Comparison of the transarterial, transvenous, and superior ophthalmic vein approaches in the treatment of indirect carotid-cavernous fistulas.

OBJECTIVE: Indirect carotid-cavernous fistulas (CCFs) are abnormal arteriovenous shunting lesions with a highly variable clinical presentation that depends on the drainage pattern. Based on venous drainage, treatment can be either transarterial (TA) or transvenous (TV). The aim of this study was to compare the outcomes of indirect CCF embolization via the TA, TV, and direct superior ophthalmic vein (SOV) approaches. METHODS: The authors conducted a retrospective analysis of 74 patients admitted to their institution from 2010 to 2023 with the diagnosis of 77 indirect CCFs as confirmed on digital subtraction angiography. RESULTS: A total of 74 patients with 77 indirect CCFs were included in this study. Embolization was performed via the TA approach in 4 cases, the TV approach in 50 cases, and the SOV in 23 cases. At the end of the procedure, complete occlusion was achieved in 76 (98.7%) cases. The rate of complete occlusion at the end of the procedure and at last radiological follow-up was significantly higher in the SOV and TV cohorts than in the TA cohort. The rate of recurrence was highest in the TA cohort (25% for TA vs 5.3% for TV vs 0% for SOV, p = 0.68). CONCLUSIONS: The rate of immediate complete occlusion was higher in the TV and SOV cohorts than in the TA cohort while the rate of complete occlusion at final follow-up was highest in the SOV cohort. The SOV approach was significantly associated with higher rates of postoperative complications. Indirect CCFs require careful examination of the fistulous point and the venous drainage to provide the most effective patient-tailored approach.

Double-hit and triple-hit high-grade B-cell lymphoma of the ocular adnexa.

High-grade B-cell lymphoma (HGBL) with c-MYC and BCL2 and/or BCL6 rearrangements, also known as a double-hit and triple-hit lymphoma, is an aggressive non-Hodgkin lymphoma affecting older adults. After formal recognition of this entity in the 2017 revision of the World Health Organization Classification of lymphoid neoplasms, only two well-documented cases of triple-hit lymphoma of the orbit appear in the literature. Herein, we describe a 70-year-old man with progressive diplopia, ophthalmoplegia, and rapidly enlarging temporal mass. Biopsy revealed a tumor morphologically consistent with HGBL, coexpressing CD20, CD10, BCL6, BCL2, and c-MYC on immunohistochemical analysis. Fluorescence in-situ hybridization showed rearrangements in c-MYC and BCL-2 genes, confirming double-hit HGBL. Systemic workup revealed Ann Arbor stage IV disease. This report reviews the existing literature on ocular adnexal double-hit and triple-hit lymphoma and provides an update on the diagnostic ancillary studies, prognostic implications, and latest management for this aggressive hematolymphoid malignancy.

Suspected orbital myositis associated with COVID-19.

A 44-year-old male patient developed proptosis, edema, and erythema progressing to complete ptosis and supraduction deficit 2 days after positive COVID-19 test. He failed to improve on systemic antibiotics. MRI showed thickening and T2 enhancement of the superior rectus/levator complex consistent with orbital myositis. He improved on intravenous corticosteroids and experienced continued gradual improvement on oral steroids.

Ocular adnexal manifestations of neuroendocrine neoplasms: a case report and a major review.

PURPOSE: To describe a patient with orbital neuroendocrine neoplasm (NEN)/carcinoid tumor and to review the clinical presentation, systemic work-up, histopathologic features, and outcome of all previously reported ocular adnexal (OA) NENs. METHODS: A systematic literature review. PubMed/MEDLINE and Google Scholar databases were searched for all well-documented cases of OA NENs. RESULTS: Final analysis yielded 94 patients with OA NENs, 50 females (53%) and 44 (47%) males with an average age of 63 years (range 14-86). Of 91 patients with known information, the most common presenting signs were proptosis (56/91, 61%) and visual disturbances (42/91, 47%), induced by a mass most commonly associated with an extraocular muscle (49/63, 78%). The majority of tumors (88/94, 94%) were metastases, most commonly from the gastrointestinal tract (52/88, 59%). OA NEN metastasis presented following detection of primary tumor in 73/94 (78%) patients (median time to metastasis 36 months, range 0-288 months) and as an initial manifestation of disease in 15/94 (16%) patients (median time to primary detection 18 months, range 1-108 months). Systemic work-up included extra-OA NEN biopsy (37/54, 69%), multimodal imaging (42/54, 78%), and other laboratory studies (32/54, 59%). Resection with or without adjuvant chemotherapy, radiotherapy, and biologics was the most common intervention for OA NENs (36/82, 44%). Of 67 patients with available follow-up, the median survival was 108 months (95% CI 55-161 months) and the absolute 5-year survival rate was 68%. CONCLUSIONS: OA NENs are almost exclusively metastases and can precede detection of primary tumor by many months, requiring appropriate diagnostic work-up.

