Oculomotor nerve schwannoma associated with ophthalmoplegic migraine

PURPOSE: To describe a patient with an oculomotor nerve schwannoma who had symptoms of ophthalmoplegic migraine. METHODS: Case report. RESULTS: A 23-year-old woman had a history of recurrent headache accompanied by transient right oculomotor palsy since age 7 years. Ophthalmoplegic migraine was diagnosed. She was subsequently found to have a structural lesion of her right oculomotor nerve on magnetic resonance imaging. The magnetic resonance image characteristics were consistent with schwannoma originating from the oculomotor nerve. CONCLUSIONS: This case illustrates that an intrinsic lesion of the oculomotor nerve (schwannoma) may be associated with a painful relapsing-remitting oculomotor palsy mimicking the clinical syndrome of ophthalmoplegic migraine.     

Eyelash alopecia areata: case series and literature review

ObjectivesTo characterize the clinical presentations, features, and outcomes of eyelash alopecia areata.DesignRetrospective chart review of patients evaluated for eyelash loss and found to have eyelash alopecia areata. A 3-year follow-up was required.ParticipantsThe study involved 15 patients.MethodsPatients who presented at a tertiary care eye clinic for evaluation of eyelash loss and were found to have eyelash alopecia areata were reviewed. Demographic considerations were categorized; they included age at presentation, areas of other hair loss, other relevant history, and treatment responses.ResultsPatients were young (mean age 18 years) and had a large female predominance (female-to-male ratio, 14:1). Concurrent scalp and brow involvement, either in isolation (20% and 13%, respectively) or together (13%), were seen in half of the patients (46%). Upper eyelid involvement was more common and severe than lower eyelid involvement, and 40% of patients experienced complete regrowth of lashes, with a mean time to regrowth of 28 months (range, 6 to 60 months). Of these, 13% experienced partial regrowth. Younger age at presentation was associated with regrowth, whereas presence or absence of other involved sites, personal or family histories of atopy, family history of alopecia, other autoimmune diseases, or the use of topical steroids did not appear to affect prognosis.ConclusionsEyelash alopecia areata is a unique entity, although it remains potentially underdiagnosed. The key differential diagnosis is trichotillomania, which is commonly associated with obsessive-compulsive disorders. The presence of exclamation-mark hairs in alopecia areata plays an important role in differentiating the 2 diagnoses.     

Primary orbital melanoma: a case series and literature review

Primary orbital melanoma (POM) is a very rare condition. We report further four cases and review all previously reported cases. We present a multicentre retrospective review of patients with POM. Clinical, radiological, surgical, histological, and follow-up data is presented. Four patients with POM were identified between 2000 and 2013. All presented with proptosis and diplopia without reduced vision. Two had known pre-existing blue cell naevi. All were stage T1N0M0. All underwent exenteration with adjuvant radiotherapy. All are disease free at follow-up durations of 24–151 months. The present three cases and review of all cases in the literature suggest a higher likelihood of disease-free survival from primary exenteration (7/8 disease-free survival, 1/8 death from metastatic disease) than wide local excision (7/16 disease-free survival, 9 recurrence or metastasis of whom 4 died). Adjuvant radiotherapy may additionally improve outcomes.     

Lacrimal sac lymphoma: a case series and literature review

AIM: To study the clinical and pathological characteristics of the lacrimal sac lymphoma, which is rare but it is the major type of non-epithelial malignant tumor in the lacrimal sac region. METHODS: Sixty-four cases of malignant lacrimal sac tumors in our hospital from 1986 to 2020 were retrospectively reviewed. Eight cases of lacrimal sac lymphoma were carefully reviewed. RESULTS: There were five mucosal-associated lymphoid tissue (MALT) lymphomas, one diffused large B-cell lymphoma, one NK/T cell lymphoma, and one mantle cell lymphoma. All eight patients represented symptoms of epiphora with swelling in the lacrimal sac for a certain period of time and showed no signs of systemic involvement at the first time of clinical visits. They had received either chemotherapy or radiotherapy after surgery. Long-term follow-up (from 11 to 220mo) showed that, except one patient with MALT lymphoma died for unknown reasons at 104mo after surgery, the other 7 patients were all alive with no signs of local recurrence, neither in other organs. CONCLUSION: Non-epithelial malignant tumors of the lacrimal sac are rare and lymphoma is the major subtype.     

