Transpalpebral transorbital neuroendoscopic (TONES) repair of orbital meningoencephalocele: a technical note.

PURPOSE: Intraorbital encephalocele (OMEC) is a rare entity in adults, usually secondary to an orbital pathology or prior trauma, in particular orbital roof fractures. Treatment of the OMEC is warranted to alleviate the pulsating exophthalmos and prevent potential visual decline. OMEC and orbital roof fractures have been predominantly treated via a craniotomy with a reconstruction of the orbital roof using various implants. With the advances in the endoscopic techniques, neuroendoscopy found its application in the treatment of orbital pathologies. We report a minimally invasive alternative: endoscopic transorbital repair of OMEC. MATERIAL AND METHODS: The repair technique is described with illustrations and clinical images. Narrated operative video demonstrating the procedure is provided. RESULTS: Illustrative case: 50-year-old female presented with progressive right eye proptosis over 6 months. Computed tomography (CT) demonstrated bony erosion in the lateral orbital roof, and magnetic resonance imaging (MRI) showed a small hyperintense T2-weighted and T1-weighted contrast enhancing lesion in the orbit, in the area of the bony erosion. Intraoperatively, the lesion was found to be an orbital encephalocele. The orbital defect was successfully repaired by employing the 'sandwich' technique, in which a dural substitute reinforced with tissue glue were deployed without repair of the osseous orbital roof. The patient tolerated the procedure well with ultimate resolution of proptosis. The cosmetic outcome was excellent. CONCLUSION: The transorbital neuroendoscopic approach (TONES) presents a feasible, minimally invasive alternative treatment option for circumscribed intraorbital encephaloceles with minimal side effects, well tolerated by patients.

Increased rate of ptosis following glaucoma drainage device placement and other anterior segment surgery: a prospective analysis.

PURPOSE: To determine the degree and rate of ptosis in patients undergoing glaucoma and cataract surgery. METHODS: Patients undergoing cataract extraction (CE), trabeculectomy, or glaucoma drainage device (GDD) placement, or a combination, were consecutively enrolled by a sole surgeon. Eyelid measurements, including margin reflex distance 1 (MRD1) and levator function, were obtained preoperatively and at 1 and 3 months postoperatively. Primary outcome measures were the change in MRD1 pre- vs postoperatively; percentage of patients with ptosis (defined as MRD1 < 2 mm pre- vs postoperatively). Secondary measures were the absolute change in MRD1 between groups, decrease in MRD1 of ≥ 2 mm, and change in levator function. RESULTS: In total, 104 eyes of 73 patients underwent CE, trabeculectomy, or GDD placement and completed at least 1-month follow-up; 93 eyes of 65 patients completed 3-month follow-up. MRD1 decreased significantly in trabeculectomy and GDD groups at 1 and 3 months postoperatively, while it did not change in the CE group. The GDD group had a significant increase in percentage of patients with ptosis at 3 months postoperatively. CONCLUSION: Patients who underwent glaucoma surgery, especially those who underwent GDD placement, were more likely to have postoperative ptosis than patients undergoing CE alone. High ptosis rates in patients undergoing glaucoma surgery may warrant preoperative counseling and post-operative referral to oculoplastic surgeons.

The spectrum of orbital dermoid cysts and their surgical management.

PURPOSE: The purpose of this study is to describe a spectrum of surgical approaches to orbital dermoid cysts, influenced by the anatomic location of the expanded cyst wall and other factors. METHODS: In this retrospective case series, we reviewed cases of dermoid cysts surgically excised during a 39-year period (1977-2016). Cysts were categorized according to the location of the expanded cyst wall and other considerations. The impact of these factors on surgical management was determined. RESULTS: We identified six dermoid cyst growth patterns based on the anatomic location of the expanded cyst wall that influence the surgical approach: anterior to the frontozygomatic suture (FZS), superior to the FZS, medial to the FZS and other lateral wall sutures, traversing the FZS and other lateral wall sutures, nasoglabellar, and sinus tract from the orbit to the skin. Two additional factors influencing surgical methodology included satellite inflammatory pseudocysts and recurrence after surgical resection. CONCLUSION: Orbital dermoid cysts are not monolithic lesions. Functional and aesthetic outcomes can benefit from considering the anatomic pattern of cyst wall expansion and other factors in their surgical management.

