Reply to Wostyn, P. Targeting the Cerebrospinal Fluid Compartment in Glaucoma: Still the Dark Side of the Moon? Comment on "Passaro et al. Glaucoma as a Tauopathy-Is It the Missing Piece in the Glaucoma Puzzle? J. Clin. Med. 2023, 12, 6900".

We are grateful to the author of the comment [...].

Analysis of visual impairment score in a series of 48 tuberculum sellae meningiomas operated on via the endoscopic endonasal approach.

OBJECTIVE: Tuberculum sellae meningioma (TSM) represents a complex skull base tumor. The primary goals of surgical treatment are represented by maximal safe resection and visual recovery; therefore, appropriate patient selection is critical to optimize results. In the last 2 decades, the endoscopic endonasal approach (EEA) has appeared as a successful and viable strategy for the management of these tumors. The authors identified preoperative factors associated with extent of resection and visual outcome after EEA for TSM. METHODS: In this retrospective cohort study, the authors analyzed patients who underwent extended endoscopic endonasal surgery for TSM between January 2005 and April 2022 at the Division of Neurosurgery, Università degli Studi di Napoli Federico II, Naples, Italy. Tumor size, vessel encasement, and optic canal involvement were classified according to University of California, San Francisco, score. Visual acuity and visual fields were analyzed according to the visual impairment score (VIS), defined as a four-level classification: grade 1 (VIS 0-25), grade 2 (VIS 26-50), grade 3 (VIS 51-75), and grade 4 (VIS 76-100). Ophthalmological functions were tested preoperatively and during the early postoperative period (within 6 months after surgery) and late postoperative period. RESULTS: A total of 48 patients were enrolled. Forty-one (85.4%) patients experienced blurred vision or visual field defect as a presenting sign. Gross-total resection was achieved in 40 (83.3%), near-total resection in 2 (4.2%), and subtotal resection in 6 (12.5%). Visual defect improved in 82.9% (34/41) of cases, 12.2% (5/41) had no significant changes, and 2.4% (1/41) had worsened visual defect. The mean change in VIS was 42% (95% CI 58.77-31.23). Visual outcome was poorer when preoperative VIS (VIS-pre) was greater than 25 (p = 0.02). Six postoperative CSF leaks occurred (12.5%), and 1 patient (2.1%) required revision surgery. CONCLUSIONS: The EEA is a safe and effective approach for TSM removal, with the advantage of preserving optic apparatus vascularization, and can promote gross-total resection and visual improvement. The authors have defined four categories based on VIS that relate to postoperative outcome: the lower the VIS-pre, the higher the rate of postoperative VIS improvement. This finding may be useful for predicting a patient's visual outcome at the preoperative stage.

Glaucoma as a Tauopathy-Is It the Missing Piece in the Glaucoma Puzzle?

Glaucoma is a chronic neurodegenerative disorder affecting the visual system which can result in vision loss and blindness. The pathogenetic mechanisms underlying glaucomatous optic neuropathy are ultimately enigmatic, prompting ongoing investigations into its potential shared pathogenesis with other neurodegenerative neurological disorders. Tauopathies represent a subclass of neurodegenerative diseases characterized by the abnormal deposition of tau protein within the brain and consequent microtubule destabilization. The extended spectrum of tauopathies includes conditions such as frontotemporal dementias, progressive supranuclear palsy, chronic traumatic encephalopathy, and Alzheimer's disease. Notably, recent decades have witnessed emerging documentation of tau inclusion among glaucoma patients, providing substantiation that this ocular disease may similarly manifest features of tauopathies. These studies found that: (i) aggregated tau inclusions are present in the somatodendritic compartment of RGCs in glaucoma patients; (ii) the etiology of the disease may affect tau splicing, phosphorylation, oligomerization, and subcellular localization; and (iii) short interfering RNA against tau, administered intraocularly, significantly decreased retinal tau accumulation and enhanced RGC somas and axon survival, demonstrating a crucial role for tau modifications in ocular hypertension-induced neuronal injury. Here, we examine the most recent evidence surrounding the interplay between tau protein dysregulation and glaucomatous neurodegeneration. We explore the novel perspective of glaucoma as a tau-associated disorder and open avenues for cross-disciplinary collaboration and new treatment strategies.

