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Optic nerve head drusen (ONHD) are typically benign hyaline and calcified concretions located within the optic nerve. Initially asymptomatic, they can increase slowly in size and number over time, and they can cause mechanical stress at the axons of the optic nerve and progressively lead to a loss of the visual field and papillary vascular changes. Here, we reported a rare case of a young patient with bilateral ONHD complicated by a nonarteritic anterior ischemic optic neuropathy (NAION) in the right eye. The aim of this case was to evaluate, using optical coherence tomography angiography (OCTA), the vascular network of the papillary region in both eyes in the presence of this ischemic event associated to ONHD. At OCTA examination, the whole papillary region revealed a general rarefaction of the vascular network, but it turned out to be greater in the right eye affected by nonarteritic anterior ischemic optic neuropathy than in fellow eye. Therefore, these findings demonstrated that ONHD may determine significant ischemic events of the optic nerve and OCTA represents a valid and noninvasive tool in the diagnosis and in the pathogenesis of these rare cases of NAION associated to ONHD in young patients.

Purpose: The aim of the present study is to analyze the changes in retinal vessel density (VD), using Optical Coherence Tomography Angiography (OCT-A), in patients that received endoscopic endonasal approach for the removal of an intra-suprasellar pituitary adenoma compressing optic chiasm., Methods: We evaluated the VD in Superficial Capillary Plexus (SCP), Deep Capillary Plexus (DCP), Radial Peripapillary Capillary (RPC) and the Foveal Avascular Zone (FAZ) area in a series of fourteen patients (7 males, 7 females, mean age 56 ± 13 years), as compared to healthy controls. We also detected the structural Spectral Domain (SD)-OCT parameters: Ganglion Cell Complex (GCC), Retinal Nerve Fiber Layer (RNFL), visual field parameters (Mean Deviation, Pattern Standard Deviation) and Best Corrected Visual Acuity (BCVA). These measurements were performed prior than surgery and 48 hours after., Results: The patients showed a significant decrease in VD of the macular and papillary regions, a significant increase in FAZ area, a significant impairment in SD-OCT, VF parameters and BCVA respect to 14 eyes of 14 healthy controls (p<0.05), at pre-op evaluation. In patients group the VD in SCP, DCP and RPC increased after surgery respect to baseline but the difference turned to be out statistically significant only in RPC (p = 0.003). Also the BCVA (p = 0.040) and the Mean Deviation at visual field (p = 0.015) significantly improved after surgery. While there was a reduction in structural OCT parameters but it was statistically significant only in GCC (p = 0.039). A positive correlation was found between the preoperative VD of the RPC, Mean Deviation, BCVA and the postoperative Mean Deviation (r = 0.426 p = 0.027; r = 0.624 p = 0.001; r = 0.515 p = 0.006)., Conclusion: OCT-A allows to detect the early changes occurring within 48 hours after surgery showing that the improvement in retinal vessel density could occur before the recovery of the structural OCT parameters and can be a positive predictive factor for the functional recovery., Competing Interests: The authors have declared that no competing interests exist.

Background: The aim of this study was to analyze patients diagnosed, staged and treated for orbital and eyelid B-cell lymphoma (OEL)., Methods: One hundred and forty-one cases of OEL were included in this study. Primary endpoints were to analyze the histopathologic findings, the main risk factors and the type of treatment and to correlate them with recurrence of OEL. The secondary endpoint was to determine the progression-free survival (PFS) time., Results: Extranodal marginal zone B-cell lymphoma was the most frequent subtype (66%), followed by small lymphocytic lymphoma (12.7%), diffuse large B-cell lymphoma (DLBCL) (9.2%), follicular lymphoma (6.6%), mantle cell lymphoma (4.3%) and Burkitt lymphoma (1.2%). The probability of relapse was influenced by the histopathologic subtype DLBCL (OR = 7.7, 95% CI 1.8-32.3) and treatment with chemotherapy (OR = 14.9, 95% CI 2.6-83.7). Multivariate analysis showed that the histopathologic subtype DLBCL and chemotherapy treatment retained statistical significance for a poorer PFS, with hazard ratios of 8.581 ( p = 0.0112) and 9.239 ( p = 0.0094), respectively., Conclusions: Five lymphoma subtypes were found in patients with OEL. The histopathologic subtype and the type of treatment were found to be the main factors influencing treatment outcome.

Background: To evaluate retinal vessel density (VD) in macular and in peripapillary regions in patients with recent onset of multiple sclerosis, at initial demyelinating event (IDE) and in matched relapsing-remitting multiple sclerosis (RRMS) patients. Methods: We evaluated VD in superficial capillary plexus, deep capillary plexus, choriocapillaris and radial peripapillary capillary plexus in IDE, RRMS patients and in matched healthy controls (HCs) through Optical Coherence Tomography Angiography (OCT-A). Clinical history, including history of optic neuritis, Expanded Disability Status scale and disease duration of patients were collected. Results: Thirty patients (20 with IDE and 10 with RRMS) and 15 HCs were enrolled. IDE patients showed a lower VD in radial peripapillary capillary plexus compared with controls (coeff. β = -3.578; p = 0.002). RRMS patients displayed a lower VD in both superficial capillary plexus and radial peripapillary capillary plexus compared with HCs (coeff. β = -4.955; p = 0.002, and coeff. β = -7.446; p < 0.001, respectively). Furthermore, RRMS patients showed a decreased VD in radial peripapillary capillary plexus compared with IDE patients (coeff. β = -3.868; p = 0.003). Conclusions: Peripapillary region vessel density reduction, revealed through OCT-A, might be considered as an early event in MS, and might be relevant as a biomarker of disease pathology., (Copyright © 2020 Cennamo, Carotenuto, Montorio, Petracca, Moccia, Melenzane, Tranfa, Lamberti, Spiezia, Servillo, De Angelis, Petruzzo, Criscuolo, Lanzillo and Brescia Morra.)