IgG4-rich reactive lymphoid hyperplasia of the lacrimal gland.

We describe an otherwise healthy 24-year-old woman with reactive lymphoid hyperplasia of one lacrimal gland. She was found to have modest elevation of serum IgG4 and high density of IgG4+ plasma cells in her lacrimal gland, ranging from 40% to 60% of IgG+ plasma cells. The patient transiently responded to systemic corticosteroids before treatment with rituximab. IgG4-rich reactive lymphoid hyperplasia of the lacrimal gland is neither an established part of IgG4-related disease spectrum nor a known precursor lesion to lymphoma. Although there is a plausible risk of transforming to IgG4-related disease or to lymphoma, the biological potential and natural history of IgG4-rich reactive lymphoid hyperplasia remains to be determined.

Inflammatory Reaction to Decellularized Porcine-Derived Xenograft for Lower Eyelid Retraction.

A 54-year-old woman underwent uncomplicated bilateral lower eyelid surgery for eyelid retraction using porcine decellularized membrane graft (TarSys). Left lower eyelid pain began several months after surgery and persisted until the graft was removed 15 months after implantation. The opposite eyelid graft caused no symptoms. Histologically, the lesion consisted of acellular collagen surrounded by florid granulomatous inflammation. Although the inflammatory reaction suggested a cell-mediated immune response, this pathway of injury is difficult to reconcile given the asymptomatic opposite eyelid. The literature reflects limited experience with porcine decellularized membranes of the eyelid. Complications and long-term tolerance remain to be determined.

Herpes Zoster Ophthalmicus With Orbital Findings Preceding Skin Rash.

The authors describe 2 patients who presented with orbital findings and later developed vesicular lesions that were positive for varicella zoster virus and consistent with Herpes Zoster ophthalmicus. One case is the first to involve dacryoadenitis and orbital myositis preceding disseminated Herpes Zoster. In the other case, a patient developed zoster orbital syndrome leading to elevated intraocular pressure, loss of vision, and afferent pupillary defect. Canthotomy and cantholysis were required to restore vision. In both cases, the orbital syndrome developed prior to the vesicular rash. These cases highlight the need to consider Herpes Zoster ophthalmicus in patients with orbital syndrome not responding to conventional treatment.

Patient and physician perceptions of medicare reimbursement policy for blepharoplasty and blepharoptosis surgery.

OBJECTIVE: To describe patient preferences regarding payment for blepharoplasty and blepharoptosis repair and physician practices before and after the 2009 change in reimbursement for these 2 procedures by the Centers for Medicare and Medicaid Services (CMS). DESIGN: Cross-sectional study. PARTICIPANTS: Fifty patients presenting for functional blepharoplasty and blepharoptosis repair at an academic oculoplastic practice and 198 members of the American Society of Ophthalmic Plastic and Reconstructive Surgery. METHODS: A 5-question paper survey was administered to patients, and a 5-question web-based survey was distributed to 510 unique physician e-mail addresses obtained from the American Society of Ophthalmic Plastic and Reconstructive Surgery mailing list server in 2010. MAIN OUTCOME MEASURES: The surveys elicited patient knowledge and attitudes regarding the reimbursement policy of the CMS and physician knowledge and behaviors before and after the reimbursement policy change. RESULTS: Ninety-one percent of patients would be opposed to having to pay out of pocket for blepharoplasty or having to wait at least 3 months after ptosis repair to have a blepharoplasty. When asked to choose between these options, 62% of the patients would rather have the 2 surgeries performed separately than pay out-of-pocket. Before the reimbursement policy change by the CMS, 77% of oculoplastic surgeons performed blepharoplasty and blepharoptosis repair in the same sitting, whereas 37% did so after the policy change (P<0.001). Compared with before the policy change by the CMS, more surgeons performed the 2 procedures at least 3 months apart (4% before vs. 29% after, P<0.001) and more often billed patients for a cosmetic blepharoplasty (5% before vs. 12% after, P = 0.009). CONCLUSIONS: Our study suggests that oculoplastic surgeons have made a change in the delivery of ptosis and blepharoplasty surgical services after the reimbursement policy change for these procedures by the CMS in 2009. This change, in which patients undergo separate surgical visits for ptosis repair and blepharoplasty, is not desirable to most patients.