Intraorbital wooden foreign bodies: case series and literature review

Intraorbital wooden foreign bodies (IOWFBs) constitute a relatively rare ocular trauma, which occupy a special type of intraorbital foreign bodies (IOFBs). Data regarding IOWFBs must be obtained from case reports or small case series due to their rarity. Here, we reported 5 cases of IOWFBs and reviewed the related literatures, which could provide comprehensive information regarding the clinical manifestations, diagnosis, and surgical treatment of IOWFBs. Combined with the published literature, a total of 51 independent cases were counted after we added 5 cases. Among them, the number of male and female patients was 35 and 16 respectively; the mean age was 27.3±18.2 (range 1-66)y. Obviously, the disorder seemed to occur mainly in young and middle-aged people. Because of the diversity in the clinical manifestations and imaging characteristics of IOWFBs, misdiagnosis and missed diagnosis often occur during the initial visit. Delayed diagnosis may lead to a high risk of orbital infection caused by IOWFBs. Surgery is the treatment of choice for most patients; however, the missed diagnosis and residue of foreign bodies after previous surgery cannot be ignored. Therefore, an accurate diagnosis is governed by the detailed trauma history, careful ocular examination, close observation of clinical manifestations, correct imaging diagnosis [e.g., magnetic resonance imaging (MRI) or computerized tomography (CT)], and timely and completely elimination of IOWFBs.     

Simple dacryops: a case series and review of the literature

In this prospective, small case series, 3 patients with simple dacryops were analyzed along with a review of literature. All 3 patients in this series had classical clinical features of simple dacryops. All of them underwent complete cyst removal with the aid of a cryoprobe. Histopathology was compatible with dacryops. In 2 of the 3 patients, the cyst revealed calcification, which to the best of our knowledge has not been reported. None of the patients developed a recurrence. In conclusion, simple dacryops is an easily recognized clinical entity. This entity has been infrequently reviewed in the older literature, and in the last decade only 6 cases have been reported. Most of the patients underwent complete excision of the cyst with no recurrences.     

Epibulbar schwannoma in a 12-year-old boy: A case report and review of literature

Schwannomas are benign, encapsulated, primary neurilemmal tumors composed of proliferating Schwann cells. Schwannomas are commonly seen in the orbit, but are rare on the epibulbar surface. Herein, we report a case of a 12-year-old boy who presented to us with a slow-growing painless subconjunctival mass in the left eye. There was no intraocular extension of the mass and intra-operatively, the mass could be clearly delineated and was excised off the underlying sclera. Histopathological examination of the mass showed typical features of schwannoma and immunohistochemistry helped to confirm the diagnosis. There was no recurrence of the lesion observed at follow-up 26 months after surgery. Here, we describe this uncommon tumor and review the available literature. Although rare, an epibulbar schwannoma should be considered in the differential diagnosis of an amelanotic, painless subconjunctival nodular mass. Excision of the lesion is the recommended treatment.     

Pyomyositis of extraocular muscle: case series and review of the literature

Pyomyositis is a primary acute bacterial infection usually caused by Staphylococcus aureus. Any skeletal muscle can be involved, but the thigh and trunk muscles are commonly affected. Only three cases of extraocular muscle (EOM) pyomyositis have been reported. We herein present four cases of isolated EOM pyomyositis. Three of our cases presented with acute onset of proptosis, pain, swelling and redness. One patient presented with mass in the inferior orbit for 4 months. One patient had central retinal artery occlusion on presentation. None of them had marked systemic symptoms. Computed tomography scan of all patients showed a typical hypodense rim enhancing lesion of the muscle involved. Three patients were started on intravenous antibiotics immediately on diagnosis and the pus was drained externally. Two patients underwent exploratory orbitotomy. In conclusion, it should be considered in any patient presenting with acute onset of orbital inflammation. Management consists of incision and drainage coupled with antibiotic therapy.     

Cystic schwannoma of the orbit-a case series

A schwannoma is an uncommon benign orbital tumor that arises from Schwann cells in the peripheral nervous system. Schwannoma with cystic degeneration is an even more rarely reported entity. Clinical examination alone is inadequate for the diagnosis. Radiological examination, like computed tomography (CT) scans, can help in the diagnosis; however, the diagnosis can only be confirmed by histopathological examination (HPE) after excision biopsy. Here, the authors report four cases of orbital schwannoma with cystic degeneration that presented with proptosis and decreased vision. CT scans showed a well-defined non-enhancing intraconal mass with cystic spaces. The histopathological examination was diagnostic for orbital schwannoma with cystic degeneration. Schwannoma should be included in the differential diagnosis of cystic orbital lesions.     