Perioperative Prophylactic Antibiotics in 1,250 Orbital Surgeries.

PURPOSE: Intravenous antibiotic prophylaxis is used for many clean-contaminated surgeries or clean surgeries with an implant, but its value for clean orbital surgery has not been determined. This study investigated infection risks and adverse effects related to antibiotics in patients undergoing orbital surgery. METHODS: A prospective, nonrandomized comparative case series of all patients undergoing orbital surgery with participating surgeons between October 1, 2013, and March 1, 2015. Types of surgery, antibiotic regimens, corticosteroid use, antibiotic side effects, and surgical site infections (SSIs) were entered into an electronic database and subsequently analyzed. Cases in which patients received postoperative oral antibiotics were analyzed separately. RESULTS: Of 1,250 consecutive orbital surgeries, 1,225 met inclusion criteria. A total of 1208 patients were included in the primary analysis: 603 received no antibiotic prophylaxis (group A), and 605 received a single dose of intravenous antibiotic (group B). Five patients (0.42%) developed an SSI, 3 in group A and 2 in group B. The difference in SSI rates was not statistically significant between the 2 groups (p = 0.66). Antibiotic prophylaxis, alloplastic implants, paranasal sinus entry, and corticosteroid use were not associated with differences in SSI rates. All SSIs resolved on a single course of oral antibiotics; an implant was removed in 1 case. There were no complications associated with a single dose of intravenous prophylaxis. However, 12% of 17 patients (group C) who received 1 week of oral postoperative prophylactic antibiotics developed antibiotic-related complications (diarrhea, renal injury), yielding a number needed to harm of 8.5. CONCLUSIONS: In this large series, antibiotic prophylaxis does not appear to have reduced the already low incidence of SSI following orbital surgery. Given the detriments of systemic antibiotics, the rarity of infections related to orbital surgery, and the efficacy of treating such infections should they occur, patients undergoing orbital surgery should be educated to the early symptoms of postoperative infection and followed closely, but do not routinely require perioperative antibiotics.

A Comparison of Endonasal Dacryocystorhinostomy and External Dacryocystorhinostomy: A Report by the American Academy of Ophthalmology.

PURPOSE: To assess the efficacy of endonasal dacryocystorhinostomy (DCR) compared with external DCR. METHODS: A literature search was conducted in the PubMed database in March 2016 and updated in October 2017 and February 2019. The search strategy was designed to update the first Ophthalmic Technology Assessment on endonasal DCR from 2001 by identifying new peer-reviewed human studies reported since 2000 in the English language that compare results of endonasal DCR with those of external DCR. The searches yielded 169 articles. Of these, 13 met the inclusion criteria and were assigned a level of evidence rating. RESULTS: Six of the 13 studies included in this assessment were rated level II and 7 were rated level III. Three of the 13 studies drew conclusions based on statistically significant results, but all of these were level III evidence. Two of these significant studies demonstrated lesser efficacy of endonasal laser DCR (63%-64%) compared with external DCR (94%; P = 0.0002, 0.024). The third study reported that nonlaser endonasal DCR was superior to external DCR (84% vs. 70%; P = 0.03). The remainder of the studies did not find statistically significant differences in success rates between the 2 techniques. CONCLUSIONS: Limited data suggest that laser endonasal DCR may be less effective than external DCR. Existing data are inadequate to draw conclusions about whether endonasal DCR is superior to, equivalent to, or inferior to the gold standard external DCR.

INI1/SMARCB1-Deficient Carcinoma (Rhabdoid Tumor) of the Lacrimal Gland.

