of pre-operative percutaneous embolization in orbital alveolar soft part sarcoma - An experience from a tertiary eye-care center.

PURPOSE: To describe the clinic-radiological, pathological profile, and management outcomes of primary alveolar soft-part sarcoma (ASPS) of the orbit. METHODS: A retrospective analysis of all histopathologically proven cases of orbital ASPS that presented between May 2016 and September 2019 was done. Data collected included demographics, clinical features, imaging characteristics, metastatic workup, management, and follow-up. RESULTS: Five patients, of which four were males, presented to us during the study period. The mean age of presentation was 12.6 years (range 3-22 years). The most common presenting features were abaxial proptosis (n = 4) and diminished vision (n = 4). Imaging showed a well-defined orbital mass in all patients with internal flow voids in three. Preoperative percutaneous embolization with cyanoacrylate glue was done in these three patients owing to high vascularity. Four patients underwent complete tumor excision. One patient underwent exenteration. Histopathology showed polygonal tumor cells arranged in a pseudo-alveolar pattern and Periodic Acid-Schiff (PAS) positive crystals in the cytoplasm in all patients. One patient had systemic metastasis at presentation and developed a local recurrence after 3 months. No recurrence or metastasis was noted in the remaining four patients at a mean final follow-up of 11.2 months (range 5-15 months). CONCLUSION: ASPS is a rare orbital neoplasm that poses a diagnostic and therapeutic challenge. Imaging might show a soft-tissue tumor with high vascularity. Multidisciplinary management with interventional radiologists for preoperative embolization of vascular lesions helps minimize intraoperative bleeding and aids in complete tumor resection. A localized orbital disease carries a better prognosis.

Extrathyroidal Manifestations of Thyroid Disease: Graves Eye Disease.

Graves disease is an autoimmune disorder caused by the breakdown of immune tolerance to thyroid antigens against the TSH receptor. In approximately 25% of patients, an inflammatory condition, Graves eye disease (GED), affects the orbital soft tissues. About 60% of patients develop mild symptoms including fat expansion and inflammation of the levator muscle complex with resultant proptosis, eyelid retraction, and exposure of the globe. The remaining patients experience enlargement of one or more of the extraocular muscles, leading to conjunctival and eyelid edema and congestion, restricted ocular movement with resultant diplopia, and optic nerve compression leading to compressive optic neuropathy.

Orbital and adnexal amyloidosis: Thirty years experience at a tertiary eye care center.

Purpose: The aim of this work was to study the clinical presentation, management and outcomes of orbital and adnexal amyloidosis. Methods: This retrospective analysis included all the patients diagnosed with orbital and adnexal amyloidosis between January 1990 and December 2019. Positive staining with Congo Red and apple-green birefringence on polarized light microscopy established the diagnosis. Data analyzed included demographic profile, varied presentations, management, and outcome. Results: Thirty-three eyes of 26 patients were included. The male:female ratio was 1:1. The mean age of the study population was 42.6 ± 16 years. The median duration of symptoms was two years. Unilateral involvement was seen in 19 eyes (right = 11, left = 8). The most common presenting feature was acquired ptosis. Eyelid was the most commonly affected site followed by orbit and conjunctiva. Two patients had systemic involvement in the form of multiple myeloma and lymphoplasmacytic lymphoma. Complete excision was done in seven (26.9%) cases while 19 (73.1%) cases underwent debulking. Three patients underwent ptosis surgery. The median duration of follow-up was 1.5 years. Three cases had recurrence and underwent repeat surgery. Conclusion: Orbit and adnexa is a rare site for amyloidosis. It is usually localized; however it can occur as a part of systemic amyloidosis. Eyelid is the most common site of involvement and patients usually present as eyelid mass or ptosis. Complete excision is difficult and most of the patients usually undergo debulking surgery. All patients should undergo screening for systemic amyloidosis.

Multidisciplinary Management of Orbital Metastasis and Survival Outcomes.

PURPOSE: To study the multidisciplinary management and survival outcomes of orbital metastasis (OM). METHODS: All patients with a diagnosis of OM treated during 1999-2019 were included. Clinical data were retrospectively collected and analyzed. RESULTS: The study included 118 patients, 71 females and 47 males, with a median age of 61 years. The most common primary tumor types were breast carcinoma (43 patients), melanoma (17), and lung (13), thyroid (7), renal cell (6), and neuroendocrine carcinoma (6). Ninety-six patients had a known history of cancer at OM diagnosis. The median time from diagnosis of primary cancer to OM was 31 months (range, 0-304). In 22 patients, OM was the first sign of cancer. In 47 patients, the orbit was the only site of metastasis. The most common presenting features were restricted by extraocular motility (77 patients) and proptosis (61). Eight patients had enophthalmos. OM was diagnosed based on clinical history and imaging studies in 81 patients and orbital biopsy in 37. One hundred nine patients were treated with chemotherapy and immunotherapy, 75 with radiation, and 21 with palliative surgical resection. Eighty-two patients died during follow up. The median overall survival (OS) time after diagnosis of OM was 17 months (95% CI: 12-28). OM from renal cell carcinoma was associated with the best and OM from thyroid cancer with the worst OS. Patients with breast cancer had longer median survival (28 months; 95% CI: 15-60) than patients with lung, melanoma, neuroendocrine, or thyroid cancer. CONCLUSION: In this large series, breast cancer and melanoma were the most common causes of OM. Most patients had a known history of cancer at OM diagnosis and did not require orbital biopsy to confirm the diagnosis. Patients with renal cell carcinoma and breast carcinoma had the best prognosis after diagnosis of OM.