Objective: To assess the presence of retinal vascular network abnormalities in amnestic mild cognitive impairment (aMCI) patients and healthy subjects (HS) through optical coherence tomography angiography (OCTA)., Methods: OCTA and SD-OCT were performed in aMCI patients and cognitive normal HS. A complete neuropsychological evaluation was performed. Differences in vessel density (VD) in each retinal vascular plexus and in foveal avascular zone (FAZ) were evaluated with linear mixed model after correction for age, sex and disease duration., Results: Twenty-seven aMCI patients (10 Single domain aMCI, 17 Multidomain aMCI) and 29 HS were enrolled. aMCI patients showed a statistically significant reduced VD in superficial capillary plexus (SCP), deep capillary plexus (DCP) and an increased FAZ compared to controls. When aMCI patients were divided in single domain (SD) and multiple domains (MD) aMCI, SD aMCI showed no VD differences in SCP, DCP and Radial Peripapillary Capillary, while the FAZ area was significantly larger compared to controls. In MD aMCI, VD values were lower and FAZ was increased compared to controls. Comparing both aMCI groups, MD aMCI showed a significant reduction in VD values of SCP. No correlation was found between mini mental state examination (MMSE) scores and OCTA parameters., Conclusions: OCTA is able to detect changes in retinal microvascular network in early cognitive deficits and, the most sensitive alteration seems to be the enlargement of the FAZ. This non-invasive tool provides useful information on retinal involvement patterns in MCI diagnosis and follow up. Vascular network impairment seems to be related to the number of domains affected and not to MMSE., Competing Interests: R. Lanzillo received personal fees for public speaking or consultancy from Merck, Novartis, Biogen, Roche, Genzyme, Teva and Almirall. All other authors declare no conflict of interest. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

Background: This study examined whether eye-sparing surgery is associated with better or worse outcomes than exenteration for the treatment of lacrimal gland carcinomas., Methods: Forty-six patients treated for lacrimal gland carcinoma were retrospectively reviewed and compared. A statistical analysis was performed using Kaplan-Meier plots., Results: The overall survival rates for eye-sparing surgery were 52% and 37% at 5 and 10 years, and those for exenteration were 37% and 25% at 5 and 10 years, respectively (P = .73). The proportion of patients with local regional control at both 5 and 10 years after eye-sparing surgery was 0.75, and that for exenteration was 0.47 (P = .30). For eye-sparing surgery, the proportions of distant metastasis-free survival at 5 and 10 years were 0.51 and 0.39 for eye-sparing surgery and 0.29 and 0.14 for exenteration (P = .50)., Conclusion: Because the outcomes were not significantly different, the authors suggest that eye-sparing surgery can be proposed as a reasonable approach for lacrimal gland carcinomas in appropriately selected patients., (© 2020 Wiley Periodicals, Inc.)

Objective: Hepatitis C virus (HCV) has been proposed as a possible etiologic factor in ocular adnexal marginal zone lymphoma (OAML). We aimed to assess the prevalence of HCV infection in patients with OAML through a systematic review and meta-analysis., Methods: Electronic databases were searched from their inception to August 2019 for studies assessing HCV seroprevalence in patients with OAML. Pooled prevalence of HCV infection was calculated with 95 % confidence interval (CI). Statistical heterogeneity among studies was quantified via the inconsistency index (I 2 ). Funnel plot symmetry was used to assess the risk of bias across studies., Results: Nine studies with 360 patients were included. Overall pooled prevalence of HCV in OAML was 12.7 %, with low statistical heterogeneity (I 2 = 17.4 %) and with asymmetrical funnel plot. The studies clustered into two groups: 5 studies (3 from Italy and 2 multicenter with a major Italian contribution) showed a higher HCV prevalence in OAML (15.6 %), while the other 4 (from countries other than Italy) showed a lower prevalence (4.7 %); in both subgroups, statistical heterogeneity was null (I 2 = 0%) and funnel plot was symmetrical., Conclusion: HCV might be a significant etiologic factor of OAML in Italy., Competing Interests: Declaration of Competing Interest The authors declare no conflict of interest., (Copyright © 2020 Elsevier GmbH. All rights reserved.)

