Prognostic factors and clinical features of rhino-orbital-mucormycosis cases: an update for patient and visual survivals.

AIM: To determine the frequency of patients' vision survival and prognostic factors and evaluate clinical features in rhino-orbital mucormycosis. METHODS: Forty-three eyes of 43 patients followed up with orbital mucormycosis infections were included in the study. Demographic characteristics of the patients, symptoms at admission, ophthalmologic and non-ophthalmologic examination findings, clinical findings during follow-up, medical and surgical procedures, and complications were recorded. Patient survival was determined by assessing the incidence of mortality, and vision survival was defined as achieving a final visual acuity of at least light perception. RESULTS: Twenty-seven (62.8%) patients were male, and 16 (37.2%) were female. When the underlying disease status of the patients was examined, it was observed that all patients had an underlying disease and diabetes constituted the majority (65.2%). Periorbital swelling (69.8%) and ophthalmoplegia (53.5%) were the most common symptoms and findings at the admission of patients with mucormycosis infection. The disease resulted in death in 22 (51.2%) patients. The presence of fever and shorter duration of antifungal therapy were associated with lower patient survival. Exenteration surgery was not found to be associated with the survival of the patients. Frozen eye, loss of pupillary light reflex, and development of central retinal artery occlusion were associated with lower vision survival. CONCLUSION: This study presents one of the most extensive patient series in the literature on rhino-orbital mucormycosis. Knowing the patients' symptoms at the time of admission and the clinical findings during the infection process will increase awareness about the disease.

Survival from Rhino-Orbital-Cerebral Mucormycosis in SARS-CoV-2-Positive Diabetic Patients: Two Case Reports.

Introduction: Rhino-orbital-cerebral mucormycosis (ROCM) is a rare angioinvasive fungal infection known to be associated with high morbidity and over 50% mortality. ROCM is becoming more common due to an increase in predisposing immunocompromising comorbidities as well as COVID-19. Case Presentations: We report 2 cases - a 75-year-old woman with diabetes and a 39-year-old man with recurrent diabetic ketoacidosis. Both presented initially with acute sinonasal symptoms, were positive for SARS-CoV-2, and diagnosed with acute ROCM. Both underwent mutilating surgical therapy as well as high-dose amphotericin B treatment. With continued oral antifungal treatment, patient 1 showed stable symptoms despite radiographically increasing disease and died of urosepsis 5 months after first surgery. With posaconazole treatment, patient 2 recovered from the disease and showed no clinical sign of disease progression after 1 year. Conclusion: Despite the rarity of the disease, ROCM should be considered if the findings of clinical and radiological examination fit, so that a delay in treatment initiation can be avoided. As our both cases show, survival from ROCM is possible - albeit at a high cost.

Planet of the apex.

A 63-year-old man with diabetes presented with unilateral ptosis and an exotropia. A diagnosis of isolated diabetic III nerve palsy was made. Subsequent neuro-ophthalmologic evaluation showed multiple cranial nerves involvement consistent with a diagnosis of orbital apex syndrome. Review of past medical history was significant for a previous nasopharyngeal carcinoma, and biopsy of the involved site was consistent with tumor recurrence. This case highlights the importance of correct medical history taking and anatomo-clinical correlation in neuro-ophthalmology.

Atypical Asymmetric Presentation of Severe Graves' Orbitopathy.

Graves' disease is a self-limiting autoimmune thyroid disorder caused by stimulating antibodies to the thyroid-stimulating hormone receptor. It usually affects middle-aged females in the fourth to sixth decade of life. It is distinguished by keratopathy, chemosis, proptosis, and eyelid swelling, in addition to ocular discomfort. A total of 3-5% of cases present with a severe form of Graves' orbitopathy, which manifests with diminution of vision, optic nerve compression, optic neuropathy, and exposure keratopathy. We describe a case of a 34-year-old female patient who presented with the chief complaint of rapid deterioration of vision over a period of three months in the right eye. Ocular examination revealed proptosis, widened palpebral aperture, elevation of intra-ocular pressure (IOP) in the upgaze, restricted eye movements, and signs of optic nerve compression. Findings were confirmed on a CT scan of the orbit. The unusual presentation in this case was that she had rapid, significant deterioration of vision in the right eye, with a progression of proptosis more marked in the contralateral eye. This underlies the importance of thoroughly examining for any possible orbital apex syndrome in both eyes, not just the eye with marked proptosis. The patient, being reluctant for orbital decompression, was prescribed IV methylprednisolone 1 g for three consecutive days, which reduced her proptosis and improved her vision. This acted as a temporary measure to increase the duration of the surgical window until the time the patient undergoes the surgery.

