Usha R Kim

Isolated optic nerve infiltration in systemic lymphoma--a case report and review of literature.

Ocular involvement in non-Hodgkin lymphoma occurs either as primary ocular, central nervous system lymphoma or isolated intraocular lymphoma. Recurrence of systemic non-Hodgkin lymphoma in the form of CNS lymphoma has been reported. However, recurrence as an isolated optic nerve lesion without involving CNS has never been reported in the pediatric age group. We report a case of systemic diffuse large B-cell lymphoma in a 2-year-old female, which primarily occurred as suprapubic mass and later recurred in the form of isolated optic nerve infiltration, after remission of the primary disease. Early detection and prompt treatment resulted in complete reversal of the disease.

Spectrum of orbital disease in South India: an aravind study of 6328 consecutive patients.

Aim: To review the incidence of orbital diseases in South India and to compare with other case series published. Methods: Retrospective review of 6328 consecutive patients with orbital disease evaluated at Aravind Eye Hospital between January 1997 and December 2008. The main outcome measure was incidence of orbital disease in South Indian population, as determined by clinical and histopathologic criteria. A literature review was conducted to compare the results of this study with those of previously published reports. Results: Of the 6328 patients, 2161 (34.1%) had inflammatory orbital disease, 1965 (31.0%) had systemic conditions involving the orbit, 1277 (20.1%) had neoplasm, 600 (9.4%) had congenital lesions, 308 (4.8%) had trauma, and 17 (0.2%) had vascular disease. Of the 2161 patients presenting with inflammatory disease, 1473 (68.1%) had idiopathic orbital inflammation, 270 (12.5%) had infection, 126 (5.8%) had dacryoadenitis, and 292 (13.5%) had other etiologies. Among the 1965 patients presenting with systemic disease involving the orbit, 1938 (98.6%) were diagnosed with thyroid orbitopathy, 22 (1.1%) with amyloidosis, and 5 (0.2%) with sarcoidosis. Of the 1277 patients with neoplasm, the tumor was vascular in 369 (28.8%), neural in 336 (26.3%), lymphoid or leukemic in 131 (10.2%), secondary neoplasm in 82 (6.4%), epithelial in 68 (5.3%), adipose in 53 (4.1%), metastatic in 39 (3.0%), and fibrous, fibro-osseous, striated muscle, histiocytic, and other cellular origin in 40 (3.1%), 37 (2.8%), 23 (1.8%), 21 (1.6%), and 78 (6.1%), respectively. Of the 600 patients with congenital lesions, 427 (71.1%) had dermoid and 170 (28.3%) had dermolipoma, followed by meningoencephalocele. Conclusions: The most common causes of orbital disease in South India are inflammatory (34.1%) and systemic conditions (31.0%). With the exception of higher incidence of inflammatory etiologies, these data are largely consistent with prior published reports.

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.

Penetrating orbital injuries from plant material during pond and river diving.

Diving into lakes and ponds is a common activity of rural children. We present two cases of penetrating orbital injuries from plant matter sustained in this manner. Such injuries pose a particular challenge because wooden foreign bodies are often missed during orbital exploration, and current imaging modalities cannot reliably identify retained organic material. When a patient presents with orbital penetration after a high-risk mechanism of injury, such as freshwater diving, the clinician must maintain a very high index of suspicion for retained wooden foreign body.

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.