A 43 year-old man presented with pain on the right tooth for three days duration. Computed tomography showed left orbital cellulitis and right parapharyngeal abscess. There was also evidence suggestive of a dental abscess over right upper alveolar region. Magnetic resonance imaging revealed left superior ophthalmic vein thrombosis. Emergency drainage of the right parapharyngeal abscess was performed. Right maxillary molar extraction revealed periapical abscess. Left eye proptosis markedly reduced after initiating heparin.
The coronavirus disease (COVID-19) has once again reminded us of the significance of host immune response and consequential havocs of the immune dysregulation. The severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) inflicts severe complications to the infected host, including cough, dyspnoea, fever, septic shock, acute respiratory distress syndrome (ARDs), and multiple organ failure. These manifestations are the consequence of the dysregulated immune system, which gives rise to excessive and unattended production of pro-inflammatory mediators. Elevated circulatory cytokine and chemokine levels are accompanied by spontaneous haemorrhage, thrombocytopenia and systemic inflammation, which are the cardinal features of life-threatening cytokine storm syndrome in advanced COVID-19 diseases. Coronavirus hijacked NF-kappa B (NF-κB) is responsible for upregulating the expressions of inflammatory cytokine, chemokine, alarmins and inducible enzymes, which paves the pathway for cytokine storm. Given the scenario, the systemic approach of simultaneous inhibition of NF-κB offers an attractive therapeutic intervention. Targeted therapies with proteasome inhibitor (VL-01, bortezomib, carfilzomib and ixazomib), bruton tyrosine kinase inhibitor (acalabrutinib), nucleotide analogue (remdesivir), TNF-α monoclonal antibodies (infliximab and adalimumab), N-acetylcysteine and corticosteroids (dexamethasone), focusing the NF-κB inhibition have demonstrated effectiveness in terms of the significant decrease in morbidity and mortality in severe COVID-19 patients. Hence, this review highlights the activation, signal transduction and cross-talk of NF-κB with regard to cytokine storm in COVID-19. Moreover, the development of therapeutic strategies based on NF-κB inhibition are also discussed herein.
Arteriovenous malformations are congenital lesions that may evolve with time and manifest in a plethora of presentations. They can occur as torrential epistaxis when it extensively involves the facial region. Multi-imaging modalities are available to assist in characterizing the structure of the lesion as well as its location and extent. This complex disease requires a multidisciplinary team approach with preoperative embolization and surgery. We present a rare cause of life-threatening epistaxis in a gentleman with a longstanding orbital and hemifacial arteriovenous malformation and discuss the complexities involved in its management.
The prevalence of thyroid-associated ophthalmopathy (TAO) has been reported to be lower in several Asian populations than in Caucasians. The risk factors for TAO that have been demonstrated in Caucasians have not been studied in Asian populations. The aim of this study, therefore, was to determine the prevalence, risk factors, and clinical features of TAO in a cohort of multiethnic Malaysian patients with Graves' disease (GD).
Optic glioma (OPG) accounts for 4-8% of all brain tumors in children. En-block removal of intraorbital tumor is recommended in cases with disfiguring exophthalmos and impaired vision. Surgical resection of intraorbital optic nerve (ON) poses the risks of permanent ptosis and globe atrophy. We present here the case of a 4-year-old boy with exophthalmos and near blindness due to an intraorbital OPG. Despite chemotherapy he showed progressive exophthalmos and vision loss. Bony orbital decompression with ON transection temporally reduced his exophthalmos. OPG resection was required later for recurrence of his exophthalmos secondary to tumor progression. Post operatively, he had preserved oculomotor nerve functions but developed globe ischemia. Unusually, his ischemic globe caused him to have pain and severe photophobia, which later lead to enucleation. Photophobia has been reported in blind patients. Animal models and MRI functional imaging showed activation of trigeminal pathway during photophobia in completely transected ON. However, the exact neuro-ophthalmology pathway requires further study.
Objective To describe the patient demographics, clinical findings, investigations, surgical outcomes, and histopathological findings of seven cases of orbital solitary fibrous tumours. Method This was a retrospective review of seven cases of orbital solitary fibrous tumour, which were followed up in Hospital Serdang, a national oculoplastic centre, from years 2008-2017. Results This study included seven patients with ages between 21 and 35 years old; two were males and five were females. All seven patients presented with painless chronic unilateral proptosis. Radiological imaging of the orbit showed a localized contrast enhancing intraorbital mass. All patients underwent orbitotomy and excisional biopsy. Intraoperative findings showed a well-encapsulated and vascularized mass. Histological findings of spindle-shaped cells were noted. All cases had positive staining for cluster of differentiation (CD) 34, five were positive for CD 99, four were positive for B-cell lymphoma (BCL-2), and five patients had positive staining for S-100. Three of the patients did not have clear margins during the primary operation and subsequently had a recurrence within two years. Conclusion A solitary fibrous tumour is a rare mesenchymal tumour with a pleural origin. The orbit is the most common extrapleural site of the tumour and they are usually benign. Immunohistochemistry is important to differentiate it from other, more aggressive forms of orbital tumours. Regular follow-up is important to monitor for recurrence.