Introduction: The aim of this study was to evaluate retinal and choriocapillaris (CC) vessel density, using optical coherence tomography angiography (OCTA), in the macular region at baseline and after three once-monthly intravitreal injections of bevacizumab (loading phase) in patients affected by exudative age-related macular degeneration (AMD)., Methods: A total 38 eyes of 38 patients with exudative AMD (16 women, 22 men; mean age 72.47 ± 7.46 years) were included in this study. We evaluated vessel density in different macular areas (whole image, fovea and parafovea) of the superficial capillary plexus (SCP), deep capillary plexus (DCP) and CC. In addition, central macular thickness (CMT) and choroidal thickness in the foveal region and 500 µm in the nasal and temporal directions were analyzed by enhanced depth image-OCT., Results: There were no significant differences in vessel density in the SCP, DCP and CC after the loading phase compared to baseline (p > 0.05). In contrast, CMT (p = 0.039; 320.63 ± 175.05 vs. 372.47 ± 167.05 µm) and subfoveal choroidal thickness (SFCT) (p < 0.001; 189.21 ± 37.66 µm vs 170.37 ± 37.96 µm) significantly decreased after the loading phase compared to baseline. No significant correlations were found between SFCT and CC vessel density in the fovea, and between CMT and SCP and DCP (p > 0.05) at baseline and after the loading phase. Also, best corrected visual acuity did not correlate with CMT, SCP and DCP at baseline and after treatment (p > 0.05)., Conclusion: Macular and choroidal thickness did not correlate with vessel density, but probably correlated to vascular exudation. The anti-VEGF treatment, acting on vascular hyperpermeability, determines the reduction of the retinal and choroidal exudation, resulting in a decrease of their thicknesses. Consequently, these parameters could reflect the effectiveness of the anti-VEGF injections for treating exudative AMD respect to OCTA parameters., Trial Registration: ClinicalTrials.gov indentifier, NTC04138420.

Background : Multi-parametric MRI used for preoperative assessment of orbital lesions does not routinely include DCE-MRI, since its accuracy in differential diagnosis of orbital mass is still under debate. Aim of this study is to characterize orbital lesions by multi-parametric MRI, analysing the incremental predictive value of DCE-MRI in differential diagnosis of orbital lesions. Methods : In this prospective triple-blind study, 43 consecutive patients with unilateral orbital lesion underwent conventional multimodal MRI and DCE-MRI before biopsy in a tertiary referral centre. Pre-operative MRI examination including conventional unenhanced MRI protocol, DWI with ADC maps, static CE 3D-T1 w and dynamic CE T1 w sequences, was performed within 1 week from surgery (anterior/lateral orbitotomy depending on location of the lesion, to carry out incisional/excisional biopsy). Results : Comparison between conventional T1 w/T2 w, DWI, CE 3D-T1 w and DCE-MRI groups showed a statistically significant difference in scores distribution ( p < .001). Statistically significant difference was found between conventional T1 w/T2 w and DWI ( p < .005), as well as between DWI and CE 3D-T1 w ( p < .001). Conversely, no significant difference was found between CE 3D-T1 w and DCE ( p < .005). Conclusions and Relevance : This study confirmed the positive effect of DWI and CE 3D-T1 w on orbital lesions diagnosis when added to conventional T1 w/T2 w sequences, whereas no substantial impact on diagnostic performance was observed with the further addition of DCE-MRI. DCE does not strongly influence diagnostic performance and inter-rater agreement in characterizing orbital lesions; therefore, it should be recommended in selected patients whose assessment of flow dynamics is particularly useful for management. Abbreviations : US = ultrasonography; MRI = magnetic resonance imaging; CT = computed tomography; STIR = Short-TI Inversion Recovery; DWI = diffusion weighted imaging; DCE-MRI = dynamic contrast-enhanced MRI; SE = Spin-Echo; TSE = Turbo Spin-Echo; THRIVE = T1-weighted high resolution Isotropic Volume Examination (dynamic contrast-enhanced ultrafast spoiled gradient echo); ROI = regions of interest; IRR = inter-rater reliability; TIC = time-intensity curve.

Merkel cell carcinoma (MCC) is one of the rarest eyelid tumors, with high mortality rate due to lymphatic and metastatic spread. We hereby report six cases of patients with histological diagnosis of MCC referred to our Orbit Unit between 2012 and 2018, focusing on diagnosis, treatment, and subsequent follow up. All patients underwent surgical excision and systemic work-up. Both MCC TNM and eyelid MCC TNM were used to stage lesions. MCC of the eyelid is usually misdiagnosed as benign or other malignant lesions. A prompt examination and a wide local excision are mandatory. A close follow-up of these patients is advised due to high recurrence rate and lymphatic spread.

Background: Fabry disease (FD) is a X-linked recessive lysosomal storage disorder characterized by altered biodegradation of glycosphingolipids. It is a multisystem pathology, also involving ophthalmological systems that show modifications of the vessel wall due to glycosphingolipid deposits. Optical coherence tomography angiography (OCT-A) allows for an objective analysis of retinal microvasculature alterations, evaluating retinal vessel density in macular region., Methods: A total of 54 FD patients (34 females, 20 males, mean age 44.1 ± 15.6 years) and 70 controls (36 females, 34 males, mean age 42.3 ± 15.6 years) were included in this study. We evaluated vessel density in different macular areas (whole image, fovea, and parafovea) of both the superficial capillary plexus (SCP) and of the deep capillary plexus (DCP)., Results: In the SCP there was a significantly lower vascular density in patients compared with controls in whole image (49.95 ± 5.17% vs. 51.99 ± 2.52%; p < 0.001), parafovea (52.01 ± 6.69% vs. 54.30 ± 2.61%; p = 0.002), and fovea (22.38 ± 9.01% vs. 29.31 ± 5.84%; p < 0.0001). In the DCP the vessel density was statistically increased in each macular area in patients compared with controls (54.82 ± 8.07% vs. 50.93 ± 5.46%; p = 0.005, 57.76 ± 7.26% vs. 53.59 ± 5.46%; p = 0.0001, and 39.75 ± 8.59% vs. 34.43 ± 8.68%; p < 0.0001 for whole image, parafovea, and fovea, respectively)., Conclusion: OCT-A analysis showed that the macular vessel density was significantly reduced in the SCP and increased in the DCP in FD patients compared with controls. These findings, which might be a consequence of the alteration of vascular wall occurring in FD, support the hypothesis that the evaluation of early retinal microvascular network changes could be a useful tool in the clinical evaluation of the disease.

