Lateral Globe Exposure Associated With Graves Upper Eyelid Retraction: The Influence of the Superior Complex Enlargement and Proptosis.

PURPOSE: To investigate the association of the lateral exposure of the globe with proptosis and the enlargement of the superior complex (SC) (levator palpebrae superioris muscle + superior rectus muscle) in patients with Graves upper eyelid retraction. METHODS: Twenty-eight patients with upper lid retraction secondary to Graves orbitopathy and 28 controls matched by age and gender participated in the study. The variables exophthalmometry, margin-reflex distance 1, and upper lid fissure temporal and nasal areas were assessed. Coronal CT scans were evaluated for the measurement of the SC area. A 5% level of significance was assumed for analysis. RESULTS: Abnormal temporal nasal area differences (T-N), defined as values above the superior limit of the control distribution (15.58mm2) were detected in 18 patients. Multiple regression analysis showed a significant correlation of abnormal temporo-nasal asymmetries with the SC area and the proptosis (T-N = -6.7 + 0.62* proptosis + 12.3 SC area, R2 = 0.37; p = 0.003). CONCLUSION: The etiology of the enhanced lateral globe exposure is not unifactorial. SC enlargement and, to a lesser extent, proptosis are important factors correlated to lateral globe exposure.

Orbital Pigmented Epithelioid Melanocytoma Tumor Associated With Nevus of Ota (Oculodermal Melanocytosis).

The authors report a patient with nevus of Ota (oculodermal melanocytosis) who presented with pigmented epithelioid melanocytoma in the right orbit. A 36-year-old female patient attended with proptosis OD and diplopia for 3 months. MRI disclosed an expansive lesion adhered to the right inferior rectus muscle, enlargement of the superior orbital fissure, and filling of the right cavernous sinus. Excision of the orbital mass through a transconjunctival inferior fornix access disclosed a heavily pigmented lesion. Anatomopathological study revealed a diagnosis of pigmented epithelioid melanocytoma. A positron emission tomography scan did not show any metastasis. The patient was submitted to radiotherapy, had an improvement of the proptosis and diplopia, and showed no distant metastases for 3 years now. Pigmented epithelioid melanocytoma belongs to the group of heavily pigmented neoplasms, as do atypical blue nevus, which is characterized by prominent melanin production and a low degree of malignancy.

Blink Lagophthalmos in Graves Orbitopathy.

PURPOSE: To investigate the relationship between the kinematics of spontaneous blinks and the anterior area of the levator palpebrae superioris muscle in patients with Graves orbitopathy (GO). METHODS: This is a case-control study. The authors measured the margin reflex distance of the upper eyelid (margin reflex distance 1), the kinematics of spontaneous blinks, and the anterior area of levator palpebrae superioris muscle in CT coronal scans of patients with Graves upper eyelid retraction (GO) and a control group. The eye with the greatest margin reflex distance 1 was selected for analysis in each group. RESULTS: A total of 68 participants were included, with 36 in the GO group and 32 in the control group. In the GO group, the mean margin reflex distance 1 measured 6.5 mm, while in the control group, it was 3.9 mm. Almost all parameters related to the closing phase of spontaneous blinking activity, including amplitude, velocity, blinking rate, and interblink time, did not differ between the two groups. However, the effectiveness of the blink's amplitude (ratio of blink amplitude to margin reflex distance 1) and the main sequence (relationship between amplitude and velocity) were significantly reduced in the GO group compared with the control group. The area of the levator palpebrae superioris muscle was significantly larger in GO than in controls, with 71.4% of patients' muscles outside of the maximum range of the controls. CONCLUSIONS: In patients with GO, there is a reduction in blinking effectiveness, also known as blink lagophthalmos, which is a factor in the common occurrence of ocular surface symptoms. The increase in velocity with amplitude is also reduced in GO.

Spontaneous Blinking Kinematics After Frontalis Muscle Flap Advancement.

