Surgery as a safe and effective treatment option for spheno-orbital meningioma: a systematic review and meta-analysis of surgical techniques and outcomes.

PURPOSE: The effectiveness and safety of surgery for spheno-orbital meningiomas remains subject of debate, as studies often describe different surgical approaches and reconstruction techniques with very heterogeneous outcomes. We aimed to systematically summarize and analyse the literature on spheno-orbital meningiomas regarding presenting symptoms, surgical techniques, outcomes and complications. METHODS: Studies were retrieved from eight databases. Original articles were included if in ≥5 patients presenting symptoms, surgical treatment and outcomes were described. Fixed- and random-effects meta-analysis was performed to estimate weighted percentages with 95%CIs of presenting symptoms, outcomes and complications. RESULTS: Thirty-eight articles were included describing 1486 patients. Proptosis was the most common presenting symptom (84%; 95%CI 76-91%), followed by unilateral visual acuity deficits (46%; 95%CI 40-51%) and visual field deficits (31%; 95%CI 20-43%). In 35/38 studies (92%), a pterional craniotomy was used. Decompression of the optic canal (82%) and the superior orbital fissure (66%) was most often performed, and usually dural (47%) and bony defects (76%) were reconstructed. In almost all patients, visual acuity (91%; 95%CI 86-96%), visual fields (87%; 95%CI 70-99%) and proptosis (96%; 95%CI 90-100%) improved. Furthermore, surgery showed improvement in 96% (95%CI 78-100%) for both diplopia and ophthalmoplegia. The most common surgical complications were hypesthesia (19%; 95%CI 10-30%), ptosis and diplopia (both 17%; 95%CI, respectively, 10-26% and 5-33%) and ophthalmoplegia (16%; 95%CI 10-24). CONCLUSION: Patients with spheno-orbital meningioma usually present with proptosis or unilateral decreased visual acuity. Surgery shows to be effective in improving visual acuity and visual field deficits with mostly minor and well-tolerated complications.

Visual outcomes endorse surgery of patients with spheno-orbital meningioma with minimal visual impairment or hyperostosis.

ABTRACT: BACKGROUND: Most spheno-orbital meningioma series span multiple decades, and predictors of visual outcomes have not yet been systemically assessed. We describe visual outcomes in a recent cohort and assess predictors of postoperative visual outcomes. METHODS: Consecutive case series operated by a team of a neurosurgeon and orbital surgeon between May 2015 and January 2019. Best corrected visual acuity (BCVA), visual fields (static perimetry), and relative proptosis were measured preoperatively and postoperatively at 3/6/12 months after which it was assessed yearly. Predictors were assessed with linear regression analysis. RESULTS: Nineteen patients (all WHO grade I) were operated by the pterional approach (median follow-up 2.4 years). Preoperative visual acuity deficits (n = 10) normalized in 70% and improved in 10% (median preoperative: 0.8, postoperative: 1.2, p = 0.021). Preoperative visual field deficits (n = 8) normalized in all patients (preoperative: - 6.5 dB, postoperative: - 1.5 dB, p = 0.008). Preoperative proptosis (n = 16) normalized in 44% and improved in 56% (preoperative: 5 mm, postoperative: 2 mm, p < 0.001). BCVA and visual fields remained stable at longer follow-up in 95% of patients, while 21% showed progression of proptosis. Predictors for worse longer-term (> 12 months) BCVA were worse preoperative BCVA (p = 0.002) and diagnosis of multiple meningioma (p = 0.021). Predictors for worse longer-term visual fields were higher diameter of hyperostosis (p = 0.009) and higher Simpson grade (p = 0.032). Predictor for short-term (3 months) proptosis was preoperative proptosis (p = 0.006). CONCLUSION: We recommend surgery, even of patients with minimal visual impairment or hyperostosis, as patients who present with deteriorated visual function or extensive hyperostosis are less likely to have postoperative visual outcomes restored to normal.

The feasibility of quantitative MRI of extra-ocular muscles in myasthenia gravis and Graves' orbitopathy.