Mucous membrane pemphigoid (MMP) is a heterogeneous group of rare, systemic, autoimmune subepidermal inflammatory disease that affects mucous membranes and the eye. In its most severe forms, this disease needs systemic therapy, usually based on steroids and immunosuppressant agents. In unresponsive cases or in the presence of contraindications or severe side effects due to conventional systemic corticosteroid and/or immunosuppressant therapy, a therapy shift to high-dose intravenous immunoglobulins (IVIg) has been recommended in other reports. This new therapy has proven to be effective in stopping ocular pemphigoid, but the data regarding the long-term effect on the disease activity or reactivation are extremely scarce, so the novel scientific aim of this study was to evaluate the clinical outcomes after a 9-year follow-up in 12 eyes (6 patients) affected by MMP with ocular involvement, successfully treated with IVIg therapy, as previously described in our report published in 2008. The evaluation of ocular and extraocular disease progression was performed at the end of IVIg therapy and at the end of the follow-up period. After 9 years, all the eyes enrolled showed a long-lasting remission of ocular and oral symptoms with a significant steroid-sparing effect. In conclusion, the IVIg has to be considered as a safe and successful alternative therapy in patients with severe ocular mucous membrane pemphigoid; furthermore, this kind of therapy seems to be effective in maintaining the clinical remission by the time.

Background: Inflammatory myofibroblastic tumor (IMT) is a rare entity characterized by the presence of myofibroblasts and inflammatory cells within a fibrous stroma, which typically occurs in children or young adults. The IMT is considered generally a benign lesion, although about 20% of cases may experience recurrence, and most rarely develop metastasis. Herein, we present the largest series of primary orbital IMT ever reported., Patients and Methods: The clinical records of 25 patients, collected between the 1995 and 2015, with biopsy-proven diagnosis of orbital IMT were retrospectively reviewed to determine demographic, clinical, radiologic and pathological features, management, and outcome., Results: The study included 13 females and 12 male patients, age ranged from 5 to 76 years. Disease onset was in all cases unilateral (25/25), with posterior location (10/25) or extending anterior to posterior (7/25). The most common signs and symptoms were: proptosis (19/25), ptosis (18/25), diplopia (10/25), periocular swelling (9/25), pain (8/25), redness (7/25). All patients underwent to incisional biopsy which included total or subtotal tumor resection avoiding arming of the adjacent structure, followed by systemic steroid therapy (22/25) or radiotherapy (3/25). The disease recurred in 6 (24%) patients who responded to the subsequent therapy. No one developed metastasis or died because of the disease., Conclusion: IMT is a distinct entity which may occur in the orbit primarily. It should be considered in differential diagnosis in all orbital masses, particularly with onset of acute or subcronic inflammation. Surgical biopsy associated to a partial debulcking of the tumor, avoiding to damage adjacent vital structure may contribute to improve the outcome. Steroid therapy, seems to be the suitable as first line medical therapy, although, as reported in literature, not all cases respond to this treatment regimen. Radiotherapy, may be considered as an alternative therapy. Recurrences occurred in 24% of patients and may be treated with additional surgical resection and a new course of steroid or radiotherapy. No specific pathological features which may correlate with the prognosis have been found in this series.

Aim: To describe a series of Italian patients with orbital metastasis focusing on the outcomes in relation to the different primary site of malignancy., Methods: Retrospective chart review of 93 patients with orbital metastasis collected in a tertiary referral centre in a period of 38y and review of literature., Results: Out of 93 patients, 52 were females and 41 were males. Median age at diagnosis was 51y (range 1 to 88y). The patients have been divided into four groups on the basis of the year of diagnosis. The frequency of recorded cases had decreased significantly (P<0.05) during the last 9.5y. Primary tumor site was breast in 36 cases (39%), kidney in 10 (11%), lung in 8 (9%), skin in 6 (6%); other sites were less frequent. In 16 case (17%) the primary tumor remained unknown. The most frequent clinical findings were proptosis (73%), limited ocular motility (55%), blepharoptosis (46%) and blurred vision (43%). The diagnosis were established by history, ocular and systemic evaluation, orbital imaging studies and open biopsy or fine needle aspiration biopsy (FNAB). Treatment included surgical excision, irradiation, chemotherapy, hormone therapy, or observation. Ninety-one percent of patients died of metastasis with an overall mean survival time (OMST) after the orbital diagnosis of 13.5mo., Conclusion: Breast, kidney and lung are the most frequent primary sites of cancer leading to an orbital metastasis. When the primary site is unknown, gastrointestinal tract should be carefully investigated. In the last decade a decrease in the frequency of orbital metastasis has been observed. Surgery provides a local palliation. Prognosis remains poor with a OMST of 13.5mo ranging from the 3mo in the lung cancer to 24mo in the kidney tumor.