PURPOSE: To measure the spontaneous blinking metrics after blepharoptosis correction with frontalis muscle flap advancement. METHODS: A video system was employed to measure the amplitude and velocity of spontaneous blinking of 24 eyelids after the frontalis muscle flap surgery for blepharoptosis correction. A control group with no eyelid disorders was also measured. The data of 13 eyelids who had frontalis slings with autogenous fascia, which were previously collected with the same method in another study, were used for comparison. Digital images were used to measure the superior margin reflex distance and the presence of lagophthalmos during a gentle closure of the palpebral fissure. Superficial keratitis was assessed by corneal biomicroscopy. RESULTS: The mean amplitude of spontaneous blinking was 6.3 mm in controls, 2.6 mm in the frontalis flap patients, and 2.1 mm in the fascia sling group. The mean blink velocity was 133.8 mm/second in controls, 39.0 mm/second (3.7 standard error) after the frontalis flap, and 36.3 mm/second in patients with frontalis sling with fascia. For these 2 parameters, there was no statistical difference between the surgical groups, but a significant reduction when compared with the control group. No significant association was found between lagophthalmos and keratitis and the surgical procedure. CONCLUSIONS: There is no difference between the blinking metrics of eyelids operated using the frontalis muscle flap advancement technique or frontalis sling with autogenous fascia. The presence of lagophthalmos and keratitis also does not differ between the 2 procedures.

Orbital invasion of a conjunctival atypical fibroxanthoma in a patient with systemic diffuse large B-cell lymphoma.

An 88-year-old male patient presented with a large mass on the left lateral bulbar conjunctiva. The tumor appeared two months after the resection of a conjunctival atypical fibroxanthoma (AFX) performed by a cornea specialist. Magnetic resonance imaging of the orbits showed deep orbital invasion along the lateral rectus muscle. The mass and the entire conjunctival sac were totally excised with lid-sparing orbital exenteration. Histopathological analysis confirmed that the mass was an extension of the AFX. Two weeks after surgery, large B-cell lymphoma was diagnosed in the oropharynx. Chemotherapy was initiated, and after seven months of follow-up, there was no recurrence of the AFX. The authors believe that this is the first report of orbital invasion by AFX.

Blinking Parameters Do Not Normalize After Botulinum Toxin Therapy in Blepharospasm and Hemifacial Spasm Patients.

BACKGROUND: Little is known regarding changes induced by botulinum toxin injections on blinking parameters in blepharospasm (BSP) and hemifacial spasm (HFS) patients. The purpose of this study was to investigate objective changes induced by botulinum toxin (BoNT) injections on blinking parameters in BSP and HFS patients. METHODS: Thirty-seven patients with BSP and HFS were evaluated before and 30 days after receiving onabotulinumtoxinA injections. Twelve age-matched control subjects were also assessed. Pretreatment and post-treatment parameters were assessed and compared with normal controls. A high-speed camera and microlight-emitting diodes were used to register the blinking in patients and control groups. Outcomes were blinking frequency, amplitude, and maximum velocity of eyelid closure. RESULTS: BoNT injections led to a significant reduction in all parameters, compared with baseline, in BSP and on the affected side in HFS, respectively: 22% ( P < 0.001) and 20% ( P = 0.015) in amplitude; 21% ( P = 0.04) and 39% in frequency ( P = 0.002); and 41% ( P < 0.001) and 26% ( P = 0.005) in maximum closing velocity. Blinking amplitude ( P = 0.017 and P = 0.019) and velocity ( P < 0.001 for both groups) were significantly lower at 30 days on BSP and on the affected HFS side, when compared with controls. BSP and HFS patients presented a significantly lower velocity of eyelid closure, even before BoNT, compared with controls ( P = 0.004. and P < 0.001, respectively). CONCLUSIONS: Although blinking frequency became close to normal, amplitude and velocity after BoNT applications were significantly lower in BSP and on the affected side of HFS patients when compared with age-matched normal controls, demonstrating that blinking parameters do not normalize after treatment. The velocity of eyelid closure was shown to be significantly lower, even before BoNT treatment, when compared with control subjects.

The Relation of the Anterior Ethmoidal Foramen to the Cranial Base.