Orbital fibrous capsule formation with abscess found 3 years after penetrating injury from a wooden chopstick.

Intraorbital wooden foreign bodies are sometimes difficult to diagnose because of nonspecific clinical manifestations and diversity of imaging characteristics. We herein report a case involving a 72-year-old woman with a history of trauma induced by a coated wooden chopstick 3 years prior. Two years after the incident, computed tomography (CT) scan revealed an intraorbital mass that was initially diagnosed as an intraorbital hemangioma. The patient presented with hyperemia, impairment of ocular movement, and optic neuropathy in her right eye. Magnetic resonance imaging (MRI) showed granulation tissue and an abscess around a foreign body, which was compressing the eyeball. Surgical extraction of the foreign body was performed, leading to resolution of symptoms. The depiction of wooden foreign bodies by imaging is complicated and affected by several factors, increasing the risk of delayed diagnosis. To avoid permanent sequelae, MRI might be helpful because its imaging capabilities are superior to those of CT.

Orbital Apex Syndrome Due to Aspergillus flavus Infection in Immunocompetent Patients: A Report of Two Cases.

Aspergillus species are fungi that are commonly found in soil and decaying vegetation and have the potential to cause an orbital apex syndrome that is marked by ophthalmoplegia or vision loss. We report the clinical and investigational findings and outcomes of two patients with orbital apex syndrome. The first patient was a 26-year-old female, premorbidly healthy, who presented with a gradually increasing proptosis of the left eye with a reduction in vision. An MRI revealed findings consistent with proptosis, pansinusitis with a soft tissue opacity involving the left orbital apex with optic nerve compression, extending to the cavernous sinus with an associated temporal meningeal enhancement. Following functional endoscopic sinus surgery (FESS), Aspergillus flavus was grown in culture, and oral voriconazole was initiated. The second patient was a 53-year-old male who presented with bilateral reduction of vision and ptosis, proptosis with total ophthalmoplegia (third, fourth, and sixth nerve palsies) of the right eye. An MRI study revealed extensive involvement of the apex of the right orbit, the right cavernous sinus, the medial aspect of the left cavernous sinus, and the pituitary gland. A FESS was done, and the histopathology specimen was suggestive of aspergillosis, and the tissue fungal polymerase chain reaction (PCR) test was positive for Aspergillus flavus. He was treated with amphotericin B and oral voriconazole with significant improvement. Physicians need to have a high index of suspicion for invasive fungal sino-orbital infections, even in immunocompetent patients. The presence of nasal congestion, recurrent sinusitis, facial pain, headache, orbital cellulitis, proptosis, or ophthalmoplegia should prompt early investigations.

Orbital Apex Syndrome caused by invasive aspergillosis in patient with post-Covid-19 infection - A case report.

INTRODUCTION AND IMPORTANCE: Orbital Apex Syndrome (OAS) are characterized by multiple symptoms, such as impaired eye movement, periorbital pain, and visual disturbance. AS symptoms may be caused by inflammation, infection, neoplasm, or a vascular lesion, potentially involving a variety of nerves, such as the optic, oculomotor, trochlear or abducens, or the ophthalmic branch of the trigeminal nerve. However, OAS caused by invasive aspergillosis in post-COVID patient is a very rare phenomenon. CASE PRESENTATION: A 43-year-old male with a history of diabetes mellitus and hypertension who had recently recovered from a COVID-19 infection developed blurred vision on the left eye field, followed by impaired vision on left eye field for 2 months then retro-orbital pain for a further 3 months. The blurring of vision and headache developed soon after recovering from COVID-19 and was progressive in left eye field. He denied any symptoms of diplopia, scalp tenderness, weight loss, or jaw claudication. The patient was treated with IV methylprednisolone for 3 days with as diagnosis of optic neuritis, followed by a course of oral corticosteroid therapy (prednisolone, starting at 60 mg for 2 days and then tapered for 1 month), which produced transient relief of symptoms that recurred when prednisone was discontinued. Then repeat MRI was perform with no evidence of lesion; treated again in a line of optic neuritis and symptoms relief transiently. After reoccurrence of symptoms repeat MRI was perform which showed a heterogeneously enhancing intermediate signal intensity lesion in the left orbital apex. The lesion was encasing and compressing the left optic nerve, without abnormal signal intensity or contrast enhancement within the left optic nerve either proximal or distal to the lesion. The lesion was contiguous with focal asymmetric enhancement in the left cavernous sinus. No inflammatory changes were seen in the orbital fat. CLINICAL DISCUSSION: OAS due to invasive fungal infection is uncommon and most often caused by Mucorales spp., or Aspergillus, particularly in those with immunocompromising conditions or uncontrolled diabetes mellitus. In OAS due to Aspergillosis urgent treatment is necessary to avoid complications such as complete vision loss and cavernous sinus thrombosis. CONCLUSION: OASs, represent a heterogenous group of disorders that results from a number of etiologies. OAS in a background of COVID-19 pandemic can be due to invasive Aspergillus infection as in our patient without any systemic illness and lead to miss diagnosis and delay in proper treatment.

