Superior orbital fissure syndrome after deep lateral orbital wall decompression in Graves' ophthalmopathy.

PURPOSE: The superior orbital fissure contains cranial nerves III, IV, VI, and V1 with their three branches: frontal, lacrimal, and nasociliary. Superior orbital fissure syndrome (SOFS) is rare and can occur as a result of compression of these nerves due to trauma, bleeding, or inflammation in the retrobulbar space, but no cases of SOFS after deep lateral orbital wall decompression (DLOWD) have been reported. The aim of this paper is to describe this pathology, its possible causes, management, and outcome. METHODS: Retrospective study of 575 DLOWD in patients with disfiguring exophthalmos due to Graves' ophthalmopathy performed in our hospital between 2010 and 2023. Three cases of postoperative SOFS were identified based on clinical presentation, history, physical examination, and radiological study. All patients were observed for a minimum of 12 months. RESULTS: SOFS was diagnosed with the presence of ophthalmoplegia, ptosis, fixed and dilated pupils, hypo/anesthesia of the upper eyelid and forehead, loss of corneal reflex, and no loss of vision after DLOWD. Fractures, edema, and hemorrhages were excluded. They were treated with high-dose intravenous steroids and the patients recovered completely. CONCLUSIONS: DLOWD challenges orbital surgeons because it requires removing bones near the globe or neurovascular structures. SOFS may occur due to the proximity and increased pressure on these structures.

Surgical removal of orbital tumors by orbital approach using ultrasonic surgical system SONOPET®.

PURPOSE: Orbital tumor surgery can be challenging when the tumor is located in a very narrow surgical field and close to important structures, such as nerves or extraocular muscles that can be damaged during surgery. Advances in technology and special surgical techniques help to avoid such damage. We describe our experience using SONOPET® ultrasonic surgical aspirator to remove 12 different orbital tumors that were difficult to treat due to their poorly defined borders, adhesions, or location. METHODS: This is a retrospective case series that describes 12 operations that occurred between March 2016 to December 2018 using an ultrasonic device to debulk or remove orbital tumors. Different approaches and handpieces were used for each case depending on the location and consistency of the tumor. RESULTS: All patients experienced an improvement in preoperative signs and symptoms, pain, proptosis, diplopia, or lagophthalmos. Visual acuity, which had been reduced due to the tumor, was also recovered. There were no intraoperative or postoperative complications due to the use of the device. CONCLUSIONS: The ultrasonic aspirator is a safe, useful device that can successfully remove or debulk infiltrating orbital masses through any orbital access, regardless of their consistency. It is helpful in cases of difficult anatomical access or difficult extraction due to size or adhesions to the surrounding tissues.

Deep lateral rim-sparing orbital wall decompression in spontaneous globe subluxation associated with shallow orbits and eyelids laxity.

Purpose: To share our experience on deep lateral wall rim-sparing orbital decompression for the prevention of further spontaneous globe subluxation, in patients with shallow orbits and eyelid laxity.Methods: This is a retrospective, interventional case series review. We report the results of deep lateral wall rim-sparing orbital decompression in 7 patients with recurrent spontaneous globe subluxation, operated in our department between 2010 and 2016. The orbital morphology was established by computed tomography scan images, and all patients with shallow orbit configuration and who in addition had eyelid laxity were included. Patients with thyroid eye disease were excluded.Results: No significant intraoperative and postoperative complications were encountered. In all cases, the patients were satisfied with the aesthetic result and none reported further episodes of globe subluxation.Conclusions: Deep lateral wall rim-sparing orbital decompression is a safe and effective decompressive procedure associated with minimal complications, which can be performed successfully in patients with spontaneous globe subluxation associated with shallow orbits with enough eyelid laxity.

Obstructive sleep apnea-hypopnea syndrome (OSAHS) and glaucomatous optic neuropathy.