PURPOSE: To perform a radiological analysis of the relation of the anterior ethmoidal foramen (AEF) to the cranial base and olfactory fossa (OF). METHODS: Retrospective analysis of computed tomography coronal scans of 35 orbits of 35 patients with Graves orbitopathy. Following a standard multiplanar reconstruction of the orbit, the authors measured the vertical and the horizontal distances of the AEF to the cranial base and to the lateral border of the OF, respectively. The height of the OF was measured at the level of the AEF and at the position of Keros classification. The presence of supraorbital ethmoid cells (SOEC) was also recorded. RESULTS: The vertical distance of the AEF to the cranial base ranged from 0.1 to 7.3 mm with a mean = 2.5 mm (SD = 2.17). In 47.5% of the orbits the foramen was adjacent (<1 mm) to the cranial base. The horizontal distance of the AEF to the lateral border of the OF ranged from 2.3 to 9.5 mm (mean = 5.4 mm, SD = 1.98). The presence of an SOEC (n = 12) increased significantly both the distance from the AEF to the cranial base and to the lateral border of the OF. The height of the OF at the level of AEF is not correlated with the depth of the OF at the level of Keros classification. CONCLUSIONS: In the absence of supraorbital pneumatization, the AEF location is a dangerous landmark for the upper limit of the transconjunctival medial wall decompression.

Bilateral Intraorbital Opticmeningoceles in Joubert Syndrome.

Congenital opticmeningoceles was the term coined to describe large pseudocystic lesions of the intraorbital segment of the optic nerve. This extremely rare congenital anomaly was reported unilaterally only in nonsyndromic patients with fully developed eyes. The authors describe here a 10-month-old girl with a previous diagnosis of Joubert syndrome who presented with the same type of optic nerve malformation in OU. Molecular genetic analysis disclosed a pathogenic variant of the TMEM67 gene which is associated with various types of ciliopathies.

Effect of Rim-Off Deep Lateral Orbital Decompression on Interpalpebral Fissure Shape.

PURPOSE: The purpose of this study is to compare the palpebral fissure shape after rim-off deep lateral decompression with and without restoration of the lateral orbital rim. METHODS: Retrospective cross-sectional quantitative analysis of the palpebral fissure images of 17 patients (25 eyes) who underwent rim-off deep lateral orbital decompression without repositioning of the rim (G1) and of 20 patients (34 eyes) operated with rim restoration. Quantification of palpebral fissure images included MRD 1 and MRD 2 , two lateral mid-pupil lid margin (MPD) distances, the width of the palpebral fissure, and the height and shape of the lateral canthus. RESULTS: Proptosis reduction did not differ significantly between G1 and G2. In both groups, decompression reduced the mean values of the MRD 1 and MRD 2 , the lateral height of the upper eyelid, and the height and angle of the lateral canthus. The palpebral fissure width and lateral height of the lateral lower eyelid were not affected. There was no significant difference between the mean changes of G1 and G2. Multivariate analysis showed that the lateral canthus decrease was correlated with changes in MRD 1 and MRD 2 . CONCLUSIONS: Small changes in palpebral fissure shape after deep lateral decompression are not dependent on the presence or absence of the lateral rim.

Medial Oblique Eyelid Split Orbitotomy.

PURPOSE: The authors describe their experience with a variant of the split orbitotomy with a small medial oblique transmarginal incision for approaching a variety of lesions involving the superonasal aspect of the orbit. METHODS: Retrospective review of medical records and clinical photographs of all patients who underwent an unilateral medial oblique incision to access various intraconal lesions abutting the superomedial quadrant of the orbit. The curvature of the medial contour of the operated and contralateral eyelids were expressed with Bézier functions and compared using the R-squared coefficient of determination (R 2 ). RESULTS: Twenty-three patients were submitted to this surgical technique for approaching various unilateral lesions on the superonasal quadrant of the orbit. Excellent cosmesis was achieved in all eyelids, with almost imperceptible scars, and no ptosis or retraction. There was no significant difference between the postoperative medial contour of the operated and the contralateral eyelid, with R 2 ranging from 0.896 to 0.999, mean 0.971. CONCLUSIONS: The authors' results show that the modified eyelid split approach provides a wide exposure of the superonasal quadrant of the orbit with no risk of eyelid dysfunctions or significant scars.

Computed tomography and magnetic resonance imaging of an unusual intraconal orbital osteoma.