Long-term effects of early/late-onset visual deprivation on macular and retinal nerve fibers layer structure: A pilot study.

BACKGROUND/AIMS: Tomographic analysis of macular and peripapillary retinal nerve fibers layer (RNFL) thickness in patients with history of congenital (CC) and developmental cataract (DC). METHODS: Analysis of macular and RNFL thickness using a spectral-domain optical coherence tomography was performed. Retinal layers thickness was measured using the internal segmentation software. Measurements of affected (unilateral and bilateral), contralateral eyes and control eyes were compared. RESULTS: Patients with history of CC or DC (n = 13 and 11 respectively) and 35 healthy control subjects were enrolled. Thicker inner and outer nuclear layers (INL, ONL) and thicker ONL were found when CC and DC group when compared to controls respectively. Bilateral CC showed the most relevant differences. Slight thickening of CC inner retinal layers were found when compared to DC. Increased superonasal RNFL thickness was found in CC group when compared to DC and controls. Thickening of RNFL of contralateral unaffected eyes of unilateral CC were found when compared to controls. CONCLUSION: Significant macular and RNFL thickness changes between CC, DC patients and controls that partially involve also contralateral unaffected eyes of unilateral congenital cataract were found. CC and DC groups show significant differences only in inner retinal layers thickness. Our data suggest that early visual deprivation may influence retinal arrangements occurring during development involving predominantly the outer nuclear layer and para/perifoveal inner retinal layers, and confirm that early treatment of CC allow to achieve better long-term visual outcome. Moreover functional and structural data support the hypothesis that unilateral amblyopia is not exclusively an unilateral issue.

Management of the skull base invasion in spheno-orbital meningiomas.

BACKGROUND: The tumor invasion of the skull base structures is very frequent in spheno-orbital meningiomas. The aim of the present study is to evaluate the invasion rate of skull base structures and the best surgical approach and management. METHODS: The surgical series of 80 spheno-orbital meningiomas was reviewed. The tumors were classified according to the intraorbital location with respect to the optic nerve axes into three types: I-lateral: II-medial; III-diffuse. The invasion of the orbital apex, optic canal, superior orbital fissure, anterior clinoid, ethmoid-sphenoid sinuses, and infratemporal fossa was evaluated. The rate and extension of involvement of these structures was correlated with the intraorbital location and the surgical approach. The preoperative ophtalmological symptoms and signs and their outcome were also evaluated. RESULTS: Proptosis was found in 79 patients (97%), variable decrease of the visual function in 47 patients (59%), and deficits of the eye movements in 28(35%). The invasion of the optic canal (74%), superior orbital fissure (65%), anterior clinoid (60%), and orbital apex (59%) was more frequently found, whereas the tumor extension into the ethmoid-sphenoid sinuses (4%) and infratemporal fossa (4%) was rare. Types II and III meningiomas showed significantly higher involvement of the skull base structures than type I ones, which only had 15% invasion of the optic canal. Remission or significant improvement of the visual function occurred postoperatively in 24 among 47 cases (51%), with a higher rate for type I meningiomas vs. other types (p = 0.021, p = 0.019) and worsening in 7 (15%). CONCLUSIONS: Spheno-orbital meningiomas growing in the lateral orbital compartment show no involvement of the skull base structures excepting the optic canal as compared to those growing medially or diffusely. The surgical resection of tumor invading the skull base structures should be more extensive as possible, but the risk of optic and oculomotor deficits must be avoided.

Optical coherence tomography angiography in Bietti crystalline dystrophy.

PURPOSE: Bietti crystalline dystrophy (BCD) is a rare autosomal recessive retinal dystrophy caused by pathogenic variants of CYP4V2 gene and characterized by shiny yellow deposits in the retina and progressive atrophy of the retinal pigment epithelium (RPE) and choriocapillaris. The main aim of the present study is to describe the optical coherence tomography angiography (OCTA) characteristics of a patient affected by BCD. METHODS: A 59-years-old female with genetically confirmed BCD underwent an ophthalmological examination complete of OCTA performed in the atrophic retina, the junctional zone and the apparently normal retina. The area of choriocapillaris (CC) atrophy was compared to the area of RPE atrophy on fundus autofluorescence (FAF) imaging. RESULTS: A severe vessel density (VD) deficit at the level of superficial and deep capillary plexa as well as CC was registered in atrophic areas, which resulted deeper with respect to the junctional area, whereas the apparently preserved retina revealed VD values similar to that of control eyes. The area of RPE atrophy on FAF was larger (55.90 mm2 in right eye and 48.76 mm2 in left eye) than the area of CC atrophy on OCTA imaging (51.86 mm2 and 42.44 mm2 respectively in right and left eye). CONCLUSIONS: Our findings suggest that VD impairment of retinal plexa and CC follows the degeneration of RPE as demonstrated by the greater size of the area of RPE atrophy compared to CC atrophy. Further investigations based on OCTA imaging are necessary to enhance our knowledge of this rare disease.