Integrase interactor 1 (INI1) is a tumor suppressor gene that is ubiquitously expressed in all nucleated cells. The loss of INI1 protein activity was first demonstrated in aggressive pediatric tumors, including atypical teratoid/rhabdoid (AT/RT) tumor of the central nervous system and malignant rhabdoid tumor of the kidney. Subsequently, INI1 deficiency was discovered in other pediatric and some adult neoplasms. The spectrum of INI1-negative tumors includes a wide variety of neoplasms that occur over a wide age range, are variably aggressive, and have a variable rhabdoid component on histopathologic evaluation. In this report, the authors describe a 27-year-old gravid woman with INI1-deficient carcinoma of the lacrimal gland, previously not described in this location.

Treatments for Ocular Adnexal Lymphoma: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the literature to determine the efficacy of available treatments for ocular adnexal lymphoma (OAL) and to evaluate the outcomes and complications of treatments in patients older than 13 years. METHODS: A literature search was conducted last in March 2017 in the PubMed and Cochrane Library databases for English-language original research investigations that evaluated treatment outcomes for OAL. The searches identified 307 unique citations, and 27 studies were selected according to the criteria outlined for this assessment. RESULTS: The 27 studies reviewed comprised 2009 patients. Seventy-five percent of the cases reported were extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT). Twenty-five studies reported results using radiotherapy with or without chemotherapy or surgery. The local control rate of MALT lymphomas with treatments involving radiotherapy averaged 95.9%. Distant and local relapses can occur, and in studies reporting only on MALT lymphomas (884 patients), the 5-year and 10-year disease-free survival rates were reported to be 86.4% and 78.7%, respectively. However, overall survival in patients receiving radiotherapy remained very good, with the 5-year and 10-year survival rates reported to be 93.8% and 84.9%, respectively. Studies that included data on multiple histologic subtypes of lymphoma or non-MALT lymphomas (988 patients) reported local control rates to be 93.1%; 5-year and 10-year disease-free survival rates to be 75.7% and 71.0%, respectively; and 5-year and 10-year overall survival rates to be 78.9% and 73.5%, respectively. Studies on the use of doxycycline for MALT lymphomas (137 patients) reported complete responses of between 4.4% and 13%. Complete and partial responses combined were between 26.7% and 65%. Disease-free survival was not reported for these 2 studies, although progression-free survival was reported to be between 55% and 60.9%. The most frequently reported complications of treatment were cataracts (12.1%) and dry eye (8.5%). CONCLUSIONS: For MALT lymphomas, local control, disease-free survival, and overall survival are good with radiation treatment. The results of treatment of non-MALT lymphomas using radiotherapy also were good, but they were not as favorable as the treatment results of MALT lymphomas.

Chemodenervation for the Treatment of Facial Dystonia: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the medical literature on the outcomes and complications of various Food and Drug Administration-approved botulinum toxins for benign essential blepharospasm (BEB) and hemifacial spasm (HFS). METHODS: Literature searches were last conducted in February 2017 in PubMed for articles published in English and in the Cochrane Library database without language limitations; studies published before 2000 were excluded. The combined searches yielded 127 citations. Of these, 13 articles were deemed appropriate for inclusion in this assessment, and the panel methodologist assigned ratings to them according to the level of evidence. RESULTS: A combined total of 1523 patients (1143 with BEB and 380 with HFS) were included in the 13 studies. Five studies provided level I evidence, 2 studies provided level II evidence, and 6 studies provided level III evidence. Pretarsal injections were more efficacious than preseptal injections (96% vs. 86%, respectively). Pretarsal injections also resulted in a higher response rate on clinical scales (P < 0.05) and a longer duration of maximum response for both HFS and BEB. Patients with HFS require lower overall doses of onabotulinumtoxinA than patients with BEB for a similar duration of effect. Adverse events were dose related, and they occurred more frequently in patients who were given more units. CONCLUSIONS: Level I evidence supports the efficacy of Botox (Allergan Corp., Irvine, CA), Meditoxin, and Xeomin (Merz Pharmaceuticals, Frankfurt am Main, Germany) for the treatment of BEB. Meditoxin and Botox have equivalent effectiveness and incidence of adverse events for BEB and HFS. Dysport (Ipsen Biopharmaceuticals, Inc, Paris, France) seems to have efficacy similar to Botox and Meditoxin for BEB and HFS, but any definitive conclusions from the 2 level II studies in this review are limited by differences in the methodologies used. Higher doses of Botox and Dysport result in more adverse events. Repeated treatments using Botox seem to maintain efficacy for treatment of facial dystonias over a follow-up period of at least 10 years, based on level III evidence.

Orbital Implants in Enucleation Surgery: A Report by the American Academy of Ophthalmology.