Although quantitative MRI can be instrumental in the diagnosis and assessment of disease progression in orbital diseases involving the extra-ocular muscles (EOM), acquisition can be challenging as EOM are small and prone to eye-motion artefacts. We explored the feasibility of assessing fat fractions (FF), muscle volumes and water T2 (T2water ) of EOM in healthy controls (HC), myasthenia gravis (MG) and Graves' orbitopathy (GO) patients. FF, EOM volumes and T2water values were determined in 12 HC (aged 22-65 years), 11 MG (aged 28-71 years) and six GO (aged 28-64 years) patients at 7 T using Dixon and multi-echo spin-echo sequences. The EOM were semi-automatically 3D-segmented by two independent observers. MANOVA and t-tests were used to assess differences in FF, T2water and volume of EOM between groups (P < .05). Bland-Altman limits of agreement (LoA) were used to assess the reproducibility of segmentations and Dixon scans. The scans were well tolerated by all subjects. The bias in FF between the repeated Dixon scans was -0.7% (LoA: ±2.1%) for the different observers; the bias in FF was -0.3% (LoA: ±2.8%) and 0.03 cm3 (LoA: ± 0.36 cm3 ) for volume. Mean FF of EOM in MG (14.1% ± 1.6%) was higher than in HC (10.4% ± 2.5%). Mean muscle volume was higher in both GO (1.2 ± 0.4 cm3 ) and MG (0.8 ± 0.2 cm3 ) compared with HC (0.6 ± 0.2 cm3 ). The average T2water for all EOM was 24.6 ± 4.0 ms for HC, 24.0 ± 4.7 ms for MG patients and 27.4 ± 4.2 ms for the GO patient. Quantitative MRI at 7 T is feasible for measuring FF and muscle volumes of EOM in HC, MG and GO patients. The measured T2water was on average comparable with skeletal muscle, although with higher variation between subjects. The increased FF in the EOM in MG patients suggests that EOM involvement in MG is accompanied by fat replacement. The unexpected EOM volume increase in MG may provide novel insights into underlying pathophysiological processes.

MR and CT Imaging of the Normal Eyelid and its Application in Eyelid Tumors.

T-staging of most eyelid malignancies includes the assessment of the integrity of the tarsal plate and orbital septum, which are not clinically accessible. Given the contribution of MRI in the characterization of orbital tumors and establishing their relations to nearby structures, we assessed its value in identifying different eyelid structures in 38 normal eyelids and evaluating tumor extension in three cases of eyelid tumors. As not all patients can receive an MRI, we evaluated those same structures on CT and compared both results. All eyelid structures were identified on MRI and CT, except for the conjunctiva on both techniques and for the tarsal muscles on CT. Histopathology confirmed the MRI findings of orbital septum invasion in one patient, and the MRI findings of intact tarsus and orbital septum in another patient. Histopathology could not confirm or exclude tarsal invasion seen on MRI on two patients. Although imaging the eyelid is challenging, the identification of most eyelid structures is possible with MRI and, to a lesser extent, with CT and can, therefore, have an important contribution to the T-staging of eyelid tumors, which may improve treatment planning and outcome.

Patients With Isolated Craniofacial Dysplasia Report Better Quality of Life Compared With Those With Craniofacial Dysplasia and Extracranial Involvement.

PURPOSE: Craniofacial fibrous dysplasia (CFD) is a subtype of fibrous dysplasia/McCune-Albright syndrome (FD/MAS) characterized by FD lesions in one or more of the skull bones. The orbit is often involved, with facial pain, facial deformity, and increased risk of compressive optic neuropathy as associated clinical manifestations possibly leading to altered illness perceptions and impairments in quality of life(QoL). The aim of this study was to evaluate illness perceptions and QoL in patients with CFD among our FD/MAS cohort. METHODS: One hundred ninety-one patients were included. Illness perceptions and QoL were assessed by using validated questionnaires, that is, the Illness Perceptions Questionnaire-Revised and the Short-Form 36. Patients were first grouped as CFD versus non-CFD, a second selection was based on the presence of "Isolated CFD" versus "CFD+PFD/MAS." Non-CFD patients were grouped as monostotic fibrous dysplasia "MFD" versus polyostotic "PFD/MAS." RESULTS: Patients with isolated CFD attributed less symptoms to their disease compared with patients with CFD+PFD/MAS (p < 0.05). Furthermore, patients with isolated CFD reported better QoL on all domains (except role emotional and mental health) compared with patients with CFD+PFD/MAS (p < 0.05). Patients with isolated CFD also reported better QoL compared with non-CFD groups (on 3 out of 8 subscales) (p < 0.05). CONCLUSIONS: Patients with isolated CFD attribute less symptoms to their disease and report better QoL compared with patients with CFD with extracranial involvement or FD without cranial involvement. These findings indicate that craniofacial involvement alone is not sufficient to cause negative illness perceptions and impairments in QoL. Therefore, it can be postulated that isolated CFD should be considered a unique patient subtype within the spectrum of FD/MAS patients.