OCT angiography indices and the choroidal vascularity index in wild-type transthyretin (TTR) amyloidosis (ATTRwt).

Purpose: Retinal angiopathy represents a well-known ocular manifestation of hereditary transthyretin amyloidosis (ATTRv). Until recently, there have been no reports on retinal changes in ATTRwt. In this retrospective observational clinical study, we aimed to determine whether vessel density (VD) indices and the choroidal vascularity index (CVI) could offer insights into retinal and choroidal vascular changes among patients affected by ATTRwt. Methods: Eighteen patients with a confirmed diagnosis of ATTRwt underwent structural optical coherence tomography (OCT) and OCT angiography (OCTA). We established a control group consisting of 16 healthy subjects for statistical comparisons. The 3D OCT and OCTA datasets were analyzed to assess retinal and choroidal thickness and VD. For measuring CVI, we obtained measurements for the total choroid area (TCA), luminal area (LA), and stromal area (SA). Results: The mean VD exhibited a statistically significant reduction in the superficial capillary plexus (SCP), deep capillary plexus (DCP), and choriocapillaris (CC) among the ATTRwt group in comparison to the control group (p < 0.0001). Notably, ATTRwt patients displayed decreased choroidal thickness (p = 0.08). Additionally, the median CVI was lower in the ATTRwt group than in the control group (p = 0.04). Conclusion: The indices from OCTA and CVI have the potential to serve as non-invasive biomarkers for the quantitative evaluation of retinal and choroidal vascular involvement in patients with ATTRwt.

Cytotoxicity, Mitochondrial Functionality, and Redox Status of Human Conjunctival Cells after Short and Chronic Exposure to Preservative-Free Bimatoprost 0.03% and 0.01%: An In Vitro Comparative Study.

Prostaglandin analogues (PGAs), including bimatoprost (BIM), are generally the first-line therapy for glaucoma due to their greater efficacy, safety, and convenience of use. Commercial solutions of preservative-free BIM (BIM 0.03% and 0.01%) are already available, although their topical application may result in ocular discomfort. This study aimed to evaluate the in vitro effects of preservative-free BIM 0.03% vs. 0.01% in the human conjunctival epithelial (HCE) cell line. Our results showed that long-term exposure to BIM 0.03% ensues a significant decrease in cell proliferation and viability. Furthermore, these events were associated with cell cycle arrest, apoptosis, and alterations of ΔΨm. BIM 0.01% does not exhibit cytotoxicity, and no negative influence on conjunctival cell growth and viability or mitochondrial activity has been observed. Short-time exposure also demonstrates the ability of BIM 0.03% to trigger reactive oxygen species (ROS) production and mitochondrial hyperpolarisation. An in silico drug network interaction was also performed to explore known and predicted interactions of BIM with proteins potentially involved in mitochondrial membrane potential dissipation. Our findings overall strongly reveal better cellular tolerability of BIM 0.01% vs. BIM 0.03% in HCE cells.

Optical Coherence Tomography Angiography Analysis of Vessel Density Indices in Early Post-COVID-19 Patients.