A 42-year-old Malay gentleman presented with progressive painless blurring of vision on his left eye associated with ocular ache, redness and increasing proptosis. Examination revealed presence of relative afferent papillary defect with visual acuity of counting finger, and 6/6 on the right eye. Extraocular muscle movement of the left eye was restricted to ten percent in all directions. Computed tomography (CT) and magnetic resonance imaging (MRI) showed enhancing mass occupying the left orbital apex. Diagnosis of optic nerve sheath meningioma was made, and patient subsequently went for radiotherapy. His symptoms subsided completely, until he presented with similar visual complains, and fullness of the upper lid two years later. A repeated MRI showed enlargement of superior and lateral recti muscles with extension to lacrimal gland region. Biopsy of the lacrimal gland revealed inflammatory cells consistent of inflammatory pseudotumor. High dose systemic steroid was instituted, followed by slow tapering of oral steroid. His symptoms completely resolved, and latest visual acuity was 6/9, with no recurrence, to date.
Ocular inflammation from various causes may have similar clinical presentation thus careful clinical evaluations are mandatory particularly when the disease appear to be resistant to treatment. This paper reports a case of ocular lymphoma which was initially treated as Vogt Koyanagi Harada disease at a different centre. A 65-year-old Sudanese man complained of gradual worsening left eye vision. Careful ocular and slit lamp examination revealed a conjunctival lesion with choroidal infiltration as well as exudative retinal detachment. Computed tomography scan (CT scan) showed left eye axial proptosis, and a homogenous enhancing mass at the posterior coat of the globe. Tissue biopsy of the conjunctival lesion revealed marginal Zone B cell (MALT type), low grade, non-Hodgkin’s lymphoma. AS the ocular signs and symptoms progressed, chemotherapy was initiated. The proptosis, exudative retinal detachment, disc hyperemia and swelling improved after chemotherapy. The post treatment CT scan showed reduction of the posterior ocular coat mass. The possibility of malignant lymphoma should be considered in patients with resistant uveitis despite administration of corticosteroid. Ocular lymphoma is an indolent tumour with good prognosis. Careful ocular examination, adequate imaging studies followed by early surgical biopsy will contribute to early diagnosis.
Aim. To report a rare case of arteriovenous malformation in temporal lobe presenting as contralateral orbital symptoms mimicking carotid-cavernous fistula. Method. Interventional case report. Results. A 31-year-old Malay gentleman presented with 2-month history of painful progressive exophthalmos of his left eye associated with recurrent headache, diplopia, and reduced vision. Ocular examination revealed congestive nonpulsating 7 mm exophthalmos of the left eye with no restriction of movements in all direction. There was diplopia in left lateral gaze. Left IOP was elevated at 29 mmHg. Left eye retinal vessels were slightly dilated and tortuous. CT scan was performed and showed right temporal arteriovenous malformation with a nidus of 3.8 cm × 2.5 cm with right middle cerebral artery as feeding artery. There was dilated left superior ophthalmic vein of 0.9 mm in diameter with enlarged left cavernous sinus. MRA and carotid angiogram confirmed right temporal arteriovenous malformation with no carotid-cavernous fistula. Most of the intracranial drainage was via left cavernous sinus. His signs and symptoms dramatically improved following successful embolisation, completely resolved after one year. Conclusion. Intracranial arteriovenous malformation is rarely presented with primary ocular presentation. Early intervention would salvage the eyes and prevent patients from more disaster morbidity or fatality commonly due to intracranial haemorrhage.
Orbital cellulitis is a relatively common disease affecting predominantly the paediatric population. Most cases occur as a result of spread from the nearby sinuses. Other causes include penetrating trauma or extension from infected adjacent structures.If left untreated, this condition may result in devastating sequelae such as orbital apex syndrome, cavernous sinus thrombosis, meningitis, cranial nerve palsies, intracranial abscess formation and even death. A 47 year old immunocompetent Burmese lady presented with left eyelid swelling of 2 days duration associated with eye redness, blurring of vision and diplopia. Previously, there was history of right maxillary sinusitis and parapharyngeal abscess 9 months prior to presentation. On examination, she was afebrile with vision of 1/60 for the left eye with positiverelative afferent pupillary defect (RAPD). The eye was proptosed and swollen with restricted extraocular movements in all gazes. Conjunctiva was injected with chemosis and there was corneal epithelial bedewing. Otherwise anterior chamber was quiet and intraocular pressure was 51mmHg. Bilateral fundus examination was normal. Computed tomography (CT) scan of the orbit and paranasal sinus showed dense sinusitis and periosteal abscess at the lateral orbital wall.She was started on intravenous (IV) Cefuroxime and Metronidazole and underwent Functional Endoscopic Sinus Surgery (FESS) and orbital decompression. Intra-operatively there was pus and debris at the left anterior ethmoid, maxillary and sphenoid air sinuses and cultures revealed Klebsiella pneumoniae which was sensitive to Cefuroxime. Despite medical and surgical treatment, left orbital swelling only reduced minimally. However after starting intravenous Dexamethasone the swelling dramatically improved. She completed 10 days of intravenous Dexamethasone. Upon discharge, she was given oral Dexamethasone 2mg daily for 2 weeks and completed 2 weeks of oral Cefuroxime and Metronidazole. Intraocular pressure normalised and vision recovered to 6/9. A repeat CT orbit 3 weeks later showed resolving preseptal and periorbital collection.