A pan-inflammatory microRNA-cluster is associated with orbital non-Hodgkin lymphoma and idiopathic orbital inflammation.

Non-Hodgkin orbital lymphoma (NHOL) and idiopathic orbital inflammation (IOI) are common orbital conditions with largely unknown pathophysiology that can be difficult to diagnose. In this study we aim to identify serum miRNAs associated with NHOL and IOI. We performed OpenArray® miRNA profiling in 33 patients and controls. Differentially expressed miRNAs were technically validated across technology platforms and replicated in an additional cohort of 32 patients and controls. We identified and independently validated a serum miRNA profile of NHOL that was remarkably similar to IOI and characterized by an increased expression of a cluster of eight miRNAs. Pathway enrichment analysis indicated that the miRNA-cluster is associated with immune-mediated pathways, which we supported by demonstrating the elevated expression of this cluster in serum of patients with other inflammatory conditions. The cluster contained miR-148a, a key driver of B-cell tolerance, and miR-365 that correlated with serum IgG and IgM concentrations. In addition, miR-29a and miR-223 were associated with blood lymphocyte and neutrophil populations, respectively. NHOL and IOI are characterized by an abnormal serum miRNA-cluster associated with immune pathway activation and linked to B cell and neutrophil dysfunction.

Two Late Recurrences of Conjunctival Melanoma.

PURPOSE: To report a patient who developed two late recurrences of conjunctival melanoma (CoM), of which one occurred after orbital exenteration. METHODS: We describe the case of a patient based on clinical and histopathological examination. RESULTS: A 52-year-old patient was treated with local excision and cryotherapy for a CoM with primary acquired melanosis (PAM) near the limbus of the right eye. Twenty-one years later, a recurrence developed in the superior fornix of the same eye in an area with widespread PAM; an orbital exenteration was performed. After another 4 years, a painful nodule developed subcutaneously at the inferior margin of the right orbital socket. Pathology showed a recurrence of CoM with a BRAF V600K mutation, similar to both of the previous lesions (of 25 and 4 years earlier). The nodule was excised without additional therapy. No recurrences or metastases have been observed in the next 2.5 years. The proposed mechanism for the recurrence after surgery could be via dormant tumor cells that have spread prior to the procedure or via residual intraepithelial malignant melanocytes. CONCLUSION: Very late recurrences of CoM are rare but may occur. Our case illustrates the need for long-term awareness of doctors and patients, even after extensive surgical procedures such as orbital exenteration.

Endoscopic dacryocystorhinostomy: long-term experience and outcomes.

PURPOSE: To report our experience in endoscopic dacryocystorhinostomy in treating nasolacrimal duct obstruction over a 14-year period. METHODS: Retrospective case series carried out over a 14-year period from 1999 to 2014. A total of 624 endoscopic dacryocystorhinostomy procedures were performed in 442 patients who were diagnosed with persistent epiphora. PARTICIPANTS: In total, 624 procedures in a total of 442 patients (<18 yo: 36 and >18 yo: 406) were included. Patients' records were assessed for demographics, intra- and postoperative complications, outcome and follow-up. RESULTS: In total, 442 patients underwent an endoscopic dacryocystorhinostomy. There were 342 females (77.3%) and 100 males with a mean age of 50.2 years. All patients presented with epiphora: 33 congenital (7.5%), 357 acquired (80.7%) and 52 functional cases (11.8%). The adult dacryocystorhinostomy (n = 577) had an anatomical success of 90.1%. Children's group (<18 yo) n = 36 with a total of 47 procedures and an anatomical success of 91.5%. Functional cases (all adults - n = 52) showed a 91% benefit after operation. In 624 DCRs, we found a complication rate of 3.2%. CONCLUSION: Endoscopic dacryocystorhinostomy offers good success rates with a safety profile and efficacy equivalent to the traditional external approach is an effective treatment with good outcome results in adults and children with persistent or functional epiphora.