Total external ophthalmoplegia and orbital apex syndrome as first presenting feature of Rhabdomyosarcoma involving petrous part of the temporal bone: A case report.

INTRODUCTION AND IMPORTANCE: Rhabdomyosarcoma (RMS) can have various unusual presentations. We report an unusual presentation of RMS as orbital apex syndrome in a six-year-old boy involving the petrous part of the temporal bone. CASE PRESENTATION: A six-year-old boy presented with drooping of the left eyelid for seven days associated with headache, left-sided hearing loss, and nasal blockage. Contrast-enhanced magnetic resonance imaging (CE-MRI) brain and orbit revealed an ill-defined expansile vascular lesion centered at the petrous part of the left temporal bone with extension to the cavernous sinus, which was confirmed as RMS on histopathology and immunohistochemistry. The patient was managed by chemotherapy and radiotherapy. CLINICAL DISCUSSION: RMS is the most common aggressive malignant soft tissue tumor in the pediatric population. It accounts for 4-8 % of all malignancies in children below 15 years of age, with strong male preponderance. The most common site for RMS is head and neck (45 %), having maximum incidence during the first decade of life. CONCLUSION: Total external ophthalmoplegia in a child is an acute emergency; it should be properly worked up, and neuroimaging should always be advised. Prompt diagnosis and management by a multidisciplinary team can be both life and sight-saving.

Orbital Apex Syndrome Secondary to Huge Primary Ethmoidal Sinus Mucocele: A Case Report.

Ethmoidal sinus mucoceles are benign expansile lesions that may progressively invade the orbit causing optic nerve compression and its nearby structures. We report a rare case of primary ethmoidal sinus mucocele instigating orbital apex syndrome. A 40-year-old man presented with right eye (RE) progressive blurring of vision with diplopia for 2 weeks. It was preceded by right-sided facial pain for 3 months. Clinical examination revealed RE proptosis with multiple cranial nerves palsy involving right cranial nerves II, III, IV, V, and VI, suggestive of right orbital apex syndrome. Magnetic resonance imaging (MRI) demonstrated right eye proptosis and right ethmoidal mucocele with intracranial and right intraorbital extension compressing the right medial rectus and optic nerve. The patient underwent an uncomplicated endoscopic sinus surgery resulting in a return to normal appearance and function post-operation. Thus, ethmoidal mucoceles are benign and curable with early recognition and intervention.

Frozen overnight: Acute orbital apex syndrome caused by aspergillosis.

PURPOSE: to describe a rare case of orbital apex syndrome caused by aspergillosis with acute presentation. CASE DESCRIPTION: retrospective case report of a 70-year-old man who developed unilateral ophthalmoplegia overnight. He was initially given the diagnosis of suspect Tolosa-Hunt syndrome, but biopsy of the involved tissue showed aspergillosis. CONCLUSION: orbital apex syndrome caused by fungal disease is a life-threatening condition that should be promptly diagnosed and treated. It may present acutely and should not be misdiagnosed as Tolosa-Hunt syndrome. To our knowledge this is the first such case report in the English ophthalmic language Literature.

Bilateral Orbital Apex Syndrome Related to Sphenoid Fungal Sinusitis.

Orbital apex syndrome (OAS) is a rare condition that usually occurs due to damage to surrounding inner and surrounding bone tissue. Orbital apex syndrome may result from a variety of conditions that cause damage to the superior orbital fissure and to the optic canal leading to optic nerve (II) dysfunction. We recently experienced a rare case of sphenoidal Aspergillosis, which damaged the adjacent cavernous sinus structures and led to the definite symptom of bilateral OAS in a 77-year-old male. We present this rare case with a brief review of these disease's entities.

