Faculty Bibliography

Purpose/UNASSIGNED:and describe its clinical and histopathological features. Findings/UNASSIGNED:involvement. Histopathological analysis demonstrated fulminant polymorphonuclear infiltration of all ocular tissue layers. Despite aggressive management including two intravitreal injections and enucleation, the patient died, ultimately after receiving care at four neighboring urban medical centers. Conclusions and importance/UNASSIGNED:. A high suspicion of its contribution to panophthalmitis could be warranted early in the evaluation and management of profoundly immunocompromised patients, particularly those who have had sequential care at multiple neighboring metropolitan hospitals.

Purpose/UNASSIGNED:We report a case a pediatric patient with an eyelid lesion found to be a basaloid follicular hamartoma. Observations/UNASSIGNED:A six-year-old female with juvenile diabetes who presented with a benign eyelid lesion harboring an aberrant eyelash. Conclusions and Importance/UNASSIGNED:Basaloid follicular hamartoma is a rare benign neoplasm arising from hair follicles. These lesions can resemble basal cell carcinomas and require complete excision.

Extraocular muscle (EOM) entrapment with resulting reduction in motility and diplopia is a known complication of orbital fractures. Less commonly, transection of the EOMs due to trauma, iatrogenic injury, or intentional myotomy may lead to persistent diplopia. The inferior oblique (IO) is often encountered during orbital surgery along the medial wall and floor, and may be disinserted to aid in visualization. The authors present a case of IO entrapment which occurred during zygomaticomaxillary fracture reduction. Intraoperatively, an IO transection was performed and the muscle was reattached within the orbit. Postoperatively, the patient did not develop diplopia or motility disruption. This technique may provide a useful solution to an unusual problem during orbital fracture repair.

Indolent lymphomas have the potential to transform into more aggressive phenotypes. This phenomenon is best exemplified by the transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma into diffuse large B-cell lymphoma. However, it is less common to find multiple small cell lymphomas in the orbit, particularly mantle cell lymphoma. We present a case of an extranodal marginal zone lymphoma arising in the orbit that acquired a subclonal t(11;14) (q13;32) CCND1-IGH translocation, giving rise to a subclone of mantle cell lymphoma. The management of this tumor was targeted towards the more aggressive mantle cell subclone. The tumor exhibited an incomplete response to rituximab alone. To the best of the authors' knowledge, this represents the first such case to be described irrespective of site of origin.

Purpose: The majority of pediatric patients with optic nerve gliomas (ONG) remain asymptomatic; however, a subset of patients suffer rapid, irreversible visual loss. The purpose of this study was to determine anatomic differences of ONG noted either by imaging or by intra-operative observation between patients with gradual visual dysfunction and those with rapid visual loss.Methods: A retrospective review was performed in patients with visual loss secondary to ONG. The clinical records, pathology, and radiographic images were reviewed for all patients. The degree of folding or plication of the optic nerve (ON) was determined by analyzing the most acute angle present in the course of the ON.Outcome measures: The primary outcome measure was the angle of plication or folding of the ON and the relationship of this to the course of visual dysfunction.Results: Six patients with ONG were included in the study. A structural difference in the ON was identified in four patients with rapid vision loss compared with two patients with more gradual visual dysfunction. In patients with rapid progressive visual loss, the ONG had a 90-degree or more acute plication of the ON. Those with more gradual visual loss had more obtuse bends in the ONG.Conclusions: We have identified that the intrinsic structure of the ONG may contribute to the rare but devastatingly rapid progression of visual dysfunction in some patients. Recognizing these changes may guide clinicians to intervene prior to the development of irreversible visual loss.

Introduction: There has been a growing interest in hyperbaric oxygen therapy (HBOT) in recent years across multiple disciplines. In the field of ophthalmology, the implications of increased HBOT use may include expanded applications in treating ocular vascular pathologies as well as a greater incidence of HBOT-induced visual complications. Areas covered: The authors review recent studies on HBOT usage in the treatment of ocular conditions. In addition to providing updates on the ophthalmic indications of HBOT, adverse visual effects of HBOT are also investigated. Expert opinion: Further evidence substantiating HBOT as an effective treatment modality for ocular vascular pathologies, such as central retinal artery occlusion and diabetic retinopathy, have been published in recent years. With the identification of more prognostic factors, increased success in HBOT has been reported. However, studies also show that adverse ocular effects associated with hyperbaric oxygen exposure include myopia and cataracts. It is important to recognize the risks of iatrogenic changes in visual acuity when considering patients for HBOT.