Clinical evaluation of ultra-high-field MRI for three-dimensional visualisation of tumour size in uveal melanoma patients, with direct relevance to treatment planning.

OBJECTIVES: To assess the tumour dimensions in uveal melanoma patients using 7-T ocular MRI and compare these values with conventional ultrasound imaging to provide improved information for treatment options. MATERIALS AND METHODS: Ten uveal melanoma patients were examined on a 7-T MRI system using a custom-built eye coil and dedicated 3D scan sequences to minimise eye-motion-induced image artefacts. The maximum tumour prominence was estimated from the three-dimensional images and compared with the standard clinical evaluation from 2D ultrasound images. RESULTS: The MRI protocols resulted in high-resolution motion-free images of the eye in which the tumour and surrounding tissues could clearly be discriminated. For eight of the ten patients the MR images showed a slightly different value of tumour prominence (average 1.0 mm difference) compared to the ultrasound measurements, which can be attributed to the oblique cuts through the tumour made by the ultrasound. For two of these patients the more accurate results from the MR images changed the treatment plan, with the smaller tumour dimensions making them eligible for eye-preserving therapy. CONCLUSION: High-field ocular MRI can yield a more accurate measurement of the tumour dimensions than conventional ultrasound, which can result in significant changes in the prescribed treatment.

Parallax-free exophthalmometry: a comprehensive review of the literature on clinical exophthalmometry and the introduction of the first parallax-free exophthalmometer.

PURPOSE: To present the first parallax-free exophthalmometer design. BACKGROUND: Exophthalmometry is an important clinical tool. We provide a historic overview of clinical exophthalmometer designs, and we review current problems encountered in exophthalmometry. METHODS: We present a new and parallax-free exophthalmometer design that we have evaluated in 49 patients visiting our orbital clinic. RESULTS: The mean age of the patients was 49.8 years and 72% were female. The Pearson interobserver variation was 0.97, and 94% of the Hertel values measured by the two observers were within the limits (1.6 mm) of agreement. CONCLUSION: This meter appears to be a reliable instrument for exophthalmometry. It is the first instrument that allows for a complete parallax-free measurement.

[A man with optic disc swelling]. / Een man met onscherpe papillen.

A 60-year-old man presented with deterioration of vision in his right eye. Funduscopic examination showed bilateral optic disc swelling. Ultrasonography revealed optic disc drusen. Optic neuropathy due to optic disc drusen can cause transient and permanent visual impairment.

Lutein and zeaxanthin measured separately in the living human retina with fundus reflectometry.

PURPOSE: To separately measure the optical densities of lutein (L) and zeaxanthin (Z) in the human retina in vivo. L and Z are the basic constituents of the macular pigment (MP). METHODS: Spectral fundus reflectance was measured in 23 subjects (group 1) at 0 degrees , 1 degrees , 2 degrees , 4 degrees , and 8 degrees eccentricity with a modified macular pigment reflectometer. A model generated the optical densities of L (LOD) and Z (ZOD), using their slightly different absorption spectra. Three other subjects (group 2) took 20 mg/d zeaxanthin for 6 months; they were measured approximately monthly for 18 months. RESULTS: Mean LOD for group 1 at the central fovea was 0.200 +/- 0.061 (range, 0.085-0.305), mean ZOD was 0.494 +/- 0.169 (range, 0.169-0.806), resulting in a mean Z fraction [ZOD/(LOD + ZOD)] of 0.71. ZOD dropped faster toward the periphery than LOD, measuring 0.044 and 0.010 (Z fraction 0.18) at 8 degrees , respectively. Zeaxanthin supplementation in group 2 caused a significant increase in ZOD, and no or minor changes in LOD. ZOD further increased over a 10-month period after supplementation in all subjects. CONCLUSIONS: LOD and ZOD had different spatial profiles that, apart from scaling factors, showed similarity to in vitro literature data. Supplementation with Z caused LOD to decrease and ZOD to increase. These results strongly suggest that the optical densities of L and Z can be assessed in vivo by fundus reflectometry, opening new ways of investigating the putative protective roles of L and Z in retinal disease.