PURPOSE: To compare the motility and complication rates of porous and nonporous implants after enucleation surgery. METHODS: Literature searches of the PubMed and Cochrane Library databases were last performed in February 2017 to identify studies published between 2003 and 2017 on outcomes after enucleation surgeries in which a variety implants were used. The searches were limited to the English language with abstracts and yielded 43 articles, which the Ophthalmic Technology Assessment Committee Oculoplastics and Orbit Panel reviewed for relevancy. Twenty-five articles were considered to have met the search strategy, and the panel methodologist assigned ratings to them according to the level of evidence. RESULTS: Only 2 of the 25 articles identified met the criteria for level I evidence. Eighteen of the studies did not assess motility after enucleation surgery, and the 7 that did evaluate this metric involved porous implants. The studies that analyzed this outcome reported favorable results, but the results were not uniformly based on objective analysis. Both porous and nonporous implants were well tolerated, and complication rates were generally low for both types. CONCLUSIONS: In keeping with increasing surgeon preference for porous implants, most studies identified in this literature search involved the use of this type of implant. These implants resulted in excellent motility after enucleation surgery, although many studies did not assess this outcome. Regardless of implant type, major complications were rare, and infection was exceptionally uncommon after enucleation. Given the paucity of data on motility and the absence of direct, objective comparisons of porous and nonporous implants, definitive conclusions about the impact of implant material on motility cannot be made. Since few studies evaluated nonporous implants, direct comparisons cannot be made definitively between implant types, and future investigations are needed to enable a critical assessment.

Balloon Dacryoplasty for Congenital Nasolacrimal Duct Obstruction: A Report by the American Academy of Ophthalmology.

PURPOSE: To identify the efficacy and adverse event rates of balloon dacryoplasty in cases of congenital nasolacrimal duct obstruction in children who fail to respond to an initial nasolacrimal duct probing. METHODS: A literature search was last performed in September 2017 in the PubMed database to identify all reports of balloon dacryoplasty. All searches up to and including the last search were limited to the English language, and they yielded 104 articles that were assessed for relevancy. Thirty-six articles were selected for full review, and 8 of these were selected for inclusion in this assessment and assigned a quality of evidence rating by the panel methodologist. RESULTS: Three of the 8 studies included in this assessment were rated level II, and 5 were rated level III. Success rates varied from 75% to 100%. Only 2 complications were identified, and these were cases of self-limited postoperative emesis. The 2 studies that compared balloon dacryoplasty with lacrimal stenting reported that outcomes were comparable between the 2 techniques. CONCLUSIONS: Although level I evidence was not available, the studies that were uncovered in the literature review indicate that balloon dacryoplasty is a safe, effective procedure to address congenital nasolacrimal duct obstruction that persists after standard probings. The outcomes of this intervention are similar to those of lacrimal stenting, and the absence of an implanted stent theoretically reduces the risk of complications.

Spheno-Orbital Meningiomas: An Analysis Based on World Health Organization Classification and Ki-67 Proliferative Index.

PURPOSE: To evaluate the clinical behavior of spheno-orbital meningiomas with regard to World Health Organization (WHO) tumor grade and Ki-67, a cellular marker of proliferation. METHODS: A retrospective review over a 16-year period of the demographic, clinical, radiographic, and surgical data of all patients with spheno-orbital meningioma who underwent surgical resection. Tumor specimens were examined histologically using the current WHO 2016 classification and immunohistochemically using Ki-67/MIB-1 monoclonal antibody. RESULTS: Thirty-eight patients met all inclusion criteria: 78.9% of tumors were WHO grade I with a mean Ki-67 of 3.76, and 93% of patients were clinically stable at last follow up; 10.5% of lesions were WHO grade II (atypical) with a mean Ki-67 of 14.93, and 10.5% of lesions were WHO grade III (anaplastic) with a mean Ki-67 of 58.3. All grade II and III meningiomas exhibited an aggressive clinical course. There were statistically significant correlations between disease clinical progression and WHO tumor grade (p < 0.001), between disease clinical progression and Ki-67 (p < 0.001), and between increasing Ki-67 index and higher WHO grade (p < 0.001). For WHO grade I lesions, a Ki-67 of ≥3.3 correlated with recurrence (p = 0.0256). Overall, disease-specific mortality occurred in 5 (13%) patients. CONCLUSIONS: Ki-67 index is a valuable marker to use in conjunction with WHO grade to predict meningioma behavior, particularly in histologically borderline lesions, and possibly to identify a subset of WHO grade I tumors at risk of recurrence. This combination of methods can aid in tailoring treatment and surveillance strategies.