Bilateral Chondroid Metaplasia of Conjunctiva Associated With Monoclonal Gammopathy of Undetermined Significance.

PURPOSE: To describe a case of a patient with bilateral proliferation of chondrocytes of the lower palpebral conjunctiva. METHODS: Observational case report and review of relevant literature. RESULTS: Conjunctival biopsy from an 83-year-old man with bilateral acquired lower conjunctival nodules revealed a benign proliferation of chondrocytes. A general medical evaluation revealed serum monoclonal gammopathy with light-chain proteinuria. CONCLUSIONS: Bilateral-acquired benign chondrocyte tumefactions of the palpebral conjunctiva have not been previously reported. This initiated a search for a biologically plausible explanation. The findings in this report support that these proliferations represent chondroid metaplasia related to light-chain deposition. The diagnosis requires exclusion of other forms of chondrocyte proliferation including well-differentiated chondrosarcoma.

Subarachnoid Hemorrhage and Internal Carotid Artery Dissection and Occlusion Following Self-Enucleation.

Self-enucleation is an uncommon type of major self-injury, which may lead to severe neurological deficits and life-threatening complications, such as subarachnoid hemorrhage (SAH) and internal carotid artery (ICA) dissection and occlusion. Our patient is a 53-year-old man with a history of bipolar disorder and schizophrenia who presented with SAH, intraventricular hemorrhage, ICA dissection and occlusion, and right cerebral infarct following self-enucleation. Despite a Glasgow Coma Score of 6 on initial presentation, he improved with conservative management. He achieved a near-complete neurological recovery, with residual left lower extremity weakness and mild confusion. Self-enucleation is a major neurologic, ophthalmologic, and psychiatric emergency with a potential for serious neurological complications and contralateral visual loss. Yet, conservative management may lead to dramatic recovery.

Melanoma-Associated Spongiform Scleropathy Adjacent to a Choroidal Nevus.

Melanoma-associated spongiform scleropathy contiguous to a choroidal nevus was an incidental finding in a 57-year-old woman whose eye was removed for a separate choroidal-ciliary body melanoma. All previously reported cases of melanoma-associated spongiform scleropathy, except for one, have been found adjacent to posterior uveal melanoma. The mechanism of scleral degeneration in melanoma-associated spongiform scleropathy is unknown. Few cases of posterior uveal nevi have been reported since the description of spongiform scleropathy, making assessment of the specificity of the degeneration to melanoma alone difficult. The presence of melanoma-associated scleropathy adjacent to a choroidal nevus indicates that the condition is not exclusively linked to posterior uveal melanoma.

Pediatric Periocular Dermoid Cysts: Incidence, Clinical Characteristics, and Surgical Outcomes.