Obstructive sleep apnea-hypopnea syndrome (OSAHS) is becoming widely accepted as a risk factor for glaucoma. We discuss the proposed mechanism involved in the pathogenesis of glaucoma in OSAHS, and review the published data on the association between these two conditions, as well as papers regarding functional and structural tests related with glaucomatous damage. There is increasing evidence that the prevalence of glaucoma is higher in OSAHS patients, especially in those with severe disease with apnea-hypopnea index (AHI) >30, and also that sleep disorders may be more frequent in patients with glaucoma, especially in those with normal tension glaucoma (NTG). Several ophthalmic signs and symptoms have been associated with this condition. Raised intraocular pressure (IOP), possibly related to increased body mass index, thinning of retinal nerve fiber layer (RNFL), and alteration of visual field (VF) indices has been demonstrated in many studies, in patients with no history of glaucoma or evidence of glaucomatous changes in the ophthalmic examination. A correlation of AHI with RNFL and VF indices has been described in some studies. Finally, corneal thinning, suspicious glaucomatous disc changes and anomalies in electrophysiological tests such as multifocal visual evoked potential have been described in patients with OSAHS, even in patients with normal findings in the optic nerve and VF, suggesting subclinical optic nerve involvement not detectable in conventional ophthalmic examinations. The pathogenesis of optic nerve involvement has been related to vascular and mechanical factors. Vascular factors include recurrent hypoxia with increased vascular resistance, autonomic deregulation, oxidative stress and inflammation linked to hypoxia and subsequent reperfusion, decreased cerebral perfusion pressure and direct hypoxic damage to the optic nerve. Proposed mechanical factors include increased IOP at night related to supine position and obesity, raised intracranial pressure and elastic fiber depletion in the lamina cribosa and/or trabeculum. In conclusion, ophthalmic evaluation should be recommended in patients with severe OSAHS, and the presence of sleep disorders should be investigated in patients with glaucoma, especially in NTG patients and in those with progressive damage despite controlled IOP, as treatment with continuous positive airway pressure may contribute to stabilizing the progression of glaucomatous damage.

Advanced Periocular Basal Cell Carcinoma with Orbital Invasion: Update on Management and Treatment Advances.

Purpose: Basal cell carcinoma (BCC) is the most frequent malignant periocular tumor. It is associated with exposure to ultraviolet radiation, and its incidence is gradually increasing. It may occasionally display more aggressive behavior and result in orbital or intracranial invasion. Mortality from periocular BBC with orbital invasion is very low, but the associated morbidity can be significant, from disfigurement to blindness. Traditionally, these cases have been treated with orbital exenteration or with radiotherapy (RT), but in recent years, hedgehog pathway inhibitors (HPIs) have emerged, are effective in more serious cases, and are used primarily or combined with surgery, changing our perspective on the management of these patients. Methods: We studied 24 cases of periocular BCC with orbital invasion, some primary and others recurrent, which were treated between 2011 and 2021 in the same hospital. All patients had clinical or radiological evidence of orbital invasion. Orbital exenteration was performed on 9/24 of the patients (1 received vismodegib after surgery), and 12/24 were treated, surgically preserving the eyeball, with 3 of them receiving adjuvant vismodegib. Three of the twenty-four patients were treated exclusively with vismodegib (Erivedge®, Genentech). Results: One patient died due to poor tumor evolution, but the rest evolved favorably and they have had no recurrences. Vismodegib was generally well tolerated, except for in one patient who discontinued treatment due to the side effects. Conclusions: In advanced BBC with orbital invasion, mutilating surgical treatments such as exenteration or potentially vision-threatening treatments such as RT remain as options. In recent years, however, very promising new medical therapies have emerged, such as HPI, which can be used effectively instead of surgery or in combination with it, preserving the eye and vision, which implies a new approach to treatment.

Eyelid Eccrine Poroma: A Case Report and Literature Review.

Eccrine poroma is a rare benign adnexal tumor arising from intradermal cells of eccrine sweat ducts. At least two-thirds of eccrine poromas present on the extremities, most commonly on the palms and soles. They are scarcely found on the face; to date, only 11 cases of eyelid poromas have been reported in PubMed. Biopsy excision with a free margin is necessary to distinguish it from malignant lesions and avoid recurrence with possible transformation to porocarcinoma. We present the case of a 23-year-old male with a histopathological confirmation of poroma using staining with hematoxylin-eosin on the eyelid, previously clinically diagnosed with molluscum contagiosum. After four years, he has not experienced a recurrence.

Periocular Desmoplastic Malignant Melanoma and Orbital Invasion With Uncontrollable Disease: A Case Report.