Coexistence of orbital cavernous hemangioma and other vascular malformations is unusual and few cases have been reported. We describe the clinical and radiological features of three cases of orbital cavernous hemangiomas associated with other vascular malformations, selected reviewing a series of 181 cases of cavernous hemangiomas. All patients were males (age ranging from 43 to 67 years) without vascular systemic disorders and/or a clinical syndrome. They experienced slow progressive exophthalmos. One of them developed acute pulsatile proptosis (case 2), while another experienced slow progressive diplopia (case 3). In one case vascular lesions were bilateral (case 3) and in two patients two different lesions coexisted in the same orbit (cases 1 and 2). All patients underwent surgical excision, which was partial in two cases. Two patients had cavernous hemangiomas in association with a venous malformation (a varix in case 1 and a lymphangioma in case 2), while in the other ones (case 3) cavernous hemangioma was associated with a low-flow arteriovenous malformation. No patient denied visual impairment postoperatively. Few cases of orbital cavernous hemangiomas coexisting with other vascular malformations have been reported in the literature. This entity seems to be an association of different variants of orbital vascular malformations, presenting with a wide spectrum of clinical forms and probably with the same pathogenesis.

Background/Aim. To evaluate the efficacy of methotrexate for the treatment of thyroid eye disease (TED). Methods. 36 consecutive patients with active TED, previously treated with corticosteroids but stopped due to the occurrence of side effects, were commenced on methotrexate therapy. Two different weekly doses were administered depending on the weight of the patient (7.5 mg or 10 mg). Clinical activity score (7-CAS), visual acuity (VA), ocular motility, exophthalmos, and eyelid position were retrospectively evaluated at 3, 6, and 12 months and compared with baseline data. Results. There was a statistically significant improvement in 7-CAS at 3, 6, and 12 months after treatment (P < 0.0001). There was no significant change in visual acuity. Ocular motility disturbances improved at 6 and 12 months (P < 0.001). There was no significant change in exophthalmos (mean 24 mm, SD 3 mm) or eyelid position (marginal reflex distance mean 6 mm, SD 1.5 mm) during the follow-up period. No side effects were registered. Conclusions. Methotrexate therapy is effective in reducing CAS and ocular motility disturbances. No significant improvement in proptosis or eyelid retraction should be expected from this treatment. Eventually, it might be considered a suitable alternative treatment in TED for patients who cannot tolerate steroids.

T(1;14) (p22;q32) involving BCL10 and IGH genes is a rare but recurrent chromosomal aberration in MALT-type lymphoma. It is rarely described in ocular adnexa B cell lymphomas, although nuclear BCL10 shuttling seems to be critical for disease progression in this district. We have evaluated the translocations MALT lymphoma-related in a series of 45 ocular adnexa cases, focusing in particular on their relation with BCL10 expression and its cellular topographic distribution. A prognostic tissue microarray (TMA) with ocular adnexa MALT lymphomas was designed. A study of BCL10 expression and its topographic distribution was performed through immunohistochemistry. In addition the assessment of t(14;18) (q32;q21), t(1;14) (p22;q32) and t(11;18) (q21;q21) was determined by Fluorescent In Situ Hybridization (FISH). Our series revealed t(14;18) (q32;q21) in 6/43 cases (14,3%). t(1;14) (p22;q32), never described in ocular adnexa MALT lymphomas, was observed in 3/31 (9,7%), two of which exhibited the gain of 3' upstream BCL10 gene signal (4%), whereas no case showed t(11;18) (q21;q21). Moreover, BCL10 expression was observed in 18/45 cases. In particular its nuclear expression was revealed in 12/45 cases, cytoplasmic expression in 5/45 and both cytoplasmic and nuclear expression in 1/45. Statistical analysis demonstrated that while BCL10 cytoplasmic expression is significantly related to the presence of the investigated chromosomal aberrations, in particular with t(14;18) (q32;q21), BCL10 nuclear shuttling does not show any correlation with these translocations. Our data support that BCL10 nuclear distribution is neither related to BCL10 rearrangement nor to other known translocations.

Dural cavernous fistulas are low-flow vascular malformations with usually benign clinical course and a high rate of spontaneous resolution. Cases with symptom progression must be treated with an endovascular approach by arterial or venous route. We report 30 patients with dural cavernous fistulas treated by coil embolization using surgical exposure and retrograde catheterization of the superior ophthalmic vein (SOV). The procedure resulted in closure of the fistula without other endovascular treatments in all 30 patients and clinical remission or improvement in 20 and eight patients, respectively. Embolization via a SOV approach is a safe and easy endovascular procedure, particularly indicated for dural cavernous fistulas with exclusive or prevalent internal carotid artery feeders and anterior venous drainage.

