Orbital Apex Syndrome – A Case Report

Orbital apex syndrome is characterized by vision loss and ophthalmoplegia due to the involvement of the orbital apex. The signs and symptoms vary depending upon the involvement of the structures within the orbital apex, the superior orbital fissure or the cavernous sinus. Clinical evaluation is the key to the diagnosis which is aided by neuro-imaging modalities including brain and orbital Magnetic Resonance Imaging and Computed Tomography scans. In rare instances, a biopsy may be needed to aid in diagnosis. Treatment depends on what the nature of the lesion.

More than meets the eye: Varicella zoster virus-related orbital apex syndrome

A 67-year-old male patient with poorly controlled type-2 diabetes mellitus developed an orbital apex syndrome and anterior cavernous syndrome secondary to herpetic zoster ophthalmicus (HZO), despite being on oral acyclovir. Urgent treatment with intravenous acyclovir led to improvement of the orbital and ocular inflammation but had no effect on the complete ophthalmoplegia and profound visual loss. At the 9-month follow-up visit, the patient had complete unilateral ophthalmoplegia and monocular blindness due to optic atrophy.

Two Cases of Orbital Apex Syndrome after Blunt Orbital Trauma

PURPOSE: To report two cases of orbital apex syndrome caused by blunt orbital trauma without structural damage of the orbit. CASE SUMMARY: (Case 1) A 50-year-old male came to our clinic complaining of visual loss after blunt orbital trauma by a metal bar. The best-corrected visual acuity was no light perception and light reflex was not observed in the affected eye. He also presented with complete ptosis and ophthalmoplegia with relative sparing of adduction and depression. High signal intensity of the orbital soft tissue including the optic nerve sheath was revealed using a T2-weighted image in magnetic resonance imaging. After starting steroid pulse therapy, his visual acuity improved to counting fingers on the third day. Ocular movement and levator function recovered to the normal range while visual acuity remained counting fingers. (Case 2) A 64-year-old female presented with complete ptosis after trauma to her right eyeball. The best-corrected visual acuity was 20/25 in the right eye. Complete ptosis and ophthalmoplegia with relative sparing of abduction and depression in the right eye were observed at the initial presentation. Magnetic resonance images showed enhancement of the right periphery optic nerve and distal rectus muscle. Two months after undergoing steroid pulse therapy, levator function and ocular movement recovered completely, and visual acuity improved to 20/20. CONCLUSIONS: The orbital apex syndrome caused by blunt orbital trauma showed good response to steroid pulse therapy. Steroid treatments may therefore be considered for the treatment of traumatic orbital apex syndrome.

A Case of Orbital Apex Syndrome with Central Retinal Artery and Vein Occlusion Following Trauma

PURPOSE: To report a case of orbital apex syndrome (OAS) combined with central retinal artery occlusion (CRAO) and central retinal vein occlusion (CRVO) following blunt trauma. CASE SUMMARY: A 4-year-old female visited the hospital following a traffic accident. She was admitted because of multiple fractures of the skull and pneumocephalus. On day 5, she was referred to us with decreased visual acuity in her right eye. Her initial visual acuity was hand motions in the right eye and 0.8 in the left eye. The right eye showed a dilated pupil, ptosis, and total ophthalmoplegia, and the left eye showed limited abduction. A fundus examination revealed multiple retinal hemorrhages, tortuous veins, and an edematous white retina with a cherry-red spot in the right eye. Brain magnetic resonance imaging revealed an entrapped right optic nerve because of bony fragments in the orbital apex. The patient was diagnosed with OAS accompanied by CRAO and CRVO in the right eye, and with traumatic abducens nerve palsy in the left eye. After 6 months, the visual acuity was hand motions, and the fundus examination showed absorbed retinal hemorrhages, pale discs, and general retinal thinning of the right eye. Ptosis of the right eye and extraocular muscle movement of both eyes were improved. CONCLUSIONS: Combined CRAO and CRVO following trauma is very rare and is even more rarely associated with OAS. It is important for clinicians to be aware of the potential for central retinal vessel occlusions and OAS in cases of blunt ocular trauma.