Laboratory Investigations for Diagnosis of Autoimmune and Inflammatory Periocular Disease: Part II.

PURPOSE: To review the important laboratory serological investigations routinely performed for the diagnosis of autoimmune and inflammatory diseases of the orbit and ocular adnexa. METHODS: Review of the literature as well as personal clinical experience of the authors. Due to the extensive nature of the topic, the review has been split into 2 parts. Part I covers thyroid eye disease, IgG4-related disease, and myasthenia gravis. In part II of the review, sarcoidosis, vasculitis, Sjögren syndrome, and giant cell arteritis will be discussed. RESULTS: Several relatively specific serologic tests are available for the diagnosis of a variety of inflammatory and serologic diseases of the orbit. CONCLUSION: In cases of limited orbital or sino-orbital disease, serologic testing may be negative in a significant number of patients. Specifically, the clinician should be wary of ruling out limited orbital sarcoid or sinoorbital granulomatosis with polyangiitis based on serologic testing alone. Part I of this review has covered serologic testing for thyroid eye disease, IgG4-related disease, and myasthenia gravis. In part II, the authors discuss serologic testing for sarcoidosis, vasculitis, Sjögren syndrome (SS), and giant cell arteritis (GCA).

Orbital Compartment Syndrome Following Post-Traumatic Subgaleal Hematoma.

Orbital extension of subgaleal hematoma is rare. This report describes the case of an otherwise healthy 10-year-old girl who developed delayed contralateral proptosis and external ophthalmoplegia after relatively minor right-sided forehead trauma. She was found to have bilateral subgaleal hematomas communicating with a left superior subperiostial orbital hematoma. Over the course of 2 days, she developed an orbital compartment syndrome requiring emergent canthotomy and cantholysis, followed by surgical incision and drainage of her scalp hematoma without orbitotomy. Hematologic work-up revealed heterozygous factor VII deficiency.

Efficacy of Orbital Color Doppler Imaging and Neuroimaging in the Diagnosis of Carotid Cavernous Fistulas.

PURPOSE: To evaluate the diagnostic sensitivity and specificity of orbital color Doppler imaging (CDI) and conventional neuroimaging (CT/MRI) compared with cerebral angiography in patients with carotid-cavernous fistulas (CCFs). METHODS: The study design was a retrospective patient chart and imaging review. The authors reviewed 655 charts of all patients who underwent CDI and neuroimaging (CT/MRI) between 2006 and 2015 at one institution. Sixty patients had a presumptive diagnosis of CCF without thrombosis. Thirty-seven patients with 43 events met the inclusion criteria of the study. The diagnostic sensitivity of the 3 noninvasive imaging modalities (CDI, CT, MRI) for CCF was compared with the gold standard 6-vessel cerebral angiography. Significance testing was performed using the 2-tailed Fisher test. RESULTS: Color Doppler imaging had high sensitivity (96.8%) but low specificity (41.7%) for the diagnosis of CCFs with anterior orbital findings. A negative CDI had more diagnostic value than a positive CDI. While an arterial wave form in the superior ophthalmic vein was the most common finding of CCF on CDI, enlargement of the superior ophthalmic vein was the only statistically significant finding. Posterior cortical venous drainage was noted in about 10% of the patients with indirect (low-flow) fistulas, who presented with unilateral orbital signs and symptoms, a finding not previously reported in the literature. CONCLUSION: Color Doppler imaging is a useful noninvasive, radiation-free modality for diagnosis of CCF with anterior drainage, with higher sensitivity than CT or MRI, but equivalent specificity. A significant limitation of CDI is the lack of usefulness in diagnosing fistulas with posterior cortical venous drainage, which carry a risk of intracerebral hemorrhage and stroke. In this series, 10% of unilateral CCFs with anterior orbital signs and symptoms showed angiographic evidence of posterior cortical venous drainage.

Oral Antibiotics for Meibomian Gland-Related Ocular Surface Disease: A Report by the American Academy of Ophthalmology.