Purpose: A hypercoagulable state has been reported to cause potential sight-threatening ischemia in patients suffering from Coronavirus disease 2019 (COVID-19). This study aimed to determine whether vessel density (VD), as measured by optical coherence tomography angiography (OCT-A), has insights into retinal and choriocapillaris vascular changes in patients affected by SARS-CoV-2 infection. Methods: Hundred and fifty two patients positive for SARS-CoV-2 infection were enrolled in this observational, retrospective, controlled study. A control group of 60 healthy subjects was selected for statistical comparisons. Raw OCT and OCT-A data were exported and 3D datasets were analyzed to determine VD. Results: Hundred and forty eyes (92.1%) were included for final analysis. The VD of the superficial capillary plexus (SCP) did not differ between the two groups. The mean VD of the deep capillary plexus (DCP) and the choriocapillaris (CC) was significantly lower in the foveal sector of the COVID-19 group compared to healthy controls. Within the post-COVID-19 group, the lowest DCP and CC foveal VD values were recorded in patients treated with antiviral therapy; no differences were observed among COVID-19 patients with other comorbidities (hypertension, diabetes, thyroid disease) or taking antiplatelet therapy. DCP and CC foveal VD were significantly lower in patients hospitalized in an intensive care unit (ICU) than asymptomatic patients. Conclusion: Foveal vessel density at the level of DCP and CC was reduced in post-COVID-19 patients. Further studies evaluating these changes over time will be needed to corroborate the hypothesis of a microvascular retinal impairment in individuals who have recently recovered from SARS-CoV-2 infection.

Topographic Outcomes in Keratoconus Surgery: Epi-on versus Epi-off Iontophoresis Corneal Collagen Cross-Linking.

Background: Corneal collagen cross-linking (CXL) has become the gold standard for mild and moderate stages to stop the progression of keratoconus. We analyzed some corneal topography indices to compare iontophoresis epi-on and iontophoresis epi-off techniques throughout a two-year follow-up. Methods: A total of 64 eyes of 49 patients who underwent CXL were recruited. In 30 eyes the epi-off technique was performed, whereas the remaining 34 eyes were treated with the epi-on technique. All patients underwent a complete ophthalmologic examination that included CDVA, central and thinnest corneal thickness, Schirmer test I, TBUT test, and the Ocular Surface Disease Index. Results: In both groups, a significant improvement in visual function was recorded. No statistically significant differences between Kmax, Mean K, Flat K, Steep K values were found. Statistically significant differences (p < 0.05) between the epi-on and epi-off groups' pachymetry before and after 24 months follow-up as well as between the epi-on and epi-off groups' topographically thinnest point in the immediate post-surgery and 24 months after surgery were recorded. Conclusion: Our study highlighted that both techniques are valid in mid-term corneal stabilization. The advantage of the new iontophoresis epi-off cross-linking technique could be found in a faster imbibing time of the cornea, therefore reducing surgical times, with a lower risk of complications.

Effect of a Fixed Combination of Curcumin, Artemisia, Bromelain, and Black Pepper Oral Administration on Optical Coherence Tomography Angiography Indices in Patients with Diabetic Macular Edema.

Background: To investigate the effects of a fixed combination of Curcumin (200 mg), Artemisia (80 mg), Bromelain (80 mg), and Black pepper (2 mg) on vascular parameters in mild to moderate diabetic macular edema (DME). Design: Prospective, case-control study. Methods: Fifty-six patients affected by diabetes mellitus type II were enrolled in the study. Twenty-eight patients with DME received 2 tablets/day, before meals of a dietary complementary supplement containing in fixed combination Curcumin (200 mg), Artemisia (80 mg), Bromelain (80 mg), and Black pepper (2 mg) (Intravit®, OFFHEALTH Spa, Firenze, Italy) for 6 months. Twenty-eight age-matched subjects affected by diabetes mellitus type II were given placebo and served as control group. Patients underwent best correct visual acuity (BCVA), swept optical coherence tomography (OCT), and OCT-Angiography (OCTA). OCTA images of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) were obtained for each eye. By the end of the follow-up patients were defined responder to the therapy when a decrease of more than 30 µm was registered in central retinal thickness (CRT) measurement, while a poor responder was determined by the absence of reduction or an increase in central retinal thickness at 6 months. We assessed the foveal avascular zone (FAZ) area, vessel density and quantified the number of microaneurysms in each layer. Results: A significant improvement of BCVA and CRT reduction was recorded at 6 months follow-up in the dietary complementary supplementation group compared to control (respectively p = 0.028 and p = 0.0003). VD of the total capillary plexus, microaneurysms count, glycaemia and HbA1c did not vary over the follow-up period between groups. Within the Intravit® group, poor responders tended to show a larger FAZ area, more microaneurysms, and a lower VD in the DCP compared to the good responders group (p < 0.0001). Conclusions: A fixed combination of Curcumin, Artemisia, Bromelain, and Black pepper oral administration may have a positive impact on central retinal thickness, visual acuity, and VD of the DCP in compensated type 2 diabetic patients with mild DME.

