Vipul Arora

Clinical profile of cerebral venous sinus thrombosis and the role of imaging in its diagnosis in patients with presumed idiopathic intracranial hypertension

Retrospective descriptive study reporting the rate of occurrence of cerebral venous sinus thrombosis (CVST), highlighting the role of magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) in patients with presumed idiopathic intracranial hypertension (IIH). Study was conducted in the department of neuro-ophthalmology at a tertiary eye care center in South India. Data from 331 patients diagnosed with IIH from June 2005 to September 2007 was included. Inclusion criteria were: Elevated opening cerebrospinal fluid (CSF) pressure of more than 200 mm of water on lumbar puncture, normal CSF biochemistry and microbiology, and normal neuroimaging as depicted by computed tomography(CT) scan. Exclusion criteria were: Space-occupying lesions, hydrocephalus, meningitis, intracranial pressure within normal range, abnormal CSF biochemistry and microbiology. The remaining patients were evaluated with MRI and MRV. CVST was present in 11.4% of patients who were presumed to have IIH (35/308). MRI alone identified 24 cases (68%) of CVST, while MRI used in combination with MRV revealed an additional 11 cases (32%). Risk factors associated with CVST were identified in nine out of 35 patients (26%). CVST may be misdiagnosed as IIH if prompt neuroimaging by MRI and MRV is not undertaken. Risk factors of CVST may not be apparent in all the cases and these patients are liable to be missed if CT scan alone is used for neuroimaging, hence MRI, combined with MRV should be undertaken to rule out CVST.

Multimodality treatment approach in management of primary peripheral primitive neuroectodermal tumor of the orbit.

Primitive neuroectodermal tumor is a small round cell malignancy which rarely involves the orbit. We report a case of a two-year old male child presenting as unilateral eccentric proptosis with extraconal and intraconal mass, diagnosed as primary peripheral primitive neuroectodermal tumor (pPPNET) on histopathology and immunohistochemistry. There is no defined consensus in the management of these tumors due to its rare presentation. We describe its distinguishing features with emphasis on multimodal and aggressive treatment approach which ensures appropriate management of these cases.

Primary adenoid cystic carcinoma of the eyelid.

Adenoid cystic carcinoma is a rare epithelial malignancy, which tends to grow slowly. It is an intractable neoplasm due to its ability to invade perineural spaces. A 59-year-old female presented with a gradually increasing mass in the right lower eyelid. An excisional biopsy with wide margins revealed a diagnosis of primary adenoid cystic carcinoma of eyelid skin with perineural invasion. Although a rare neoplasm, primary adenoid cystic carcinoma of eyelid skin should be included in the differential diagnosis of eyelid tumors.

Ophthalmic complications including retinal detachment in hyperimmunoglobulinemia E (Job's) syndrome: Case report and review of literature.

Hyperimmunoglobulinemia E (Jobs) syndrome is characterized by markedly increased levels of immunoglobulin E, recurrent cutaneous and systemic pyogenic infections, atopic dermatitis, and peripheral eosinophilia. Although ocular involvement in Jobs syndrome is rare, there are reports of keratoconus, staphylococcal chalazia with blepharitis, and Candida endophthalmitis by various authors. We present the first case report of retinal detachment with complicated cataract in Jobs syndrome.

Delleman Oorthuys syndrome: 'Oculocerebrocutaneous syndrome'

Delleman Oorthuys syndrome (oculocerebrocutaneous syndrome) is a rare, congenital sporadic disorder affecting the skin and central nervous system. We present the case of a one-month-old male who presented with an orbital cyst in the left eye since birth along with other manifestations of this syndrome. The manifestations of this syndrome resemble other developmental disorders like Goldenhar and Goltz syndrome. Conservative management of the orbital cyst in these cases have been described. The need to diagnose this rare congenital anomaly with cerebral malformations as a separate entity is crucial in the management of these children.

Atypical presentation of an unusual foreign body.

A 14-year-old boy presented with intractable diplopia for 10 days following an assault. A thorough history revealed that he was unaware of any penetrating injury. However, imaging demonstrated a radiolucent foreign body between the globe and the orbital floor. On surgical exploration, it was found to be the proximal part of a ball point pen. Its removal resulted in complete resolution of diplopia. Thorough clinical and radiological examination is recommended when a foreign body is suspected in pediatric patients. Prompt diagnosis will aid in early intervention and prevention of long-term complications.

Clinical features and management of posttraumatic subperiosteal hematoma of the orbit

Traumatic subperiosteal hematoma (SpH) usually presents late, after the initial trauma. It is generally seen in young males. Computed tomography is the best mode of imaging and helps to rule out orbital fracture or associated subdural hematoma. We present the clinical features and management of four patients seen at the orbit clinic with SpH. Management is based on time of presentation, visual acuity and any communicating bleed. The prognosis of traumatic SpH is excellent if treated with an individualized patient approach.

Neglected giant atypical fibroxanthoma of the eyelid.

Atypical fibroxanthoma is an uncommon neoplasm of the superficial soft tissue that occurs in actinically damaged skin of elderly patients. It is characterized by a pleomorphic histologic appearance but has a generally favorable clinical course. These lesions are usually small in size and rarely occur on the eyelid. The authors present an unusual case of neglected giant atypical fibroxanthoma of the eyelid in an elderly patient.

Case report: Ocular malformation with a 'double globe' appearance

Colobomatous cyst of the orbit is a rare congenital cystic malformation associated with ocular maldevelopment. Usually, the cyst is associated with a microphthalmic globe. We present a rare case of a unilateral large colobomatous cyst associated with a normal-sized globe, giving the appearance of a double globe on imaging.

Orbital hyalinizing spindle cell tumor with giant rosettes.

Purpose: To evaluate the clinical and histopathologic characteristics of orbital hyalinizing spindle cell tumor with giant rosettes (HSCTGR). Methods: Interventional case series of 2 patients, aged 4 and 9 years referred to the Ocular Oncology Unit. Both patients presented with slowly progressive proptosis with dimness of vision in the second patient. CT was performed for both patients followed by excision of the masses. Histology was performed on tissue sections. Results: CT revealed a localized tumor in the first case and extensive mass extending to the optic canal in the second patient. Histologic analysis revealed features of HSCTGR with characteristic spindle-shaped cells with giant rosettes with hyalinized foci. Tumor cells were positive for vimentin in both cases and focal S100 positivity in first case. However, the second case showed a higher Ki-67 index compared to the first case, suggestive of moderately high proliferative activity. After excision, neither of the tumors demonstrated local recurrence, and both patients were without regional or distant metastases. Mean clinical follow-up was 12 months. Conclusions: HSCTGR involving the orbit has not been previously reported in the literature. We report 2 cases of orbital presentation at a much younger age than has been shown in patients with this type of tumor in other areas of the body. Complete excision of this tumor with close follow-up is the preferred treatment. HSCTGR should be considered in the differential diagnosis of orbital fibrous tumor presenting as painless progressive proptosis.