Primary orbital melanoma and metastatic cutaneous melanoma of the orbit are extremely rare. Desmoplastic melanoma (DM) is an infrequent variant of melanoma that can extend from a superficial location into deep tissues by neurotropic mechanisms. A 78-year-old male was referred to us with a periocular mixed malignant melanoma (spindle cell melanoma with desmoplastic reaction) in his left lower eyelid with uncontrollable disease (orbital and inferior orbital rim invasion) despite treatment. The surgical technique consisted of an extended orbital exenteration, maxillectomy, and ethmoidectomy, with a 2 cm macroscopic surgical margin. We performed a delayed socket reconstruction with a temporalis muscle flap using a transorbital approach. The patient remained disease-free for 1.5 years with a good quality of life since exenteration surgery. At this time, he presented a recurrence in the area of the malar scar with a new orbital invasion, and finally, he died due to mediastinal, pleural, and pulmonary metastasis. The treatment of a cutaneous melanoma arising in the periocular region is a challenging reconstructive problem and it may compromise the globe and visual function.

Evaluation of the visual function in obstructive sleep apnea syndrome patients and normal-tension glaucoma by means of the multifocal visual evoked potentials.

BACKGROUND: This study was designed to objectively evaluate visual function and the causal relationship between sleep apnea and optic nerve dysfunction in patients with obstructive sleep apnea syndrome (OSAS) with or without diagnosis of normal-tension glaucoma (NTG) using multifocal visual evoked potentials (mfVEP). METHODS: This observational, cross-sectional study assessed 20 patients with recently diagnosed OSAS with or without NTG. Diagnosis of sleep apnea was based on overnight polysomnography (ApnoeScreen). All participants underwent a complete physical and ophthalmologic examination. MfVEP recordings obtained using VERIS software (Electro-Diagnostic Imaging, San Mateo, USA), SITA-standard 30-2 automated perimetry (Humphrey Visual Field Analyzer II) and optic coherence tomography (Topcon 3D OCT-1000) exams were performed to evaluate the changes. RESULTS: Abnormal mfVEP amplitudes defects (interocular and monocular probability analysis) were found in 40% of the eyes in the non-glaucoma group and in 90% of eyes of the NTG patients. As well, delayed mfVEP latencies (interocular and monocular probability analysis) were seen in 30 and 60% of the eyes of the non-glaucoma and NTG groups, respectively. The average RNFL (retinal nerve fiber layer) thickness was significantly reduced in the NTG eyes compared to the control database and the non-glaucoma eyes. On the Humphrey Visual Field total deviation analysis, all the NTG eyes showed significant clusters of abnormal points but none was detected in the non-glaucoma group eyes. However, the mfVEP amplitude and latency did not show any significant correlation with the standard perimetry and OCT variables, because the mfVEP technique was able to detect far more early visual defects in these patients. Systolic blood pressure, sleep efficiency, arousal index, mean and minimum arterial oxygen saturation (SaO(2)), time SaO(2) < 90%, oxyhemoglobin desaturation index, number of central and mixed apneas and apnea-hipopneas index were shown to be significantly correlated with mfVEP amplitude and latency. CONCLUSIONS: A significant incidence of subclinical optic nerve involvement, not detected with other structural and psychophysics diagnostic techniques was seen by means of the mfVEP. In this sense, the mfVEP may be a useful diagnostic tool in the clinic for early diagnosis and monitoring of optic nerve function abnormalities in patients with OSAS.

Isolated tarso-conjunctival superior eyelid traumatic laceration.

To report a unusual case of an isolated traumatic stellated tarso-conjunctival laceration located in the upper left eyelid without eyelid margin involvement and with normal ocular examination. A 19 year-old male wounded by a bokken (wooden katana) while practicing a sham duel had an isolated eyelid tarso-conjunctival laceration, without any other eyelid layer damage, neither skin nor muscle. Treatment was conservative without suture, by means of compressive occlusion fixing the pieces of broken tarsus in the correct position. The aim of the treatment was to avoid any wrong eyelid position secondary to healing. The eyelid maintained normal structure and movement in the follow-up at 8 months. Tarsal plate rupture is usually combined with other eyelid layer damage, which usually requires suture by layers. If eyelid skin and muscle are intact, we may choose conservative management.