Predicting the early visual outcomes in sellar-suprasellar lesions compressing the chiasm: the role of SD-OCT series of 20 patients operated via endoscopic endonasal approach.

BACKGROUND: Tumors involving the sellar area often cause visual disorders due to optic nerves/chiasm compression, so that surgery is required. However, the likelihood of visual improvement/restoration is variable. Optical coherence tomography (OCT) is a valid diagnostic tool for defining pathological conditions of the optic apparatus. We herein investigated the role of the SD-OCT and the best corrected visual acuity in predicting postoperative visual recovery, in patients complaining of chiasm compression due to sellar-suprasellar lesions. METHODS: We retrospectively analyzed 20 patients (7 females and 13 males; mean age 50.8±17.87 years, range 11-83), with optic chiasm/nerve compression that underwent endoscopic endonasal approach at the Division of Neurosurgery of the University of Naples Federico II, Naples, Italy, between June 2014 and February 2015. Preoperative ophthalmological assessment focused on BCVA Snellen charts, standard automated perimetry test and SD-OCT. Test were repeated at two weeks, one and three months after surgery. RESULTS: A significant thickness reduction in all the patients, as compared to the control group was noted: GCC thickness reduction remained mostly unchanged postoperatively: 42.9% of the patients had altered visual acuity (VA), while the Perimetry revealed 90% of altered preoperative MD values (MD>-2.00): perimetry improvement was noted in 93% of cases and visual acuity in 91.3% of cases. A direct correlation between preoperative retinal status and functional recovery was identified considering the values of pRNFL and GCC. CONCLUSIONS: OCT provides valuable information regarding the entity of compression also in patients without any visual field defects. Although several factors should be taken into consideration, we retain that preoperative RNFL and GCC thickness values can be claimed as a reliable predictor of visual recovery in those patients presenting chiasm compression.

Retinal and Choriocapillaris Vascular Changes in Patients Affected by Different Clinical Phenotypes of ß-Thalassemia: An Optical Coherence Tomography Angiography Study.

In this cross-sectional study we assessed the vascular alterations in retinal and choriocapillaris perfusion in patients affected by ß-thalassemia, by means of optical coherence tomography angiography (OCTA). A total of 124 eyes of 62 patients (mean age 44.74 ± 5.79 years old) affected by ß-thalassemia (transfusion dependent thalassemia (TDT), non-transfusion dependent thalassemia (NTDT) and minor) were compared to 40 eyes of twenty healthy subjects. We evaluated the vessel density (VD) in superficial capillary plexus, deep capillary plexus, radial peripapillary capillary, choriocapillaris and the foveal avascular zone area. The TDT group showed a statistically significant reduction in retinal and choriocapillaris VD respect to controls and the other groups (p < 0.05). No statistically significant difference was found in OCTA parameters between ß-thalassemia minor and controls. The NTDT group showed a significant reduction in VD in deep capillary plexus respect to controls and ß-thalassemia minor. Significant negative correlations were shown in TDT group between foveal avascular zone and hemoglobin (r = -0.437, p = 0.044) and between ferritin levels and VD of choriocapillaris (r = -0.431, p = 0.038). The OCTA parameters provided a deeper understanding on retinal and choriocapillaris vascular impairment affected by tissue hypoxia levels and the oxidative stress in different clinical phenotypes of the ß-thalassemia.

Anterior Segment-Optical Coherence Tomography features in Blau syndrome.

Blau syndrome (BS) is a rare granulomatous auto-inflammatory disease, characterized by the classic clinical triad of joints, skin and ocular involvements. Ocular manifestation usually consists in a bilateral insidious chronic anterior uveitis with a potential evolution to panuveitis. We describe the case of two siblings, an 8-years old female and a 5-years old male, with a diagnosis of BS, evaluated by Anterior Segment-Optical Coherence Tomography (AS-OCT). In the female patient, slit-lamp examination revealed bilateral anterior granulomatous uveitis and inflammatory sequelae. AS-OCT revealed high intensity reflective layers in the anterior cornea, hyperreflective dots both in the aqueous humor and in the posterior corneal surface. In the male, no signs of inflammation were detected both on slit-lamp examination and AS-OCT scans. AS-OCT is a valuable, non-invasive tool that could improve the diagnosis of ocular involvement, better characterize and follow-up corneal alterations and anterior segment features in pediatric patients with BS.