Clinical Analysis of 18 cases with orbital apex syndrome / 中国神经精神疾病杂志

Objective To explore the clinical characteristics, etiology and prognosis of orbit apex syndrome. Methods Clinical data of 18 patients with orbit syndrome was summarized and analyzed. Results All patients developed vision loss, ptosis and diplopia, such as a headache or sore eyes forehead performance. However, the onset symptoms varied including vision decline or loss in 8 cases, headache in 7 cases, eye pain in 1 case, toothache in 1 case, stuffy nose and runny nose in 1 case and exophthalmos in 1 cases. The Causes included trauma in 6 cases (33％), mass lesion in 5 cases (28％), nonspecific inflammation in 1 cases (22％), infection in 3 cases (17％). Vision and eye movements disorder were improved in 5 cases of 6 patients with trauma in which 3 mild cases recovered better and one severe case did not recovered. Of 5 patients with mass lesion, the vision and eye movement were improved in one patients with sphenoid sinus mucocele receiving surgical operation. but not in the rest 4 cases with orbital adenoid cystic carcinoma or osteosarcoma patients receiving either surgical operation or radiotherapy. Of 4 patients with nonspecific inflammatory disease, eye movements were improved in 2 mild cases but not in 2 severe cases. Of 3 infectious diseases, eye movements were improved in 1 mild case. Conclusion Orbital apex syndrome is a heterogeneous disorder with multiple etiologies, presenting with a variety of initial symptoms. The prognosis depends on causes, timely diagnosis, early diagnosis and early treatment.

Orbital Apex Syndrome after Uneventful Phacoemulsification

PURPOSE: We report a case of orbital apex syndrome associated with ocular ischemic syndrome after unenventful cataract surgery. CASE SUMMARY: A 74-year-old female came to our clinic with vision loss, ptosis, total ophthalmoplegia and ocular pain in the left eye after cataract surgery. On radiologic examination, diffuse hypertrophy of the extraocular muscles and a crowded orbital apex were observed. Additionally, the arm to retina and choroidal filling times were delayed on fluorescence angiography. Based on these findings, the patient was diagnosed with ocular ischemia complicated by orbital apex syndrome. After prompt pulse steroid therapy, improvement in extraocular muscle and pupil movement, ptosis and proptosis were observed; however, visual acuity failed to return to baseline. CONCLUSIONS: We report a rare case of orbital apex syndrome associated with ocular ischemic syndrome after cataract surgery, indicating the orbital apex syndrome may occur in healthy patients after uneventful cataract surgery.

Successful delayed treatment of the traumatic orbital apex syndrome by nasal endoscopic decompression surgery.

To report a patient with traumatic orbital apex syndrome, who fully recovered visual and extraocular function following surgery. A 34‑year‑old male presented with visual and extraocular function disorders in his right eye following traffic accident, who was referred to our hospital 5 weeks after accident. The patient underwent endoscopic optic nerve and orbital apex decompression with topical and systemic application of nerve growth factor and steroids after a failed trial of mega‑dose intravenous corticosteroids. Visual acuity improved to 20/20 at 3 weeks after surgery, and the right eye globe moved in most directions at 1 year, which remained stable at 3 years. Surgical decompression should be considered even when symptoms have been present for over a month.

A Case of Fungal Infection in the Onodi Cell with Diplopia / 대한이비인후과학회지

The orbital apex syndrome is a very rare complication of fungal sinusitis, as well as of other conditions, and is characterized by blindness, diplopia, proptosis of the eye and ophthalmoplegia. We present a case of diplopia caused by a fungal ball in the Onodi cell. A 62-year-old woman, diagnosed with orbital apex syndrome and suffering from ophthalmalgia and diplopia in the right eye, was presented to our outpatient clinic. Computed tomography showed a soft tissue lesion occupying the right Onodi cell with chronic inflammation. Patients who have an Onodi cell carry a high risk for optic nerve injury, so endoscopic sinus surgery using navigation was performed. Histopathology examination confirmed the diagnosis of a fungal ball. We experienced a rare case of a fungal ball in the Onodi cell, which caused damage to the adjacent cavernous sinus structure and led to diplopia and orbital pain. We present this case with a brief review of these disease entities.

Orbital Apex Syndrome Related to Isolated Sphenoid Fungal Sinusitis

Orbital apex syndrome (OAS) has been described as malfunction of cranial nerve II in the optic canal and cranial nerves III, IV, V1, and VI in the superior orbital fissure due to vascular compromise, compression, or infiltration. Symptoms include ophthalmoplegia, ptosis, decreased corneal sensation, and vision loss. There are many potential causes of OAS including inflammation, infection, iatrogenic, neoplasm, vascular disease, or trauma. Among these, however, fungal sinusitis is one of the more rarely reported etiologies. Infections of the sphenoid sinuses typically occur in conjunction with other paranasal sinuses, while isolated sphenoid sinusitis is uncommon. In this case report, we describe OAS due to isolated sphenoid fungal sinusitis in a 67-year old man who presented with periorbital pain, acute vision loss, and ophthalmoplegia. He was treated surgically and was given postoperative antifungal agents as well as steroids for three months. We present this rare case along with a literature review of OAS.