Classical familial homocystinuria in an adult presenting as an isolated lens subluxation.

To report a case of late diagnosis of a classical familial homocystinuria based on an ophthalmologic examination. A 35-year-old male with Marfan-like phenotype complained of a progressive increase of myopia during the previous 2 years. Ophthalmologic exploration showed a bilateral subluxation of the lens with inferior and nasal displacement. Biochemical study detected a profile of increased amino acid levels (homocysteinemia) consistent with suspected homocystinuria. Vascular and skeletal studies ruled out Marfan syndrome. Response to treatment demonstrated B(6)-non-responsive homocystinuria. Molecular study showed compound heterozygous T353 N and D444 N mutations of the cystathionine beta-synthase (CBS) gene, and also a C667T homozygous mutation of the methylenetetrahydrofolate-reductase (MTHFR) gene. Family study showed classical homocystinuria in his father and sister, although they did not present any systemic or ocular features of the disease. Homocystinuria is a metabolic disease usually presenting at an early age as vascular, skeletal and neuropsychiatric abnormalities, as well as ectopia lentis. Our case is atypical because of the absence of thromboembolism and the mild phenotype, in spite of being B(6)-non-responsive, and the association of a rare compound heterozygous mutation of the CBS gene and also an homozygous mutation of the MTHFR gene. It is necessary to rule out homocystinuria in patients with ectopia lentis, even in the absence of systemic symptoms.

Vitreociliary Block in a Patient With Uveitis and Previous Laser Posterior Capsulotomy.

PURPOSE: To report a case of vitreociliary block (VCB) six months after a laser posterior capsulotomy (LPC). CASE REPORT: A 25-year-old man with uveitis, retinal vasoproliferative tumor, cataract, and acute angle-closure glaucoma due to pupillary seclusion, which required laser iridotomies, implantation of an Ahmed valve, phacoemulsification, and LPC. Six months after capsulotomy, he presented a generalized flattening of the anterior chamber (AC) and ocular hypertension, with patent iridotomies. Hyperechoic anterior hyaloid and hypoechoic spaces in the vitreous were seen in ultrasound imaging. The VCB did not respond to pharmacological treatment and was solved immediately after laser hyaloidotomy. CONCLUSION: There are three cases of VCB after LPC described in the literature. Our patient presented a chronic inflammatory process that generated an inflammatory membrane at the level of the anterior hyaloid with adhesion to the ciliary processes, causing posterior misdirection of the aqueous humor, decreased permeability of the anterior hyaloid, and finally, VCB.

Orbital Solitary Fibrous Tumor: Four Case Reports-Clinical and Histopathological Features.

PURPOSE: To retrospectively describe the clinical characteristics, management, and outcomes of four cases of orbital solitary fibrous tumor (SFT). In one patient, we present an ultrasonic aspirator system for tumor removal. METHODS: Four patients with orbital SFT were selected: one patient with orbital SFT, another patient with frontal and ethmoidal SFT and orbital affectation with high rates of recurrence, the third patient with frontal lobe SFT and orbital invasion with multiple recurrences, and the fourth case with a history of craniopharyngioma surgery and SFT located on the orbital apex. RESULTS: All cases showed proptosis, eye movement restriction, and, in three cases, visual acuity alteration. Different treatments were applied: in three cases, excision was performed, one of them with an ultrasonic aspirator system, and in the remaining case, an exenteration was done (in two cases, radiosurgery treatment was also applied). The immunohistochemical study revealed SFT, similar to hemangiopericytomas (HPCs). No recurrence has been observed after surgical treatment. CONCLUSION: The SFT is a spectrum of different tumors with similar histopathological characteristics. The use of immunohistochemical markers is very helpful in the diagnosis. The main problem of orbital involvement is the risk of damaging important structures adjacent to the tumor during the surgical removal. The ultrasonic aspirator system allows elimination of the tumor without damaging other orbital structures.

Giant Cell Reparative Granuloma of the Orbit: Clinicopathological Characteristics and Treatment.