OBJECTIVE: To review the existing medical literature on the role of oral antibiotics in the management of ocular surface disease (OSD) that arises from disorders of the meibomian glands and to assess the efficacy of oral antibiotics in the management of this common ocular disease. METHODS: A literature search was last conducted on August 12, 2015, in the PubMed and Cochrane databases for English-language original research investigations that evaluated the role of doxycycline, minocycline, and azithromycin in OSD among adult patients. The searches identified 87 articles, and 8 studies ultimately met the criteria outlined for this assessment. RESULTS: The 8 studies identified in the search documented an improvement in meibomian gland-related OSD after treatment with these agents, although side effects were common. This search identified only 1 randomized, controlled trial to assess the efficacy of these medications. CONCLUSIONS: Although oral antibiotics are used commonly in the management of OSD, there is no level I evidence to support their use. There are only a few studies that have assessed the efficacy of oral antibiotics in clinically meaningful ways in the management of OSD that arises from disorders of the meibomian glands. The current level of evidence is insufficient to conclude that antibiotics are useful in managing OSD arising from disorders of the meibomian glands. The few existing studies on the topic indicate that oral antibiotics may be an effective treatment for OSD that results from meibomian gland disease.

Orbital sarcomas in retinoblastoma patients: recommendations for screening and treatment guidelines.

PURPOSE OF REVIEW: Retinoblastoma is the most common primary ocular malignancy in children. Although currently retinoblastoma has an excellent survival rate in developed countries, hereditary retinoblastoma survivors as well as those with a history of radiation therapy as children are at an increased risk for second primary tumors (SPTs), and specifically, for orbital sarcomas. Despite the known increased risk for SPTs in retinoblastoma survivors and the associated morbidity and mortality, no screening or treatment guidelines exist. RECENT FINDINGS: Understanding of retinoblastoma tumorigenesis and genomic expression has expanded significantly, and treatment has evolved with a shift away from radiotherapy. Until the last two decades, however, radiation was the therapy of choice for patients with bilateral disease. Because both hereditary retinoblastoma and radiation are independent risk factors for the development of SPTs such as sarcomas and these SPTs are often fatal, appropriate surveillance for retinoblastoma survivors is crucial. SUMMARY: As a result of the excellent survival rates for retinoblastoma patients, it is important to: recognize the risk of sarcoma, particularly in patients with hereditary retinoblastoma and/or prior radiation therapy; establish a screening protocol, such as the one proposed, to maximize early detection; and discuss and develop treatment guidelines for high-risk patients. Future directions of research for these patients will involve the development of molecularly targeted agents and the use of proton radiotherapy.

Odontogenic Sinusitis Resulting in Abscess Formation Within the Optic Chiasm and Tract: Case Report and Review.

A 33-year-old immunocompetent man developed rapid visual loss and a third nerve palsy secondary to acute rhinosinusitis and intracranial abscess formation. Despite endoscopic drainage of the ethmoid and sphenoid sinuses and empiric broad-spectrum antibiotics, the patient experienced progressive visual and neurological decline and ultimately required craniotomy for drainage of an optic apparatus abscess. Although odontogenic sinusitis rarely results in abscess formation of the visual pathways, early recognition and immediate treatment is imperative to decrease the risk of profound and permanent visual impairment.

Laboratory Investigations for Diagnosis of Autoimmune and Inflammatory Periocular Disease: Part I.

PURPOSE: To review the important laboratory serological investigations routinely performed for the diagnosis of autoimmune and inflammatory diseases of the orbit and ocular adnexa. METHODS: Review of the literature as well as personal clinical experience of the authors. RESULTS: Periocular inflammation is a blanket term which refers to a wide variety of conditions affecting the orbit and the ocular adnexa. Although the etiology of each of these conditions is different, their similarity lies in their clinical presentation and in the fact that each of them can be vision-threatening and even life-threatening if left untreated. CONCLUSIONS: This review covers important laboratory serological investigations specific to entities routinely encountered by orbitologists. The technique, parameters measured, and clinical importance of each of these laboratory tests are discussed in detail. Due to the extensive nature of the topic, the review has been split into 2 parts. Part I covers thyroid eye disease, IgG4-related disease, and myasthenia gravis. In part II of the review, sarcoidosis, vasculitis, Sjögren syndrome, and giant cell arteritis will be discussed.

Tension Pneumocephalus Following Orbital Exenteration.

Pneumocephalus is a known complication of skull base surgery, but is rarely seen by orbital surgeons. We report a case of postoperative mental status changes after exenteration due to tension pneumocephalus. After surgical and medical management, the patient's pneumocephalus resolved and she recovered fully. Risk factors for tension pneumocephalus, mechanism, clinical presentation, and management techniques are discussed.