Intravitreal bevacizumab for retinal capillary hemangioblastoma: A case series and literature review

ObjectiveTo evaluate the long-term outcomes of intravitreal bevacizumab for peripheral and juxtapapillary retinal capillary hemangioblastoma (RCH).DesignWe conducted a retrospective noncomparative interventional case series.ParticipantsThere were 4 patients (5 eyes) presenting with RCH.MethodsFive eyes with RCH presented with exudative changes and visual loss. Three eyes of 2 patients with peripheral RCH were treated with cryotherapy and 2 intravitreal injections of bevacizumab (0.5 mg). Two eyes with juxtapapillary RCH were treated with 3 intravitreal injections of bevacizumab. The main outcome measures were changes in best-corrected visual acuity (BCVA), lesion size, exudation, and retinal thickness.ResultsIn peripheral RCH, improvement of BCVA from counting fingers to 20/400 was obtained in 1 eye. One patient with bilateral RCH maintained a vision of 20/20 in 1 eye with complete anatomic regression of the 3 small peripheral RCH lesions. The fellow eye with fibrotic bands from the RCH to the optic nerve head developed a tractional retinal detachment after the first injection and was treated with pars plana vitrectomy. In patients with juxtapapillary RCH, bevacizumab injections resulted in an improvement of BCVA from 20/80 to 20/20 in 1 eye, whereas the second eye did not show an improvement of BCVA despite a regression of the tumour.ConclusionsIntravitreal anti–vascular endothelial growth factor agents, alone or in combination with other treatment modalities, may improve visual acuity. Further trials evaluating the dose, the number of injections, and the route of administration will be important in advancing antiangiogenic therapies for RCH.     

Impact of superior ophthalmic vein thrombosis: a case series and literature review

Purpose: To present nine new cases of superior ophthalmic vein thrombosis (SOVT) and compare these with the literature, and to assess the impact of SOVT for the clinician.

Methods: Using the data bases of the Department of Ophthalmology of the AMC, we searched for patients with radiologically evidenced SOVT between January 2006 and December 2014. In addition, a PubMed search, using the mesh term ‘superior ophthalmic vein thrombosis’, was done.

Results: We found nine patients with SOVT. In three patients, SOVT was related to dural arteriovenous fistulae. In one patient, it was caused by the acute reversal of warfarin by vitamin K. In two patients, an infectious cause was found. In three patients, the cause of SOVT was not found despite screening for coagulation and other disorders. All patients presented with eyelid swelling, proptosis, and/or motility impairment. We found complete recovery in four patients. Three patients had mild sequelae and two patients had severe visual impairment. In the literature, we found 60 cases reporting on SOVT with various aetiologies. Clinical presentation, treatment modalities, and outcomes were comparable to our findings.

Conclusion: Our case series and literature review show that SOVT can occur simultaneously with cavernous sinus thrombosis (CST) but can also be a separate entity. Clinical presentation can mimic orbital cellulitis (OC) or CST and when no signs of OC can be found, an alternative cause for SOVT should be sought. When timely and adequate treatment is conducted, the prognosis is predominantly favourable.     

Bloody tears of unknown cause: case series and review of the literature

PURPOSE: To report 4 cases of recurrent unilateral bloody tears. METHODS: Retrospective case series. RESULTS: One boy and 3 girls, ranging in age from 6 to 14 years, reported spontaneous bloody tearing. Workup included probing and irrigation of the nasolacrimal system, blood and coagulation profiles, blood typing, serum hormone levels, conjunctival biopsy, and imaging. All findings were normal and failed to suggest a cause in any of the cases. In all patients, bloody tearing eventually resolved without further sequela. No recurrence has been reported over a follow-up period of 9 months to 11 years. CONCLUSIONS: Bloody tearing is an unusual clinical entity that concerns patients and can perplex physicians. A thorough examination and proper workup are necessary to rule out serious conditions but may fail to determine a cause. These idiopathic cases typically resolve without treatment.     

Oculomotor paresis with cyclic spasms. A critical review of the literature and a new case

Oculomotor paresis with cyclic spasms is a rare syndrome, usually noticeable at birth or developing during the first year of life. On a background of oculomotor paresis, spastic movements occur at regular intervals: about every second minute the lid is raised, the globe wanders toward midline, the pupil contracts, and accommodation increases. These spasms last from 10 to 30 seconds, and then give way again to paresis. Since this phenomenon was first described, opinions were divided about its mechanism and about the site of the responsible defect. Based on a quantitative analysis of a new case, and of all previously published reports, a new theory is proposed which can account for all features of the syndrome.