We report the case of a 64-year-old male patient with a 5 month history of proptosis, motility limitation and vision loss in OD. Visual acuity (VA) was 20/200 in OD and 20/20 in OS. CT showed a large, round, intraconal lesion, with bony density and no apparent connection to adjacent orbital walls. MRI showed a T1-weighted hypointense lesion surrounded by a contrast enhancing capsule. The orbital tumor was excised through a lateral orbitotomy revealing a nodular, round, osseous structure. Histological examination disclosed well-formed lamellar bone trabeculae, with no necrosis or mitosis figures. Immunohistochemical staining was negative for MDM2 and CDK4. After 3 years, there was no evidence of tumor recurrence and VA had improved to 20/30. Intraconal osteomas with no clear attachment to orbital walls are extremely rare. We are aware of a few reported cases in the lid, hand, thigh, tongue, pterygopalatine fossa and brain. To the authors' knowledge, this is the first report in English literature of an orbital intraconal osteoma without any visible relation to the orbital walls.

A quantitative analysis of the brow shape after frontalis muscle contraction.

PURPOSE: To measure the changes of the eyebrow's contour after frontalis muscle (FM) contraction. METHODS: Two consecutive pictures of the eyes and frontal region of 36 volunteers (15 men and 21 women) were obtained with the forehead relaxed and upon maximal frontalis muscle contraction. Bézier lines representing the brows' contours, obtained with the ImageJ software, were graphically sampled with a resolution of 0.025 mm. The contours of the relaxed and elevated brows were compared regarding the position of the contour peak (CP), the degree of elevation of brow central point, and the medial and lateral areas outlined between the brow contours before and after frontalis contraction. The asymmetry of the brow contour after FM contraction was calculated by the Naeije formula as (Medial-Lateral)/(Medial + Lateral). A difference of 10% or less between the lateral and medial areas was taken as a cutoff value for symmetrical brow elevation. RESULTS: In 62 (86.1%) of the eyes, the location of the brow's CP was lateral to the brow's midpoint. When the brows were raised, the CP of 60% of the brows was displaced medially and 40% laterally. The motion of the CP with medial displacement (mean = 3.7 mm) was significantly larger than those displaced laterally (mean = 1.9 mm) decreasing the number of brows with lateral peaks from 62 (86.1%) to 54 (75%). No difference was observed between the mean elevation of the central point of the brow for males (7.9 mm (3.2 SD) and females (7.4 mm, 3.0 SD). In 58.3% of the sample, the positional change of the medial and lateral sectors of the brows was symmetrical, and among the 41.7% asymmetrical cases, the elevation of the medial side was higher than the lateral in 66.7% of the brows. CONCLUSIONS: The brow changes during FM contraction concur well with the anatomic data of the FM shape. The contour of most brows is displaced toward the medial direction when the brow is elevated. The pattern of brow shape change is an indication of the FM anatomy and must be carefully analyzed before any procedure involving the FM, such as chemodenervation injections or frontalis slings for blepharoptosis correction.

Bilateral Dacryoadenitis and Central Nervous System Involvement in a Child With Kimura Disease.

Kimura's disease (KD) is a systemic inflammatory condition characterized by lymphadenopathy and subcutaneous nodules in the head and neck region. The lesions have a distinctive histopathological pattern formed by follicular hyperplasia, eosinophilic infiltrates, fibrosis, and vessel proliferation. The disease may occur at all ages but predominates among young males with autoimmune dysfunctions. Visceral and orbital involvement is uncommon. We report a girl with KD who developed bilateral enlargement of the lacrimal glands and a lesion in the left lateral ventricle of the brain indistinguishable from a central nervous system neoplasia. A biopsy of both the lacrimal gland and the lateral ventricle was consistent with KD.

Progressive chronic calvarial osteomyelitis in rhino-orbital mucormycosis associated with COVID-19.

