Akshay Gopinathan Nair

Intraocular malignant melanoma of the choroid presenting as orbital cellulitis

We report a case of choroidal melanoma with features suggestive of orbital cellulitis. A 24-year-old Asian Indian male presented with a 20-day history of sudden loss of vision in the right eye. Edematous eyelids with complete mechanical ptosis, complete ophthalmoplegia, gross proptosis accompanied by massive chemosis, and prolapse of the inferior forniceal conjunctiva were noted. He denied perception of light in the right eye. The left eye was unremarkable. B-scan ultrasonography of the right eye showed a large dome-shaped mass filling the posterior segment suggestive of choroidal melanoma. Computed tomography confirmed those findings and showed no extraocular tumor extension. The patient was conservatively treated with systemic steroids following which the inflammation subsided. He underwent enucleation of the right eye and a diagnosis of spindle A cell choroidal melanoma was confirmed by histopathological examination. Although rare, orbital cellulitis is one of the presenting features of choroidal melanoma. Uveal melanoma-associated orbital cellulitis can be well controlled with systemic steroids, does not always indicate extraocular tumor extension, and can occur in spindle A cell melanomas.

Conjunctival Leiomyosarcoma: A Report of Two Cases.

ABSTRACT Purpose: Leiomyosarcoma is a common soft tissue tumor in the body. However, ocular leiomyosarcoma is rather uncommon. Herein, we describe the clinical and histopathological features of two cases of conjunctival leiomyosarcoma. There have only been three previously documented cases of conjunctival leiomyosarcoma. Results: A 34-year-old male presented with a 2-year history of a whitish mass in the right eye. He underwent an incisional biopsy of the mass, which supported the diagnosis of leiomyosarcoma on histopathological examination. Computed tomography showed orbital extension of the mass, following which he underwent an eyelid sparing orbital exenteration of the right side. The second case was that of a 39-year-old male, who had a history of a whitish limbal mass, which had been previously excised elsewhere. The pre-operative clinical photographs and histopathology slides of the excised mass were reviewed. A histopathological diagnosis of conjunctival leiomyosarcoma was established and due to base positivity, he was treated with plaque radiotherapy. Both the cases showed no tumor recurrence or systemic metastasis at one-year follow-up. Conclusion: Primary conjunctival leiomyosarcoma is uncommon. Appropriate treatment of the tumor is associated with good prognosis.

Retinal Findings on OCT in Systemic Conditions

ABSTRACT Purpose: Imaging technology has advanced by leaps and bounds in the recent past and has resulted in a much greater understanding of ocular diseases. The aim of this review article is to summarize optical coherence tomography (OCT) findings of various systemic conditions. Method: A systematic literature search of the Medline/PubMed database was performed. English articles up to April 2015 were included. Terms used for search included: Alzheimer’s Disease; Multiple Sclerosis; Parkinson’s Disease; Behçet’s Disease; Schizophrenia; Migraine; Obstructive Sleep Apnea Syndrome; Neurofibromatosis; Sickle Cell Disease; Renal diseases; Lupus Retinopathy; Valsalva Retinopathy; Whiplash Retinopathy; Shaken-Baby Syndrome; Choroidal metastases; Intracranial Hypertension; Drug toxicity; Deferoxamine; Sildenafil; Tamoxifen; Hydroxychloroquine; Chloroquine; Ethambutol; Lead; Sickle Cell Disease; and Thalassemia along with OCT. Results: Studies have shown that inner retinal thinning could be the earliest sign of neurological diseases and may help to differentiate individuals with abnormalities. Outer retinal damage was noted in cancer-related retinopathy and secondary to drug toxicity as a diagnostic sign. This review article summarizes the OCT findings and their importance in early diagnosis, treatment, and follow-up in a varying spectrum of systemic diseases including neurological diseases, hematological diseases, cancer-related retinopathies, and systemic drug toxicity. Conclusion: OCT findings are useful to predict the probability of a disease, to diagnose it early, to differentiate between healthy and unhealthy tissue, and to assess the effect of therapeutic interventions in many systemic diseases.

Minimally invasive surgery for thyroid eye disease.

Thyroid eye disease (TED) can affect the eye in myriad ways: proptosis, strabismus, eyelid retraction, optic neuropathy, soft tissue changes around the eye and an unstable ocular surface. TED consists of two phases: active, and inactive. The active phase of TED is limited to a period of 12–18 months and is mainly managed medically with immunosuppression. The residual structural changes due to the resultant fibrosis are usually addressed with surgery, the mainstay of which is orbital decompression. These surgeries are performed during the inactive phase. The surgical rehabilitation of TED has evolved over the years: not only the surgical techniques, but also the concepts, and the surgical tools available. The indications for decompression surgery have also expanded in the recent past. This article discusses the technological and conceptual advances of minimally invasive surgery for TED that decrease complications and speed up recovery. Current surgical techniques offer predictable, consistent results with better esthetics.