Purpose: To describe the case of a patient affected by orbital lymphoma mimicking pleomorphic adenoma of the lacrimal gland., Methods: This was a retrospective case report., Results: We present the case of a patient with 15-year history of slowly progressive left proptosis and inferomedial bulbar dislocation who had the presumptive diagnosis of lacrimal gland pleomorphic adenoma based on clinical and radiological features. The patient underwent lateral orbitotomy and lacrimal gland excision. Postoperative histological features were consistent with low-grade B-cell non-Hodgkin lymphoma., Conclusion: The accepted clinico-radiological criteria used for the diagnosis of lacrimal gland fossa lesions might have a certain false-positive rate, even in recent years. The initial surgical approach with the appropriate choice between fine-needle aspiration biopsies, intraoperative biopsies and lacrimal gland excisions might be a challenge.

Objective: Spheno-orbital meningiomas often present with visual deficit due to invasion of the optic canal by the tumor. This study discusses the reasons of visual impairment, the choice of the surgical approach according to the type of optic canal involvement, and the factors correlated to the visual outcome in patients harboring a spheno-orbital meningioma., Materials and Methods: A surgical series of 60 spheno-orbital meningiomas is reviewed. The preoperative visual symptoms, the involvement of the optic canal in both neuroradiological studies and surgical descriptions, the different surgical approaches are reviewed. These data are correlated with the postoperative visual outcome., Results: The 60 spheno-orbital meningiomas were classified in 4 types according to the intraorbital tumor localization: type I, supero-lateral (18 cases); type II, inferomedial (8 cases); type III, orbital apex (22 cases); type IV, diffuse (12 cases). Thirty-six of the 60 patients (60%) had variable decrease of the visual acuity on the tumor side. Forty-three patients (71.6%) had tumor extension into the optic canal on imaging studies. On the whole, 36 patients among 43 with invasion of the optic canal (83.7%) had preoperative visual dysfunction; on the other hand, none among 17 patients without tumor invasion of the optic canal had visual dysfunction. The surgical approaches according to the tumor location were as follows. A supraorbital-pterional approach was used in the 8 inferomedial tumors, in the 22 orbital apex tumors, and in 9/12 diffuse tumors; these last two types had concentric involvement of the optic canal. Three diffuse tumors with significant extension in the infratemporal fossa were operated on via a frontotemporal-orbitozygomatic approach. A wide decompression of the optic canal was performed in all cases, excepting in two inferomedial tumors without optic canal invasion. The 18 patients with lateral tumors were approached via a lateral orbitocranial approach, including removal of the sphenoid wing and lateral orbital wall without bone flap; the resection of the lateral aspect of the optic canal was performed in the 3 cases with canal invasion. Postoperative improvement of the visual function was observed in 18 of 36 cases with visual dysfunction (50%). The rate of visual improvement was significantly higher in cases with lateral involvement (3/3 or 100%) than in those with concentric involvement of the optic canal (11/27 or 40.7%)., Conclusion: The invasion of the optic canal by the tumor is the main reason of visual dysfunction in patients with spheno-orbital meningiomas. A wide opening of the optic canal must be performed routinely in patients with orbital apex and diffuse orbital tumors, where there is concentric invasion of the optic canal wall. In these cases the supraorbital-pterional approach is the technique of choice. In selected cases with lateral intraorbital tumors and invasion of the lateral aspect of the optic canal the complete tumor resection coupled with good decompression of the optic nerve may be achieved via a less invasive lateral orbitocranial approach without craniotomy., (Copyright © 2013 Elsevier B.V. All rights reserved.)

Purpose: To evaluate the frequencies of orbital space-occupying lesions seen at single orbital unit in a period of 35 years., Methods: In this retrospective case series, the authors reviewed the medical records of 2,480 consecutive patients referred to the authors' Orbital Unit for evaluation of an orbital mass between 1976 and 2011. The final diagnosis in each case was established by a combination of history, ocular findings, diagnostic imaging, and histopathologic analysis, when available. The number and percentage of benign and malignant tumors were determinated, also according to the age of the patients and the tumor location within the orbit. This study adhered to the principles of the Declaration of Helsinki., Results: Of the 2,480 lesions, 1,697 (68%) were benign and 783 (32%) were malignant. The most frequent benign tumors were dermoid cyst (14%) and cavernous hemangioma (9%). The most common malignant tumors were non-Hodgkin lymphoma (12%), basal cell carcinoma (3%), and orbital metastases (3%). In patients younger than 60 years, benign tumors are more frequent, whereas in patients older than 60 years, the frequency of malignant tumors increased. Regarding the distribution in the orbit, the most common tumors were dermoid cyst (206 cases) in the upper-outer quadrant, mucocele (155 cases) in the upper-inner quadrant, basal cell epithelioma (35 cases) in the lower-inner quadrant, cavernous hemangioma (68 cases) in the lower-outer quadrant, and meningioma (90 cases) in central space. Most of the tumors were located in the upper-outer quadrant. In the lower-inner quadrant, malignant tumors were more frequent than benign tumors., Conclusions: The authors' findings highlight the distinctive nature of the orbital oncology experience at an ocular oncology department.