We describe two cases of extensive indolent calvarial osteomyelitis after rhino-orbital-mucormycosis in diabetic patients previously diagnosed with COVID-19. Both patients presented with acute rhino-orbital symptoms about one month after being diagnosed with COVID-19. Treatment with intravenous liposomal Amphotericin B and prompt radical surgical debridement was instituted, but calvarial osteomyelitis ensued and persisted chronically despite maintenance of antifungal therapy and partial debridement of necrotic calvarial bone. The patients were discharged to continue antifungal therapy on a day-hospital regime. After more than 8 months of treatment, they remain with radiological signs of osteomyelitis but with no symptoms or intracranial extension of the infection. Calvarial indolent osteomyelitis secondary to mucormycosis is extremely rare, and little is known regarding its treatment. We believe it can be controlled with medical treatment and partial bony debridement although more studies are necessary to better define therapy.

Ophthalmic Rosai-Dorfman disease: a multi-centre comprehensive study.

BACKGROUND: To provide basic demographic information and clinicopathologic features of ophthalmic Rosai-Dorfman disease (RDD) with a literature review. METHODS: A multi-centre retrospective case series reviewing all patients with histopathologically confirmed ophthalmic RDD at three tertiary eye care centres between January 1993 and December 2018. RESULTS: Eleven eyes of eight patients with histopathologically confirmed ophthalmic RDD were included, with equal numbers of males and females. The median age was 40.25 years (range: 26.6-72.4). Two patients had familial RDD. The orbit was the most commonly involved site (90.9% eyes). One patient (one eye) presented with a scleral nodule, anterior uveitis and cystoid macular oedema. Visual acuity ranged from 20/25 to light perception. Six patients had an extra-nodal ophthalmic disease, and the remaining two had an associated submandibular lymphadenopathy (nodal RDD). CONCLUSIONS: Ophthalmic RDD can be the only manifestation of this systemic disease, with the orbit being the most commonly involved site, exhibiting bone destruction, intracranial and/or sinus involvement and variable degree of visual loss. Ophthalmic familial RDD represent a severe form with a malignant course. Steroid monotherapy may be inadequate to control orbital RDD; thus, combined treatment is usually necessary. A comprehensive approach to assessment and management is recommended.

Massive Orbital Infiltration and Trigeminal Enlargement in Churg-Strauss Syndrome Associated With IgG4 Plasma Cell Positivity.

The association of C-antineutrophil cytoplasmic antibody (ANCA) vasculitis and IgG4 positivity is a new condition not well described in clinical terms. The authors examined a 28-year-old man with a previous diagnosis of eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss disease, who presented with bilateral orbital inflammation. Magnetic resonance imaging revealed diffuse orbital infiltration and enlargement of the major divisions of the trigeminal nerve. Biopsy of the orbital contents showed necrotizing granulomatous vasculitis and a high number of IgG4-positive plasma cells (IgG4/IgG = 60%).

Involvement of Multiple Trigeminal Nerve Branches in IgG4-Related Orbital Disease.

PURPOSE: To describe the occurrence of multiple trigeminal nerves (TGNs) enlargement in patients with orbital IgG4-related disease. METHODS: Retrospective review of MRI findings and medical records of 6 patients (10 orbits) with orbital IgG4-related disease and enlargement of more than 1 TGN. Orbital biopsies were performed in all cases revealing the typical lymphoplasmacytic infiltrate with significant plasma cell positivity for IgG4 (IgG4+/IgG ratio ≥ 40%). Three experienced neuroradiologists reviewed the MRI sequences using a digital imaging viewer system (Horos, https://horosproject.org/). RESULTS: Bilateral involvement of at least 2 TGNs divisions was detected in all 6 patients. Enlargement of both V1 and V2 nerves was diagnosed in 5 patients, and in 3 cases, all TGN divisions were involved. V2 nerves were the most affected. In this division, all 12 infraorbital nerves were enlarged, followed by lesser palatines (10/83.3%), superior alveolar (10/83.3%), and zygomatic (6/50%). V1 and V3 nerves were less affected albeit 9 (75%) frontal branches (V1), and 50% of the inferior alveolar (V3) nerves were also enlarged. CONCLUSIONS: Widespread involvement of the TGN is an important feature of IgG4-related disease.

Deep lateral orbital decompression for Graves orbitopathy: a systematic review.