Role of neuroimaging in cases of primary and secondary hemifacial spasm.

Purpose: The objective of this study was to analyze the role of neuroimaging and documenting various intracranial pathologies in primary and secondary hemifacial spasm. Methods: This retrospective study included patients with HFS who had undergone neuroimaging. The demographic profile, onset, progression, neuroimaging findings, and types of HFS were documented and analyzed. Results: A total of 202 patients (male = 110, female = 92) were included. The mean age of the study population was 51.81 ± 11.76 years. The right side was involved in 104 patients, the left side was involved in 97 patients and bilateral involvement was observed in one patient. Primary HFS: secondary HFS was 9.6:1. The mean age of onset of the spasms in the primary HFS group was 49.26 ± 8.35 years and in secondary HFS was 43.13 ± 12.12 years respectively. The anterior inferior cerebellar artery was the major vessel causing neurovascular conflict in primary HFS (n = 55). Facial nerve palsy was the most common cause (n = 13) of secondary HFS followed by cerebellopontine angle (CPA) tumors. Conclusion: The hemifacial spasm occurs mostly in the fifth decade of life. Primary HFS is more prevalent than secondary HFS. Clinical distinction between them is difficult. Neuroimaging is essential to detect the conflicting vasculature in cases of primary HFS and pathologies like CPA tumor, cyst, and aneurysms in cases of secondary HFS.

Intracanalicular antibiotic ointment loading as a management option for canaliculitis.

AIM: The aim of the study is to describe intracanalicular antibiotic ointment loading as a treatment option for canaliculitis. MATERIAL AND METHODS: It was an interventional prospective study. The study was conducted over a 3 year period from June 2015 to June 2018. All patients of canaliculitis managed with intracanalicular antibiotic ointment loading and with a minimum follow up of 3 months were included in the study. The antibiotic ointment was loaded into the canaliculus based on the antibiotic sensitivity of the material expressed out from the canaliculus. Patients' demographics, clinical details, microbiological profile and management outcomes were evaluated. RESULTS: A total of 24 patients were included in the study. Mean age at presentation was 58.41 ± 9.4 years. The right eye was involved in 15 (62.5%, p = .0424) cases. The incidence of lower canaliculus involvement (14, 58.33%, p = .022) was significantly higher as compared to the upper canaliculus (8, 33.33%). Staphylococcus epidermidis and Actinomyces israelii (6, 25%) were the most commonly isolated organisms. Mean number of intracanalicular antibiotic loading sessions required was 4.21 ± 1.69 (range 2-8). Complete resolution of canaliculitis was seen in all (100%) cases. CONCLUSION: Canalicular antibiotic ointment loading is a minimally invasive, safe, and effective procedure. It preserves the anatomy and function of the punctum and canaliculus and can be considered as one of the management options for canaliculitis.

Superior ophthalmic vein thrombosis post manual carotid compression for indirect carotid-cavernous fistula.

PURPOSE: To report 5 cases of superior ophthalmic vein thrombosis (SOVT) following intermittent manual carotid compression (IMCC) for indirect carotid-cavernous fistula (CCF) and to outline the management. METHODS: Retrospective observational case series of all patients who developed SOVT secondary to IMCC for indirect low flow CCF's at a tertiary care center. The demographic profile, clinical, imaging findings, treatment, and outcomes were studied. RESULTS: The mean age at presentation was 60.2 years (Range: 42-87 years). Four patients were male. All patients had a unilateral presentation. The mean time interval between starting IMCC and the development of SOVT was 1.18 months (Range: 0.25-3 months). Acute exacerbation of proptosis and chemosis associated with a decrease in vision was the presenting feature in all the patients. The mean visual acuity at presentation was 0.89 on the logMAR scale. The causes of reduced visual acuity were venous stasis retinopathy (n = 4) and compressive optic neuropathy (n = 1). Magnetic resonance imaging revealed enlarged superior ophthalmic vein with absent flow voids and post-contrast filling defects. Four patients received anticoagulation treatment with subcutaneous injection of enoxaparin 1 mg/kg twice daily for 5 days followed by oral warfarin 5 mg once daily along with oral steroids. Complete recovery of SOVT was noted in all patients at a mean duration of treatment of 0. 75 months (Range: 0-2 months). CONCLUSION: SOVT is a rare but possible complication in patients on IMCC for indirect CCF, and hence warrants close follow up. Early diagnosis and prompt management will help in preventing complications like permanent vision loss.