Orbital Apex Syndrome Related to Isolated Sphenoid Fungal Sinusitis

Orbital apex syndrome (OAS) has been described as malfunction of cranial nerve II in the optic canal and cranial nerves III, IV, V1, and VI in the superior orbital fissure due to vascular compromise, compression, or infiltration. Symptoms include ophthalmoplegia, ptosis, decreased corneal sensation, and vision loss. There are many potential causes of OAS including inflammation, infection, iatrogenic, neoplasm, vascular disease, or trauma. Among these, however, fungal sinusitis is one of the more rarely reported etiologies. Infections of the sphenoid sinuses typically occur in conjunction with other paranasal sinuses, while isolated sphenoid sinusitis is uncommon. In this case report, we describe OAS due to isolated sphenoid fungal sinusitis in a 67-year old man who presented with periorbital pain, acute vision loss, and ophthalmoplegia. He was treated surgically and was given postoperative antifungal agents as well as steroids for three months. We present this rare case along with a literature review of OAS.

A Case of Orbital Apex Syndrome Induced by Penetrating Orbital Injury with Long-Term Results

PURPOSE: To report a case of orbital apex syndrome induced by penetrating orbital injury by a wire with the recovery process and clinical outcomes. CASE SUMMARY: A 40-year-old female visited our clinic after a penetrating orbital injury through the left inferomedial conjunctiva by a wire. The best corrected visual acuity of the left eye was 0.6, and ptosis and total ophthalmoplegia were observed. The patient showed a dilated pupil, swelling of the optic disc on fundus exam, and an inferior field defect on the automated perimetry. The computed tomography image revealed mild retrobulbar hemorrhage, but there was no orbital bony fracture. Enhancement of the optic nerve sheath was observed on the magnetic resonance image. The patient was admitted and received systemic antibiotics and steroid treatment. After 1 month, visual acuity, ptosis, and limitation in adduction were partly improved. After 3 months, depression and adduction were improved and the pupil size was normalized. However, further improvement was not observed after the one-year follow-up. CONCLUSIONS: The recovery from orbital apex syndrome was achieved until 3 months after injury. The final outcomes may depend on the mechanism and pathophysiology of the injury. Emergent diagnosis and proper management are essential to achieve optimal clinical results.

Superior Orbital Fissure Syndrome after Repair of Blow Out Fracture

PURPOSE: Superior orbital fissure syndrome is a rare neurological complex. Superior orbital fissure syndrome may result from a variety of inflammatory, infectious, neoplastic, iatrogenic, traumatic, vascular cause. The author report a patient who suffered from superior orbital fissure syndrome after inferior orbital wall reduction. METHODS: A 26-year-old female suffered from inferior orbital wall fracture with inferior gaze limitation and orbital soft tissue herniation. On posttrauma 10 day, inferior orbital wall was reduced using endoscope and porous polyethylene(Medpor(R)) was inserted. On immediate postoperation, she reported that extraocular movement was limited in almost any directions. She underwent exploration surgery to release the presence of extraocular muscle impingement. But, there was no observation of extraocular muscle impingement. On postoperative one day, high- dose steroid therapy was started to release superior orbital fissure syndrome which was defined in postoperative computed tomography. RESULTS: After one month of high-dose steroid therapy, extraocular movement limitations improved progressively in all directions. In four months, extraocular movement recovered completely. CONCLUSION: Superior orbital fissure syndrome may occur after surgical procedure of orbital wall reduction. Prompt diagnosis and treatment with mega-dose corticosteroid is an effective option for avoiding disaster from compressive syndrome.

A rare case of orbital apex syndrome with herpes zoster ophthalmicus in a human immunodeficiency virus-positive patient.

We report a rare instance of favorable outcome in orbital apex syndrome secondary to herpes zoster ophthalmicus (HZO) in a human immunodeficiency virus (HIV)-positive patient. The patient complained of pain and decrease in vision in one eye (20/640) for 2 weeks accompanied with swelling, inability to open eye, and rashes around the periocular area and forehead. The presence of complete ophthalmoplegia, ptosis, relative afferent pupillary defect, and anterior uveitis with decreased corneal sensation prompted a diagnosis of HZO with orbital apex syndrome. The enzyme-linked immunosorbent assay test and a low CD4 count confirmed HIV. Highly active antiretroviral therapy (HAART), systemic acyclovir, and systemic steroids were started. Visual acuity and uveitis improved within 10 days. By the end of the fourth week, ocular motility also recovered and the final visual acuity was 20/25. We highlight the role of HAART, used in conjunction with systemic steroid and acyclovir therapy, in improving the outcome.