A high-pressure situation.

An 8-year-old boy presented with acute visual loss in the right eye and nausea, vomiting, and diplopia. Imaging revealed a right orbital apex mass. Biopsy showed Langerhans cell histiocytosis (LCH), and the patient was diagnosed with isolated orbital LCH causing an orbital apex syndrome. A 12-month cytarabine chemotherapy course was begun, during which the patient developed bilateral optic disc edema. He was diagnosed with cytarabine-induced intracranial hypertension, which was successfully treated with acetazolamide. The cytarabine course was completed with complete resolution of the LCH lesion. The ophthalmologic relevance of this rare disorder is discussed.

Orbital Apex Syndrome Secondary to SMARCB1-Deficient Invasive Sinonasal Carcinoma.

Orbital apex syndrome (OAS) is a clinical entity defined by ophthalmoplegia and optic nerve dysfunction due to local disruption of the orbital apex. The causes of OAS are extensive and include infectious, inflammatory, traumatic, iatrogenic, and neoplastic conditions. Thus, appropriate management is dependent on an accurate and timely diagnosis of the underlying etiology. We present a case of a 58-year-old female who presented to the emergency department with ophthalmoplegia of subacute onset and diminished visual acuity in the setting of two weeks of headache, ocular pain, and facial swelling. She was ultimately diagnosed with OAS and admitted to the hospital for five days for further evaluation. She was found to have an incurable primary SMARCB1-deficient sinonasal carcinoma with an invasion of her orbital apex. A multidisciplinary management approach involving chemotherapy, radiation, and surgical intervention was performed, and the patient responded well. Nearly two years after her diagnosis, she continues to have stable residual carcinoma without evidence of recurrence or metastatic disease. Her visual acuity has returned to normal limits, and her oculomotor function has returned to near-normal levels.

Orbital Complications of Acute Invasive Fungal Rhinosinusitis: A New Challenge in the COVID-19 Convalescent Patients.

Purpose: Increased incidence of acute invasive fungal rhinosinusitis (AIFR) in the setting of COVID-19 is undeniable. This can be attributed to its effect on innate immunity and extensive use of corticosteroids. The goal of our study was to assess the orbital complications of AIFR and its management in the COVID-19 convalescent patients. Methods: Our longitudinal prospective study included 45 patients with orbital complications of AIFR in recently recovered COVID-19 patients. We performed otorhinolaryngological, ophthalmological, and neurological examinations to monitor the manifestations of the disease. Computed tomography and contrast enhanced magnetic resonance imaging were performed to detect the extent of infection. Antifungal medications, surgical intervention, and general condition management were all provided to all the patients. Results: We reported pre-septal cellulitis, orbital cellulitis, and orbital apex syndrome in 18, 13, and 10 patients, respectively. Four patients had cavernous sinus thrombosis. Mucormycosis and Aspergillus species were detected in 80% and 11.11% of our patients, respectively, while the mixed infection was found in 8.88% of our patients. Diabetes mellitus was the most common cause of immunocompromise (95.55% of our patients). Orbital pain and ophthalmoplegia were the most common ocular manifestations, followed by proptosis and relative afferent pupillary defect. All patients underwent surgical intervention, except for one patient who was unfit for surgery. One patient had orbital exenteration. The ophthalmological manifestations were reversible in cases of orbital and pre-septal cellulitis. The overall survival rate was 66.67%. Conclusion: Early diagnosis and treatment of AIFR can decrease the morbidity and mortality rate of affected patients.

Presentation and Management of a Complex Orbital Apex Syndrome From Penetrating Mechanical Spring Hook: A Case Report.

Our case report demonstrates the management of a unique penetrating orbital injury. The intraorbital foreign body was an approximately 22 cm long metal dishwasher spring hook lodged into the left orbital apex. An ophthalmological check-up a couple of weeks following the removal surgery discerned the patient had an unprecedented case of orbital apex syndrome. We present this unique case so physicians, medical students, and other emergency and medical professionals can learn about the diagnostic, surgical, and multidisciplinary management necessary to achieve a favorable clinical outcome.

Orbital apex syndrome secondary to myocysticercosis: A case report from Nepal.