Conjunctival Carcinomas Arising in the Anophthalmic Socket.

PURPOSE: The aim of this study was to report management and outcomes of patients in which conjunctival carcinomas arose in the anophthalmic socket. METHODS: The authors retrospectively analyzed the data of patients which presented the anophthalmic socket after surgery. The clinical records of 4 patients with histological diagnosis of conjunctival carcinoma in the anophthalmic socket, referred to our Department, between January 2014 and December 2019 was collected. RESULTS: The study included 4 men (median age 58 years). A previous enucleation surgery had been performed in all patients due to previous ocular trauma for three patients (Case 1, 3, and 4) and due to a retinoblastoma for 1 patient (Case 2). All patients underwent surgical excision of the lesion and only 1 patient (case 3) underwent orbital exenteration due to the high-grade of the cancer. The most common tumor was the squamous cell carcinoma (2 cases) followed by 1 case of high-grade mucoepidermoid carcinoma and 1 case of carcinoma in situ. Systemic work-up was performed for all the patients. Conjunctival TNM, according to the American Joint Committee on Cancer TNM staging system was used to stage the lesions. The median follow-up time was 15 months. CONCLUSION: Considering the severity and rarity of these tumors, a careful ophthalmological examination of the cavity and a correct revision of the prosthesis should be mandatory in order to ensure the elimination of any macro and microscopic irregularities that can then in the long-term cause chronic inflammation of the tissues and consequently promote the growth of carcinomas.

A rare case of bilateral conjunctival Kaposi's sarcoma in a HIV-negative patient.

PURPOSE: Kaposi's sarcoma (KS) is a rare multi-centric vascular neoplasm, first described by Moritz Kaposi in 1872. It can appear in four different forms: classic, endemic, HIV-related and post-transplant form. We present an uncommon case of seronegative HIV infection patient with skin KS involving conjunctiva of both eyes. OBSERVATIONS: Firstly, right eye lesions were surgically removed. Subsequently, the patient was administered with systemic chemotherapy (doxorubicin) to treat both skin and left eye lesions. No signs of recurrence were observed at 20 months' follow-up. CONCLUSIONS: Both eyes involvement in KS is rarely described in scientific literature. Both surgery and chemotherapy could be considered valid treatment options for conjunctival KS.

Evaluation of vessel density in disorganization of retinal inner layers after resolved diabetic macular edema using optical coherence tomography angiography.

PURPOSE: To evaluate the retinal vessel density (VD) in the macular region and the foveal avascular zone (FAZ) area using optical coherence tomography angiography (OCTA) in patients with and without disorganization of retinal inner layers (DRILs) after resolution of diabetic macular edema. METHODS: Thirty-seven eyes of 37 DRIL patients (mean age 63 ± 13.97 years), 30 eyes of 30 no DRIL patients and 35 eyes of 35 controls were enrolled in this study. We evaluated the VD in the macular region of superficial capillary plexus (SCP), deep capillary plexus (DCP) and FAZ area. RESULTS: DRIL and no DRIL groups showed decreased VD in SCP and DCP (p<0.05) and a larger FAZ area (p<0.001) compared to controls. However, DRIL patients revealed a statistically significant reduction in VD of SCP (p = 0.041) and a greater FAZ area (p<0.001) with respect to no DRIL patients. We found a significant negative correlation between the VD of the foveal SCP (r = -0.414, p = 0.011), foveal DCP (r = -0.358, p = 0.025) and best corrected visual acuity (BCVA) in DRIL group. Moreover there was a significant positive correlation between the FAZ area (r = 0.425, p = 0.034) and BCVA. Therefore, in presence of DRILs lower VD values of SCP and DCP and a larger FAZ area correlated with a worse visual acuity. In no DRILs group, there was a significant negative correlation between the VD of the foveal DCP and BCVA. CONCLUSION: OCTA highlights the role of retinal vascular ischemia in the pathogenesis of DRILs. This parameter could represent an important functional predictive factor in diabetic patients.