Giant cell reparative granuloma (GCRG) is a rare fibroosseous lesion uncommonly seen in the orbital area. Although benign, it is known to be recurrent and locally destructive. We report two cases of GCRG of the orbit. In both cases, computed tomography revealed a heterogeneously growing well-defined mass, arising from the roof of the orbit, affecting the cortex, and invading the orbit. In the first case, the mass extended into the anterior cranial fossa. Magnetic resonance imaging with gadolinium showed, in both cases, a cystic character of the lesion with fluid levels. The surgical treatment was performed via an upper crease incision. An ultrasonic aspirator system was used to remove the tumor tissue and its extension into cranial fossa. Careful histopathologic analysis established the diagnosis of GCRG. Symptoms resolved completely with no evidence of recurrence after a follow-up of 18 and 14 months, respectively. We present the clinicopathological and radiological findings, and we describe the surgical approach. As a rare entity, GCRG of the orbit should be considered in differential diagnosis of fibroosseous orbital masses. Complete surgical excision carries a low risk of recurrence.

Conjunctival Tarsal Actinic Keratosis Treated with Interferon Alfa-2b: A Rare Case Report and Literature Review.

Conjunctival neoplasia is one of the most frequent tumors in the eye. Actinic keratosis (AK) or solar keratosis is a precancerous lesion that is included with other epithelial tumors. This alteration does not break the basal membrane. There is enough evidence of successful outcomes to consider interferon alfa-2b (IFN alfa-2b) as the first choice of treatment for this type of tumors. In addition, side effects are mild and uncommon. We report a case in an 83-year-old woman who was referred to evaluate a leukoplakia in the tarsal conjunctiva of the lower left eyelid that measured 1 cm in diameter. Pathological study revealed AK. After the INF alfa-2b treatment, we observed conjunctival hyperemia, noninfiltrated upper nasal de-epithelization, and inferior nasal bulla. AK with presentation in conjunctiva is rarely described and in tarsal conjunctiva is exceptional. It is the first case published with only tarsal conjunctiva affectation.

Localized Bilateral Superior and Inferior Orbital Neurofibroma in the Absence of Neurofibromatosis.

Localized or isolated neurofibromas are peripheral nerve sheath tumors. They are rare in the orbit and occur without a systemic neurofibromatosis. There are few cases of bilateral tumors reported but none affecting both supraorbital and infraorbital nerves. We report a 45-year-old female who presented an extraconal mass in the right orbit as an incidental finding in a head computer tomography, without ocular symptoms. Magnetic resonance image showed a well-defined oval mass in the right supraorbital and infraorbital nerves, of similar characteristics, as well as smaller masses in the left supraorbital and infraorbital nerves. A progressive increase in size of the left supraorbital and infraorbital tumor motivated their surgical excision. The histological result was compatible with a neurofibroma. These uncommon orbital tumors are slow growing and affect the sensory nerves of the trigeminal nerve. Neurofibromas usually present progressive symptoms due to the orbital mass, proptosis, or visual changes although not in this case. Surgical removal is the only definitive treatment.

Management of congenital glaucoma in neurofibromatosis type 1: a report of two cases.

The most frequent ophthalmic lesions associated to neurofibromatosis type 1 include iris Lisch nodules, optic nerve gliomas, and neurofibromas located on the eyelid, conjunctiva or orbit. Glaucoma is much less frequent, and it may be difficult to diagnose and treat. We present two patients with neurofibromatosis type 1 and associated congenital glaucoma. In case 1, the glaucoma was present at birth, and was the first symptom of the disease. It was surgically treated by means of an Ahmed glaucoma valve implantation, with good intraocular pressure (IOP) control after 13 months of follow-up. In case 2, the glaucoma appeared at 5 months old, and a trabeculectomy was initially performed; it failed 2 years later and an Ahmed glaucoma valve was then implanted, with adequate IOP control after 7 years of follow-up. Both children were males, and in both cases there were orbital neurofibromas and a dysplasia of the greater wing of the sphenoid of the same side. In case 2, orbital enlargement surgery was also performed at 4 years old. Congenital glaucoma management in the context of neurofibromatosis is very complex due to the frequent association of orbital and eyelid tumors and bone dysplasia, and its prognosis is usually poor. We present two cases in which good control of IOP has been achieved with Ahmed glaucoma valve implantation after 13 months and 7 years of follow-up.

Iris Vascular Malformation with 360-Degree Iridocorneal Angle Affectation.