PURPOSE: To systematically review the literature on the deep lateral orbital decompression (DLD). METHODS: The authors searched the MEDLINE, Lilac, Scopus, and EMBASE databases for all articles in English, Spanish, and French that used as keywords the terms orbital decompression and lateral wall. Two articles in German were also included. Data retrieved included the number of patients and orbits operated, types of the approach employed, exophthalmometric and horizontal eye position changes, and complications. The 95% confidence intervals (CI) of the mean Hertel changes induced by the surgery were calculated from series with 15 or more data. RESULTS: Of the 204 publications initially retrieved, 131 were included. Detailed surgical techniques were analyzed from 59 articles representing 4559 procedures of 2705 patients. In 45.8% of the reports, the orbits were decompressed ab-interno. Ab-externo and rim-off techniques were used in 25.4% and 28.8% of the orbits, respectively. Mean and 95% CI intervals of Hertel changes, pooled from 15 articles, indicate that the effect of the surgery is not related to the technique and ranges from 2.5 to 4.5 mm. The rate of new onset of diplopia varied from zero to 8.6%. Several complications have been reported including dry eye, oscillopsia, temporal howling, lateral rectus damage, and bleeding. Unilateral amaurosis and subdural hematoma have been described in only one patients each. CONCLUSIONS: The low rate of new-onset diplopia is the main benefit of DLD. Prospective studies are needed to compare the rate of complications induced by the 3 main surgical techniques used.

Evaluation of botulinum toxin effects in hemifacial spasm patients: correlation between clinical rating scales and high-speed video system measurements.

The purpose of this study was to compare the scores of two clinical rating scales and high-speed video system measurements obtained during spontaneous eyelid movements in hemifacial spasm (HFS) patients before and after treatment. Patients were evaluated before and 30 days after receiving treatment with onabotulinumtoxinA injections. Using a high-speed video system, the eyelid movements were recorded bilaterally for 3 min and the energy power generated by the upper eyelid during spontaneous eyelid movements was assessed before and after treatment. The scores of the Jankovic rating scale (JRS) and Hemifacial Spasm Grading System (HSGS) were also assessed before and after treatment. The authors studied 22 patients. Significant reduction in JRS and HSGS scores and in the energy generated by the upper eyelid was observed after treatment. A power spectrum of less than 23,000 was associated with JRS and HSGS scores less than 4 and 6.25, respectively and a power spectrum greater than or equal to 23,000 was associated with JRS and HSGS scores greater than or equal to 4 and 6.25, respectively (p < 0.0001 and p = 0.0025). Rating systems are easy to use, but they may exhibit limitations in sensitivity to assess differences between distinct disease patterns and between subtle differences in treatment responses. The high-speed video system permits a greater degree of accuracy, which allows for the assessment of differences in eyelid movement patterns and would permit better tailoring of treatment to patients. However, simpler devices employing this system would need to be developed, so that it could be used in clinical practice.

Analysis of blink activity and anomalous eyelid movements in patients with hemifacial spasm.

To assess spontaneous blinking and anomalous eyelid movements in patients with hemifacial spasm with an emphasis on interocular differences. Spontaneous eyelid movements were registered bilaterally for 3 min using a high-speed video camera in 28 patients with hemifacial spasm (HFS) who had not been treated with botulinum toxin injections for at least 5 months. The degree of blink conjugacy, maximum velocity, and amplitude of the closing phase of the blinks were determined for the affected and non-affected sides. Out of the 28 subjects, 23 (82%) presented with abnormal nonconjugate spasms that were similar to blinks, and in 17 (61%), high-frequency eyelid twitches were detected between blinks on the affected eye. The rate of nonconjugate blink-like spasms ranged from 0.3 to 24.7 movements/min. With regard for conjugate blinks, there was no significant interocular difference in amplitudes or eyelid closure velocities. The amplitude and velocity were significantly lower for nonconjugate movements than for spontaneous blinks. HFS is a unique condition in which complex patterns of eyelid movements, including both conjugate and nonconjugate movements, are present. Conjugate movements correspond to spontaneous blinking, and the same metrics were observed in affected and non-affected eyes. Nonconjugate movements correspond to anomalous nonconjugate blink-like spasms and high-frequency eyelid twitches in the affected eye, both of which were characterized by lower amplitudes and velocities than were observed in conjugate movements.