Optical coherence tomography angiography in quiescent choroidal neovascularization associated with choroidal nevus: 5 years follow-up.

PURPOSE: To report a case of quiescent choroidal neovascularization (CNV) associated with choroidal nevus using optical coherence tomography angiography (OCTA) during 5 years follow-up. METHODS: Observational case report. RESULTS: A 53-year old woman was referred our department with diagnosis of choroidal nevus. At 1 year follow-up, fundus autofluorescence revealed hyper/hypoautofluorescent area, infrared image showed hyperreflective area and enhanced depth imaging OCT presented a shallow pigment epithelium detachment without exudation or hemorrhage. Fluorescein angiography and indocyanine green angiography suspected the presence of CNV. Finally, OCTA confirmed a quiescent CNV with an increased flow area of the vascular lesion without any changes in best-corrected visual acuity. At 20 months and 5 years follow-up, the CNV remained quiescent showing no clinical activity on structural SD-OCT and no changes in CNV morphology on OCTA. CONCLUSION: This case shows the features of quiescent CNV secondary to choroidal nevus after long-term follow-up. OCTA represents a valid imaging technique that allows to identify the CNV and to monitor its progression over time.

Primary cutaneous anaplastic large-cell lymphoma of the eyelid: report of two cases and review of the literature.

PURPOSE: Two new cases of primary cutaneous CD30+ anaplastic large-cell lymphoma (cALCL) of the eyelid are reported; these are analysed alongside existing cases to identify challenges relating to the diagnosis and management of such rare lesions. MATERIAL AND METHODS: A review of existing literature on the PubMed database is conducted using the keywords: 'eyelid lymphoid proliferations', 'lymphoma of the eyelid', and 'primary cutaneous CD30+, ALK-anaplastic large-cell lymphoma of the eyelid'. Two new cases of cALCL are reported. Cases where patients present solely with a nodular periocular lesion are analysed for recurrence and survival rate. RESULTS: Two new patients with a painless ulcerated nodule on the upper eyelid receive a confirmed diagnosis of cALCL after undergoing an excisional biopsy. The first, elderly patient has spontaneous remission; the second patient, with a concomitant chronic infection of hepatitis C virus (HCV), presents a more diffuse disease at the onset and requires radiotherapy. Together with 13 patients a primary cALCL identified from 11 previous studies, this constitutes a cohort of 15 patients. Of these, 10 present with an exclusively nodular lesion of the eyelid and four experience disease recurrence; no deaths from cALCL are reported. CONCLUSION: Differential diagnosis between primary cALCL and lymphomatoid papulosis is essential and requires careful consideration of clinical and pathologic features. Radiologic staging examination is crucial in order to exclude systemic ALCL, particularly for patients with comorbidity. Though cALCL has the pathological features of a malignant lesion, the prognosis seems favourable for patients; a relatively high percentage even experience spontaneous resolution.

The role of ultrasonography in differential diagnosis of orbital lesions.

AIMS: The aim of this study is to assess the value of ultrasound in the differential diagnosis of orbital lesions. METHODS: One hundred and thirty-eight patients with clinical features of an orbital mass were examined by orbital ultrasound prior to undergoing surgery, from January 2000 to January 2017. All patients underwent excisional or incisional orbital biopsy. The results of orbital ultrasonography were compared with the final histological diagnosis. RESULTS: Orbital lesions were localized by ultrasonography in 133/138 cases (96.4%); the false-negative rate of orbital echography was 3.6% (5/138). The nature of the orbital lesions was correctly determined by ultrasonography in 54.3% of the cases (75/138) preoperatively (true positives). In 58/138 (42%) patients, there was no correspondence between the ultrasound diagnosis and the histological diagnosis (false positives). The sensitivity of orbital ultrasonography for the detection of an orbital mass was 93.75% (CI 87.87-99.63%), while the specificity yielded no meaningful result (CI 0.00-60.24%). Moreover, the specificity of orbital ultrasonography to identify a malignant tumor falls within a CI of 0-62.72%. CONCLUSIONS: Orbital ultrasonography is a rapid and noninvasive test that is highly sensitive in displaying an orbital mass; however, the specificity in the differential diagnosis of orbital lesions is not meaningful, particularly if malignancy is suspected. The assessment of orbital diseases requires multiple diagnostic approaches to balance the strengths and weaknesses of each method.