Purpose : To describe clinical features requiring urgent surgical intervention for superior orbital blow-in fractures with and without globe compression. Methods : Retrospective, consecutive case series between 6/1/2018 and 12/1/2019. Results : Six patients (6 eyes) with superior orbital blow-in fractures were identified, 50% with bone fragments compressing the globe. The mean age of presentation was 31.8 +/- 10.5 years. The majority of patients were male (83%), involved the left eye (83%), and were assault victims with a swinging object (50%). All patients had periorbital edema and a brow laceration. No patients had clinical evidence of a ruptured globe. The visual acuity of the affected eye ranged from 20/20 to 20/30. Post-operative visual acuity worsened in one case (16%) due to vitreous hemorrhage. Surgical approaches included lateral orbitotomy through the brow laceration (66%), through an eyelid crease incision (17%) and a craniotomy (17%). The most common fracture types on computed tomography included communicated fractures of the frontal sinus to the superior orbital rim (83%), involvement of the zygomatic bone (33%) and an inferiorly displaced superior orbital rim (17%). Additional orbital injuries included left rectus hematoma (16%), bone fragment abutting the lacrimal gland (50%) and intraconal hematoma (33%). An intracranial injury was seen in 33% of patients, including a parieto-occipital hematoma and frontal white matter contusion. Fifty percent of patients had bone fragments directly compressing the globe with deformation of the globe contour, which was visible on fundoscopic examination and bedside ocular ultrasound. Distinguishing features of orbital blow-in fractures with globe compression compared to those without included supra-and abduction motility deficits, proptosis, globe dystopia and abnormal posterior segment exam, which were seen in all cases. Posterior segment findings included choroidal folds (100%), choroidal rupture (33%), commotio (33%) and vitreous hemorrhage (33%), however there were no retinal tears or detachments. Conclusions : In cases of periocular trauma, the presence of exophthalmos, globe dystopia, ocular motility deficits, and choroidal folds may suggest an orbital blow in fracture with globe compression. These findings should prompt expedited imaging and surgical intervention when appropriate

OBJECTIVE:Rituximab, a monoclonal antibody that targets CD20 receptors on mature B lymphocytes, is used in various diseases, including granulomatosis with polyangiitis (GPA) and difficult-to-treat orbital inflammatory diseases. We aimed to describe clinical and histopathologic findings in patients treated with rituximab refractory GPA who had post-rituximab orbital biopsies. DESIGN/METHODS:Retrospective review. PARTICIPANTS/METHODS:Patients with orbital GPA refractory to rituximab. METHODS:We examined the clinical outcomes, imaging results, and histopathological findings of all patients from September 1, 1994, through July 1, 2017, with a diagnosis of orbital GPA who, after treatment with rituximab, required an orbital excisional biopsy for refractory orbital disease. RESULTS:Among the 7 patients included the clinical indication for biopsy was pain (n = 3), new mass lesion despite treatment (n = 2), and persistent lesion despite treatment (n = 2). Post-rituximab biopsies showed marked collagenized fibrous tissue, with areas of concentric fibrosis resembling the framework of pre-existing lymphoid follicles, and minimal residual lymphocytic infiltrates (n = 7). Three patients had entrapped nerves. Four biopsies showed active inflammation; clinically, all patients were not responding to treatment. CONCLUSIONS:This study highlights the clinical and histopathological changes seen in patients with orbital GPA refractory to rituximab therapy. The post-rituximab biopsies show marked fibrosis in all patients. Four patients had active inflammation without evidence of entrapped nerves, whereas 3 patients had entrapped nerves and no active inflammation on their biopsies. Knowledge of the histopathology may guide future management decisions in patients with orbital GPA refractory to rituximab treatment.

Ischaemic oculomotor nerve (CN III) palsies frequently present with abrupt onset ptosis, ophthalmoplegia, diplopia, ipsilateral pain, and little to no anisocoria. An isolated microvascular ischaemic insult to the superior division of CN III is uncommon, and usually affects both the superior rectus and levator muscles. We present a rare case of an ischaemic CN III palsy isolated to the levator palpebrae superioris muscle only. Although rare, microvascular ischaemic CN III palsies should be on the differential of isolated ptosis. Other causes of isolated ptosis, such as myasthenia gravis or an orbital lesion, should be excluded.