A Case of Orbital Apex Syndrome Related to Sphenoid Fungal Sinusitis / 대한이비인후과학회지

Orbital apex syndrome (OAS) has been described previously as a syndrome involving damage to the oculomotor nerve, trochlear nerve, abducens nerve, and ophthalmic branch of the trigeminal nerve in association with optic nerve dysfunction. The conditions and symptoms of OAS are characterized by blindness, fixed dilated pupils, proptosis, ptosis of the eye and ophthalmoplegia. Infectious diseases involving the central nervous system, paranasal sinuses, and periorbital structures may lead to an OAS. We recently experienced a rare case of sphenoidal aspergillosis, which damaged the adjacent cavernous sinus structures and led to the definite symptom of OAS in a 75 year-old female. We present this rare case with a brief review of these disease's entities.

Clinical analysis of orbital apex syndrome caused by sinus diseases / 临床耳鼻咽喉头颈外科杂志

Objective:To investigate the clinical characters and therapeutic methods of orbital apex syndrome caused by sinus diseases.Method:Six cases of orbital apex syndrome originated from sinus diseases were retrospective analyzed in our hospital from August 2003 to February 2009. Different therapeutic methods were taken according to different causes of disease.Result:Four cases of sinus infection,one cases of sphenoethmoid mucocele , one cases of sinus squamous cell carcinoma. The results of patients with orbital apex syndrome included cure and effectiveness in 2 cases respectively, death from myocardial infarction in one case, and one case is still following up.Conclusion:Orbital apex syndrome originated from sinus diseases was rare, which was mainly caused by sinus infection . The key point is the correct etiology diagnosis in early phase . Treatment with endoscopic sinus surgery early is a good effective method for orbital apex syndrome caused by sinus diseases, in the same time with enough dosage of antibiotic and glucocorticoid therapy intravenously.

Orbital Apex Syndrome with Nasal Type Natural Killer(NK)/T-cell Lymphoma of Sphenoid and Ethmoid Sinus

PURPOSE: To report a case of nasal-type NK/T cell lymphoma occurring in the sphenoid and ethmoid sinuses of an orbital apex syndrome patient. CASE SUMMARY: A 61-year-old male patient visited our hospital for the impairment of vision in the right eye for the previous month, and for right-side blepharoptosis, pain around the eyeball, and limitation of extraocular movement in the right eye for three days earlier. In MRI (magnetic resonance image) and CT (computed tomography), shades with vague boundaries were observed in the right sphenoid and ethmoid sinuses, and open biopsy and decompression were performed for the lesions. After the surgery, the vision of the right eye increased, and improvement was observed in the right blepharoptosis, the pain around the eyeball, and in the limitation of motility of the extraocular muscle in the right eye. The patient was diagnosed with nasal type NK/T cell lymphoma in biopsy, and radiotherapy and chemotherapy were performed.

Síndrome do ápice orbitário causada por herpes zóster oftálmico: relato de caso e revisão da literatura / Herpes zoster ophthalmicus and orbital apex syndrome: case report and literature review

OHerpes Zoster Oftálmico (HZO) decorre da infecção pelo vírus da varicela-zoster que permanece latente no gânglio de Gasser até que seja reativado e comprometa a divisão oftálmica do nervo trigêmeo. HZO freqüentemente causa manifestações oftalmológicas como lesões vesiculares palpebrais, ceratoconjuntivite, esclerite, uveíte, paralisia oculomotora, miosite orbitária e neurite óptica. Raramente o acometimento do ápice da órbita pode ser a manifestação inicial desta grave afecção. Este trabalho relata um caso de síndrome do ápice orbitário associado à meningite, causado por HZO e que foi tratado com corticosteróide e aciclovir sistêmicos.

Herpes Zoster ophthalmicus (HZO) is caused by a varicella-zoster virus infection which remains latent in the ganglion of Gasser until it is reactivated and compromise the ophthalmic division of the trigeminal nerve. HZO commonly causes neuro-ophthalmic complications such as vesicular lesions in the eyelids, keratoconjunctivitis, sclertis, uveitis, ocular palsy, orbital miositis and optic neuritis. HZO rarely presents as an orbital apex syndrome. This paper describes a patient with of orbital apex syndrome associate and meningitis caused by HZO which was treated with systemic steroids and acyclovir.