Introduction: Cysticercosis is caused by parasitic infestation mainly by the larval form of Taenia solium. Orbital cysticercosis may involve both the intraocular structures and orbit particularly the extraocular muscles. The clinical manifestations are caused mainly by the mass effect of the cyst in the initial period resulting in ocular motility restriction and proptosis and depends primarily on the site of the lesion. Case presentation: Here we report a case of 27 years old male with orbital apex syndrome secondary to myocysticercosis. Discussion: Orbital myocysticercosis often mimics various eye pathologies like as isolated nerve palsy, orbital pseudotumor, orbital cellulitis. Acute vision loss in a case of orbital cysticerosis is mainly due to compressive optic neuropathy due to cystic lesion of extra ocular muscle or by direct invasion of the optic nerve. In our case, MRI orbit revealed cysticercosis of lateral rectus at orbital apex where it compressed the optic nerve resulting compressive optic neuropathy. Albendazole along with steroid was used as the first-line treatment. Conclusion: This case highlights that Orbital Apex Syndrome Secondary to Myocysticercosis is a rare and can lead to severe visual loss if not managed timely.

Radiation induced tissue necrosis mimicking orbital apex syndrome.

We are reporting a unique case with findings resembling orbital apex syndrome due to radiation-induced ischemic tissue necrosis following the treatment of meningiomatosis. In our patient, radiation injury caused multiple neuropathies including: 2nd, 3rd, 4th, 5th, and 6th neuropathies with oculosympathetic pathway involvement. To our knowledge, our patient has some unique features of complications related to radiation necrosis. Describing this case will help clinicians to have a better understanding of the extent of ocular manifestations secondary to radiation necrosis.

Orbital apex syndrome associated with intraorbital metastasis of lung cancer.

We present the case of a patient with lung adenocarcinoma stage IVB diagnosed as orbital apex syndrome (OAS) associated with intraorbital metastasis of lung cancer. When patients with lung cancer have diplopia, ptosis or ocular motility disorder, identifying OAS is important.

Orbital Apex Syndrome: A Case Series in a Tertiary Medical Center in Southern Taiwan.

Background: Orbital apex syndrome (OAS) is a rare ocular complication following by infection, inflammation, trauma, neoplasms, and vascularity. The epidemiological features of OAS remained limited, so this study aimed to present ophthalmic clinical features, determine the causes to evaluate the visual prognosis of orbital apex syndrome (OAS) patients in Taiwan. Methods: This was a retrospective study by reviewing the electronic medical records from National Cheng Kung University Hospital in Taiwan during 2017-2019. We included patients diagnosed with OAS to review their ocular symptoms and signs, visual acuity, ocular images, etiologies, treatment and visual prognosis. Results: Twenty cases (mean age: 65.55 ± 13.06; male: 75%) with the diagnosis of OAS were included in this study. All patients presented as unilateral involvement, but the initial ocular presentations and etiologies varied. For example, blurred vision was reported in 80% of these patients, and tumor-related compression (55%) and infection (15%) were the most frequent causes for the OAS. After the follow-up, we found 35% of patients' visions declined or worsened to the blindness, 15% of patients' visions remained stable, 20% of patients' visions had mild improvement, and 35% of patients' visions were not measured because of debilitating clinical condition. We identified three OAS patients with mortality (15%), and all of them were attributed to the underlying malignancies. Conclusion: The clinical magnifications and etiologies of OAS are heterogeneous in Taiwan. Our findings indicated the tumor-related compression is the most frequent causes of OAS in Taiwan, and it is also related to poor clinical outcomes.

Multiple Cranial Neuropathies Similar to Orbital Apex Syndrome Associated with Pembrolizumab: A Case Report.

Neurotoxicity is one of the more serious immune-related adverse events (irAEs) linked to immune checkpoint inhibitors and calls for prompt diagnosis and treatment. We describe a case of posttreatment anti-programmed death-1 immune checkpoint inhibitor pembrolizumab-induced oculomotor, optic, and trigeminal neuropathy in an 84-year-old female patient with recurrent pulmonary adenocarcinoma. After she received 13 cycles of pembrolizumab, she experienced hyponatremia, anorexia, and right ptosis. There were signs of the suspected irAEs of pembrolizumab, including trigeminal neuropathy, optic neuropathy, and oculomotor neuropathy. Steroid pulse therapy had good results for her neurological findings. We reported this case despite reports of pembrolizumab-induced mononeuropathy of the oculomotor and optic nerves because multiple cranial neuropathies like orbital apex syndrome are thought to be uncommon.