Optic nerve sheath meningioma (ONSM) is a rare orbital tumor that generally induces a slow progressive visual loss in affected patients. Radiotherapy (RT) has currently become the first choice to treat ONSM. In this study our experience in ONSM treatment with fractionated stereotactic radiotherapy (FSRT) is reported. Five patients with diagnosis of orbital ONSM were treated between April 2007 and December 2009 at the Radiation Oncology department of our institution. All patients underwent history and physical, and ophthalmic examinations. Orbital MRI was performed before and 6 weeks after treatment; thereafter every 6 months for the first 2 years. By previous stereotactic localization of the target, RT was delivered with 28 daily fraction of 1.8 Gy by multiple non coplanar arcs dynamically conformed by a micro multileaf-collimator. At diagnosis, in all 5 patients, visual acuity limitations of different degrees were found, while exophthalmos was present in 2, diplopy in 2, orbital pain in 1, and proptosis in 1. In all patients pre-treatment MRI showed an orbital mass involving the optic nerve. After radiotherapy, previous symptoms improved in all patients. However, after RT the MRI consistently showed a stationary status compared to the MRI before RT. At a median follow up of 26 months (range 9-37) all patients had a subjective and/or objective better visual performance than before RT without any evidence of disease progression. No late side effects were recorded. Accordingly to the current literature, our experience confirms the efficacy and the safety of FSRT in patients with orbital ONSM.

Objective: This study defines the indications, results, and limits of lateral orbitotomy coupled with resection of the sphenoid wing for removing lateral sphenoid wing meningiomas with intraorbital extension., Methods: Eighteen patients with lateral sphenoid wing meningiomas and tumor extension into the lateral or superolateral compartments of the orbital cavity were treated by microsurgical lateral orbitotomy and resection of the sphenoid wing without craniotomy. The approach consisted of a linear skin incision along the upper eyelid crease extending to 2 cm from the canthal angle and resection of the lateral orbital rim, lateral orbital wall, and infiltrated sphenoid wing., Results: A complete resection (Simpson I), including the infiltrated bone, dura, and periorbita, was obtained in 13 patients (72%); in the other 5 cases (28%), the tumor mass and most infiltrated dura were removed, but the entity of dural resection up to the normal tissue could not be exactly defined (Simpson II). Follow-up ranged from 5 to 17 years (mean 9.7 years)., Conclusion: A select group of lateral sphenoid wing meningiomas with tumor extension in the lateral or superolateral compartments of the orbital cavity may be successfully approached and removed through a lateral orbitotomy with resection of the sphenoid wing and without craniotomy. Cases with tumor extension to the anterior clinoid process and superior orbital fissure and those with extension medial to the axis of the optic nerve require a transcranial approach.

Background: Primary orbital lymphoma is a rare disease that accounts for 10% of all orbital tumors. Radiotherapy on the orbital cavity is the treatment of choice for this unusual presentation of localized non-Hodgkin's lymphoma (NHL). The aim of this study is to retrospectively evaluate the effectiveness and the toxicity of radiation treatment in patients with primary orbital lymphoma., Methods: Forty-seven consecutive patients having primary orbital lymphoma treated in our department between May 1983 and September 2006 were investigated in a retrospective study. Either 60Co gamma rays or 6 MV X rays were used to deliver daily fractions of 1.8 or 2.0 Gy, 5 times/week, with total doses ranging from 34.2 to 50 Gy. Forty-three patients had stage IE, three had stage II and one stage IV disease. Thirty-eight patients had marginal zone B-cell lymphoma, 5 diffuse large B cell lymphoma, 3 mantle cell lymphoma and 1 Burkitt lymphoma. Local control (LC), disease free survival (DFS), overall survival (OS) and late side effects were evaluated in all patients., Results: With a median follow up of 45 months, LC was obtained in 100% of patients. The estimated 5- and 7-year DFS rates were 75.8% and 55.3%, and the 5- and 7-year OS rates were 88.7% and 79.9% respectively. Acute toxicity was minimal. Late toxicity such as cataract, keratitis, retinopathy and xerophthalmia occurred respectively in 12 (25.5%), 5 (10.6%), 1 (2.1%), and 9 (19.1%) patients., Conclusion: Radiotherapy is an effective and at the same time well tolerated treatment for primary orbital lymphoma.

Background: As for rhabdomyosarcoma (RMS) of other anatomic regions, the evaluation of traditional clinicopathological parameters does not allow the unequivocal outcome prediction of the single cases of orbital RMS. We investigated the role of DNA ploidy and immunohistochemical expression of p53, bcl-2, MDR-1 and Ki67 (MIB1) in the prognostic evaluation of orbital rhabdomyosarcomas., Materials and Methods: The study population consisted of 11 selected cases. Serial sections of each tumor, stained with Feulgen's technique, were analyzed for the DNA content, using the QUANTIMET 500c Leica analyzer, QWINVO200A software. The results were compared with the immunohistochemical expression of p53 (wild plus mutated, W&M and mutated), bcl2, MDR-1 and Ki67 (MIB1), and with follow-up data., Results: The statistical analysis of results showed that the cases of tetraploid and/or multiploid RMS, overexpressing p53 (W&M and mutated) and MDR-1, were characterized by an overall worse prognosis. On the contrary, the tumors with a favourable clinical course showed hyperexpression of MIB1 and absence of mutated p53 expression. Significantly higher MIB1 expression was found in the relapse-free group of tumors, with respect to the RMS with relapse (both in primary tumors and relative relapses, p<0.05). This finding could justify the higher sensibility to pharmacological therapy of RMS of the first group. The group of RMS with a worse prognosis (primary tumors and relapses) showed instead p53 overexpression (W&M and mutated), MDR-1 expression and multiploidy, with high 5cEE values and tetraploid peaks. No significant difference was found concerning the expression of bcl-2 among the two groups of RMS (p>0.05)., Conclusion: The evaluation of DNA ploidy, p53, MIB1 and MDR-1 expression could be used for subtyping of orbital RMS into two prognostically different subcategories, respectively RMS responder to the therapy, with favourable clinical outcome, and RMS with a worse prognosis, requiring more aggressive therapeutic protocols.