PURPOSE: To report the incidence, clinical findings, and surgical outcomes of periocular dermoid cysts diagnosed among children over a 20-year period. METHODS: All patients ≤5 years of age, who were diagnosed with a periocular dermoid cyst in Olmsted County, Minnesota from 1986 to 2005 were reviewed to determine the population incidence, clinical presentation, and management outcomes. RESULTS: A total of 54 children were diagnosed with a periocular dermoid cyst during the 20-year period, yielding a birth incidence of 1 in 638 live births. The mean age at diagnosis was 12 months (range, 1 month-60 months) and 29 (53.7%) were female. A total of 44 cysts (81.5%) occurred in the superotemporal orbital rim, 6 (11.1%) in the superonasal orbital rim, 3 (5.6%) in other periocular areas, and one (1.9%) within the orbit. A total of 34 (63%) children had an ophthalmic exam, all without amblyopia or other ocular sequelae. A total of 48 (88.9%) patients underwent surgical excision with 5 (10.4%) having documented intra-operative cyst rupture, none of whom had post-operative complications. Two (4.2%) other patients were, however, noted to have lesion recurrence following surgical intervention. CONCLUSIONS: Periocular dermoid cysts occur in approximately 1 in 650 live births and most commonly occur in the superotemporal region of children aged 1 year or less. Ocular sequelae are rare and surgical excision often yields excellent results.

Small lymphaticovenous malformation of the orbital apex clinicopathologic correlation.

PURPOSE: To familiarize clinicians with the clinical and magnetic resonance imaging (MRI) features of a small orbital apex lymphaticovenous malformation that resulted in blindness and evaded timely clinical diagnosis. OBSERVATIONS: A 68-year-old man presented with severe vision loss due to a 9 mm mass at the apex of the orbit above the optic nerve. When surgically removed 4 years later, the lesion was characterized by vascular spaces of varying size. Larger ones were filled with fibrin and organized thrombi. Stromal septa of endothelial-lined cavernous spaces were partially necrotic and there was evidence of remote hemorrhage. Some endothelial cells expressed D2-40, a marker of lymphatic channels. CONCLUSIONS AND IMPORTANCE: Unless a high index of suspicion is maintained for a lymphaticovenous malformation the clinical diagnosis of a small but vision-threatening lesion can be overlooked.

Outcomes of Patients Initially Treated with Intravitreal Bevacizumab for Central Retinal Vein Occlusion: Long-Term Follow-Up.

BACKGROUND AND OBJECTIVE: To assess outcomes of visual acuity (VA) and central retinal thickness (RT) in patients with macular edema (ME) secondary to central retinal vein occlusion (CRVO) who were initially treated with bevacizumab and followed for up to four years. STUDY DESIGN/MATERIALS AND METHODS: In this observational case series, 51 patients with non-ischemic and ischemic CRVO who had initial treatment with bevacizumab were included. Main outcome measures were VA and RT at one year, with follow-up of up to four years. RESULTS: Mean VA improved from 20/214 at baseline to 20/107 at one year (p = 0.0009) and this improvement was maintained clinically at four years. RT decreased from 595 µm to 339 µm at one year (p = 0.0027) and this was maintained at four years. CONCLUSION: Patients who received bevacizumab as initial therapy for ME from CRVO maintained VA and RT improvement for up to four years.

A case of bilateral human herpes virus 6 panuveitis with genomic viral DNA integration.

BACKGROUND: We report a rare case of bilateral panuveitis from human herpes virus 6 (HHV-6) with genomic viral DNA integration in an immunocompromised man. FINDINGS: A 59-year-old man with history of multiple myeloma presented with altered mental status, bilateral eye redness, and blurry vision. Examination revealed bilateral diffuse keratic precipitates, 4+ anterior chamber cell, hypopyon, vitritis, and intraretinal hemorrhages. Intraocular fluid testing by polymerase chain reaction (PCR) was positive for HHV-6. The patient was successfully treated with intravitreal foscarnet and intravenous ganciclovir and foscarnet. Despite clinical improvement, his serum HHV-6 levels remained high, and it was concluded that he had HHV-6 chromosomal integration. CONCLUSIONS: HHV-6 should be considered in the differential for infectious uveitis in immunocompromised hosts who may otherwise have a negative work-up. HHV-6 DNA integration may lead to difficulties in disease diagnosis and determining disease resolution.