Vascular iris lesions are rare and can sometimes be associated to systemic vascular lesions. They usually cause spontaneous recurrent hyphema. The differential diagnosis should be considered primarily with iris rubeosis and with highly vascular tumors as iris melanoma. Generally, vascular lesions of hemangioma type are located in the iris without extension to iridocorneal angle. We present a case of a 77-year-old male with an iris vascular lesion suggestive of racemose hemangioma, who is asymptomatic, and with 360-degree iridocorneal angle affectation showing no lesions at any other location.

Eyelid Eccrine Poroma: A Case Report and Review of Literature.

Poroma is a rare benign tumor of the epidermal sweat duct unit with predilection for the head and neck. Only six cases with eyelid location have been described in the literature (PubMed). A 34-year-old male presented with a single tumor on the left upper eyelid. It was skin-colored, nodular, solid, tender with some telangiectatic vessels, and showed no ulcerated lesion. Clinical diagnosis was basal cell carcinoma. This type of lesion can mimic a malignancy. Complete excisional biopsy revealed features consistent with eccrine poroma. After three year of follow up, no recurrence was observed. The authors reviewed all the cases reported in the literature and made a summary comparing them.

Orbital-Conjunctival-Eyelid Hemorrhage-Hematoma due to Inappropriate use of FFP2/NK95 Facial Mask in COVID-19.

A non-traumatic or spontaneous orbital hemorrhage (NTOH) is usually caused by the presence of an orbital mass, an inflammation, an infection, a bleeding disorder or those called idiopathic. This entity usually affects elderly adults and some risk factors can be identified. The NTOH normally acts like a benign and self-limited process, but attending to its anatomical pattern, may need specific management. A 64-year-old male referred to us with sudden binocular double vision, without loss of visual acuity (VA) or pain immediately after fitting the FFP2/NK95 facial mask for air protection during the pandemic COVID-19. He presented sudden orbital-subconjunctival-eyelid cutaneous hemorrhage-hematoma with conjunctival protrusion from the palpebral fissure without proptosis. There was also limitation in adduction and a cutaneous hematoma in the inner third of the lower eyelid. After 48 hours, the diplopia had resolved and ocular motility was completely re-established with persistence of a massive hyposphagma. No radiological image test was performed due to the COVID-19 epidemiological situation, as the patient was in good systemic situation and it was not a vital emergency. The evolution was favorable with conservative treatment, cold application, and moisturizing eye drops. After 10 days, the bruising was almost completely gone. During the ongoing novel coronavirus disease (COVID-19) pandemic caused by the novel enveloped RNA virus named severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), face mask use has drastically increased among the healthcare professionals and the general population. The importance of this case lies in the new adverse effects caused by the misuse of mandatory face masks in the general population.

Evaluation of Anxiety Levels in Patients Undergoing Intravitreal Injections and Associated Risk Factors Related to the Disease.

PURPOSE: To analyze patients' anxiety levels using the Visual Analog Scale for Anxiety (VASA), in regard to intravitreal injection treatment and to determine possible associated risk factors related to the disease and treatment characteristics. METHODS: Cross-sectional observational study with consecutive sampling of patients who were going to receive an intravitreal injection. Subjects completed the VASA prior to the procedure, and afterwards, their data were collected from the electronic medical history. Analysis was performed through a linear regression model. RESULTS: Fifty-five men and forty-seven women were enrolled. The mean age was 73.9 ± 12.4 years (mean ± standard deviation (SD)), and the mean ± SD of previous injections was 12.8 ± 12. The most frequent pathologies found were age-related macular degeneration with 46.1% and diabetic macular edema with 36.3%. The median of anxiety levels measured in millimeters (mm) was 16 (interquartile range: 0-48). In univariate models, women presented a mean of 10.8 mm of anxiety more than men (p=0.03). The adjusted multivariate analysis demonstrated that younger patients declared higher anxiety levels (p=0.036). No significant association was found between the best corrected visual acuity (BCVA) on the day of the injection, the change in BCVA since the beginning of the treatment or the number of injections received, and the registered anxiety levels. CONCLUSIONS: Sex and age may have an influence on anxiety levels. BCVA and the number ofinjections received did not seem to have an influence on our patients anxiety levels.