Background: Uveal malignant melanoma (MM) is characterized by a marked variability in biological behavior and by an unpredictable clinical course. Therefore the search for reliable prognostic parameters is active. In this study we investigated the immunohistochemical expression of cyclin D1, a cell cycle regulatory molecule, to analyze the possible significance of the protein in the prognostic evaluation of these neoplasms., Materials and Methods: Forty-five selected uveal MM were incubated with the anti-cyclin D-1 antibody; the findings were compared with the follow-up data, with the cell type and with the largest tumor dimension., Results: The overexpression of cyclin D1 was found to be inversely related to disease-free interval (p < 0.001) and to the spindle A/B tumors with good prognosis (p = 0.05). On the other hand, a direct correlation was found with epithelioid/spindle B melanomas with poor clinical course (p = 0.05) and with sclera infiltration (p = 0.01). No statistically significant relation emerged between cyclin D1 expression and LTD., Conclusion: These data may suggest a possible role of the overexpression of cyclin D1 in the tumorigenesis of uveal MM and in the prognostic evaluation of these tumors, representing a useful tool to subclassify lesions with similar morphological features.

BACKGROUND/AIMS. Localized orbital non-Hodgkin's lymphoma is a rare form of extranodal lymphoma. The aim of this study was to evaluate the outcome of 48 patients presenting with stage I-E non-Hodgkin's lymphoma (NHL). METHODS. Out of 118 charts of patients with lymphoproliferative lesions, those of 48 patients with stage I-E orbital lymphoma seen over a 22-year period from 1977 through 1999 were reviewed. RESULTS. Twenty-five patients were male and 23 were female. Their mean age was 58.7 (12-85) years. The mass was localized inside the orbit in 37 cases and in the lacrimal gland region in 11 cases; bilateral involvement was observed in two cases. The duration of the symptoms at the time of presentation ranged from 10 days to 10 years with a mean duration of 18 months. The 24 cases with low-grade lymphoma are all alive without disease, whatever therapeutic approach was performed (biopsy, radiotherapy, chemotherapy). In intermediate and high-grade NHL the rate of recurrence after chemotherapy has been 50% and 33%, respectively, compared to 75% and 50% after biopsy alone and 75% after radiotherapy (not administered in high-grade forms). CONCLUSION. The therapeutic approach to localized orbital non-Hodgkin's lymphoma must take the histologic grading into consideration. The 24 cases with low-grade lymphoma had a good prognosis. The 17 cases with the intermediate-grade form and the 7 cases with the high-grade form had a better prognosis when treated with chemotherapy.

Dermis-fat grafts are currently used in orbital reconstruction in a variety of procedures. The most frequent harvesting site is the gluteal area. However, we encountered some patients with anophthalmic socket who wished to avoid a visible scar on the buttock. In this article, we describe the effort to offer the patient an alternative donor site. Of the last 36 patients with anophthalmic socket who needed a dermal fat implant, 11 wished to avoid a visible scar on the buttock. To satisfy their requests we have endeavored to harvest the dermis graft from the periumbilical area. The rate of absorption, the motility, and the satisfaction of the patients were used as outcome measures and were analyzed carefully. Of 11 patients, 4 were women and 7 were men. The ages of these patients ranged from 24 to 56. The maximum follow-up was 137 months and the minimum 22 months, with a mean follow-up of 79 months. Some degree of absorption of the graft developed in one patient who had a severe absorption and required further operation. Of 11 cases, there were 7 with excellent motility, 3 with good motility, and 1 not evaluated. The motility was measured with the final prosthesis. The results for all patients were satisfactory. The periumbilical area has sufficient concentration of subdermal fat, and it is a relatively hair-free region as the lateral quadrant of the buttock. This area is a suitable alternative donor site of dermal fat implant for anophthalmic socket, especially in young women.

Purpose: To describe a case of Warthin tumor involving the lacrimal gland., Methods: Case report. The patient underwent a lateral orbitotomy to remove a nodular lesion involving the lacrimal gland. The specimen was submitted for histopathologic examination., Results: The lesion was characterized by epithelial columnar cells arranged in solid nests or lining cystic spaces containing an exudative fluid and by pronounced lymphoid infiltrate with focal follicular formation., Conclusions: To our knowledge, this is the first case of Warthin tumor involving the lacrimal gland. Management requires complete excision of the lesion.

A total of 145 patients with orbital dermoid cysts examined at the Orbital Clinic, Institute of Ophthalmology, School of Medicine, University of Naples "Federico II" over a period of 16 years were reviewed. The orbital cysts were classified as exophytic and endophytic, according to their site of attachment in relation to the orbital rims. This classification can explain the different natural history of these lesions. The exophytic cysts growing externally are discovered in childhood, whereas the endophytic ones are discovered later in life when they produce bone damage, with or without invasion of the adjacent structures.