Absceso subperióstico y síndrome del vértice orbitario: complicación posterior a la extracción del tercer molar mandibular / Subperiostial orbital abscess and apex syndrome as a complication after a mandibular molar extraction

Introducción: La diseminación de las infecciones que se extienden de dientes mandibulares y estructuras adyacentes hacia la órbita y tejido periorbitario son infrecuentes, pero graves ya que tienen el potencial de causar la pérdida de la visión. Objetivos: El propósito de este artículo es publicar un caso clínico muy interesante por lo infrecuente, y explicar a juicio de los autores las vías de diseminación de la infección, a su vez alertar a los diferentes especialistas involucrados sobre las severas consecuencias que pueden resultar tras la extracción de piezas dentales, como es la pérdida definitiva de la visión. Reporte del caso: Se trata de un paciente masculino de 43 años, sin antecedentes mórbidos relevantes, que fue enviado al Servicio Médico Legal para examen clínico forense con pérdida total de la visión del ojo derecho por atrofia óptica como complicación de un síndrome del vértice orbitario, luego de la exodoncia del tercer molar mandibular derecho. Conclusiones: Es fundamental el tratamiento oportuno y agresivo para detener la diseminación de una infección desde las estructuras adyacentes hacia la órbita. La comunicación efectiva interdisciplinaria entre los profesionales involucrados evita complicaciones de tipo médico-legales y secuelas definitivas al paciente.

Introduction: Infection dissemination from mandibular teeth to the orbit and adjacent structures is infrequent, but nevertheless serious, as they may cause severe problems, including vision loss. Aim: The purpose of this study is to present a rare and interesting case of such a dissemination, to discuss the possible routes of infection propagation, and to alert the different specialists involved on the possible complications after dental extractions, such as visual loss. Report of the case: The case of a 43-year-old previously healthy male is presented. The patient was sent to the Medical Legal Service for clinical forensic examination after total vision loss of the right eye, caused by optic atrophy as a complication of orbital apex syndrome, after the extraction of the third right mandibular molar. Conclusions: Opportune and aggressive treatment is crucial to stop infection spread from adjacent structures to the orbit. Effective communication among the professionals involved avoids medical - legal complications and precludes permanent sequels to the patient.

A Case of Nasal Endoscopic Treatment for Paranasal Mucocele

PURPOSE: We report a case of sphenoid and ethmoid mucocele causing orbital apex syndrome, which was successfully treated with drainage under nasal endoscopy. METHODS: A 82-year-old female visited the hospital complaining of decreased visual acuity in her left eye for 15days. Visual acuity of the left eye was hand motion . She also complained of left ocular pain and headache. In ophthalmologic examination, exophthalmos, mild ptosis, extraocular muscle movement limitation and RAPD (relative afferent pupillary defect) of the left eye were found. Orbital CT showed a 3.3 X 2.9 X 4.1 cm sized well-demarcated cystic lesion involving the left sphenoid sinus, left ethmoid sinus, and posterior portion of the right ethmoid sinus. The ethmoid air cell was remodeled by this cystic mass. The medial wall of the left orbit was protruded outward. So, the medial rectus muscle and optic nerve in the left orbit were compressed. We found a bulging yellowish cystic mass supero-posterior to the middle turbinate under nasal endoscopy. We performed incision and drainage under nasal endoscopy. RESULTS: At post-operative 2days, orbit CT showed the removed large mucocele in the sphenoid and ethmoid sinuses. At post-operative 2weeks, corrected visual acuity of the left eye was 0.6. There was no exophthalmos, ptosis, or extraocular muscle movement limitation. CONCLUSIONS: Endoscopic surgery in the treatment of paranasal mucocele can be performed during a short time under local anesthesia and anatomical change of the paranasal sinus can be minimized. We therefore suggest that drainage through nasal endoscopy is effective method in the treatment of paranasal sinus mucocele.

Two Cases of Orbital Apex Syndrome

PURPOSE: To report two cases of orbital apex syndrome: one induced by penetrating orbital injury and the other by severe optic nerve swelling associated with bacterial meningitis. METHODS: We have experienced a 40-year-old male after penetrating orbital injury who complained of loss of vision, ocular pain, ptosis, hypesthesia of forehead, and total ophthalmoplegia. The CT image of this patient showed a severe retrobulbar hemorrhage and marked enlargements of extraocular muscles. In a 62-year-old male who had a same symptom after headache and decreased mentality, a bacterial meningitis was diagnosed from CSF study and we saw a very severe swelling of the unilateral entire optic nerve and optic nerve sheath in CT. We managed these two patients with a large amount of systemic RESULTS: In 40-year-old man, there were improvements of ptosis and visual acuity from negative light perception to positive, but ophthalmoplegia was not improved. In 62-year-old man, ptosis and ophthalmo-plegia were improved after early antibiotics administration and papilledema disappeared two months after development, but visual acuity was not improved from negative light perception.