Epibulbar schwannoma in a 12-year-old boy: A case report and review of literature

Schwannomas are benign, encapsulated, primary neurilemmal tumors composed of proliferating Schwann cells. Schwannomas are commonly seen in the orbit, but are rare on the epibulbar surface. Herein, we report a case of a 12-year-old boy who presented to us with a slow-growing painless subconjunctival mass in the left eye. There was no intraocular extension of the mass and intra-operatively, the mass could be clearly delineated and was excised off the underlying sclera. Histopathological examination of the mass showed typical features of schwannoma and immunohistochemistry helped to confirm the diagnosis. There was no recurrence of the lesion observed at follow-up 26 months after surgery. Here, we describe this uncommon tumor and review the available literature. Although rare, an epibulbar schwannoma should be considered in the differential diagnosis of an amelanotic, painless subconjunctival nodular mass. Excision of the lesion is the recommended treatment.

Micturition associated with painless lacrimation

A 34-year-old woman presented to the ophthalmic clinic with complaints of watering from both eyes during micturition. The complaint existed from childhood, and was painless and not associated with any visual complaints. She was distressed as colleagues often mistook her as being overly emotional due to her teary-eyed appearance after visits to the bathroom. Thorough ophthalmic and gyneco-urological examinations were normal. Irrigation of the lacrimal passages showed the drainage pathway to be freely patent. Imaging of the brain and orbit, and blood and urine cultures showed no abnormalities. The phenomenon was witnessed by one of the authors and confirmed. In this communication, we report a rare and rather unusual association of painless lacrimation with micturition. Bulwer et al. have described a similar case in a 3-year-old, who presented with painless lacrimation and micturition. 1 The patient, at the time of micturition, dropped her jaw to show her lower teeth, and had a “vacant” look. Systemic examination failed to find any abnormality. There have been two other cases with similar clinical scenarios, which failed to explain any anatomical or physiological basis for the complaints. 2,3 The facial nerve parasympathetic pathway originates from the superior salivatory or lacrimal nucleus in the pons. The lacrimal nucleus receives inputs from the hypothalamus (emotional stimuli) and the trigeminal nucleus (sensory inputs from the cornea) and finally, through the geniculate and the pterygopalatine ganglia, innverate the lacrimal gland. 1 The Gert’s Nucleus in the sacral cord receives information about bladder contents from the bladder and bladder sphincter, and relays this to the tegmentum of the midbrain (periaqueductal gray), which in turn, excites the pontine micturition center (PMC). The PMC along its long descending pathways to the sacral cord, induces micturition. 4

Orbital signet-ring cell lymphoma of diffuse, large, B-cell type

Signet-ring cell lymphoma (SRCL) is a rare morphologic variant of non-Hodgkin lymphoma with a “signet-ring cell” appearance. It has been described in both B-cell and T-cell lymphomas. We report an orbital SRCL of diffuse large B-cell type in a 60-year-old male, which, to the best of our knowledge, is only the second case to be described in the literature. A 60-year-old male presented to our clinic with complaints of painless, gradually progressive protrusion of his left eye for 18 months. External examination of the left eye showed a firm swelling superomedial and inferomedial to the globe associated with lid swelling (Fig. 1A). The distinct masses were nodular, and the posterior extent could not be ascertained. Orbital rims were intact and the overlying skin was normal. The masses were not freely mobile. Anterior segment evaluation, visual acuity, and fundus examination were normal. Computed tomography showed a well-defined hypodense to isodense, lobulated soft tissue lesion on the left side extending from the preseptal plane to the mid-orbit nasally and into the extraocular muscle cone. Anteriorly the mass extended up to the bridge of the nose; the bony orbit showed no change, and there was no involvement of the nose or sinuses (Fig. 1B, 1C). Microscopic examination of the incision biopsy from the orbital mass showed a diffusely infiltrating cellular tumour composed of large, noncohesive cells in a sclerotic stroma with foci of necrosis. Many of the tumour cells had large, round, cytoplasmic vacuoles and eccentrically placed atypical nuclei (Fig. 2A). Immunostaining was positive for leukocyte common antigen (Fig. 2B) and CD20 (Fig. 2C) in all tumour cells. Fifty-three percent of the cells were immunoreactive for Bcl-6. A positive reaction was seen for lambda light-chain restriction. Ki-67 activity was 70% to 80% (Fig. 2D). The tumour cells were negative for CD5, CD10, CD23, HMB45, pancytokeratin, S100, epithelial membrane antigen, smooth muscle actin, cytokeratin 7, and cytokeratin 20. They were also negative on periodic acid–Schiff (PAS) and Alcian blue staining. Systemic examination and bone marrow examination were normal. Our patient was thus categorized as Ann Arbor stage I and American Joint Committee on Cancer stage T3N0M0. He was started on a CHOP regimen composed of cyclophosphamide, hydroxydaunorubicin, Oncovin (Vincristine), and prednisolone. In addition, external beam radiotherapy (EBRT) of 4000 cGy was also given. A marked reduction in size of the tumour was seen after the first cycle, and a CT scan at the end of third cycle did not show any residual tumour. Ocular adnexal lymphomas are rare, accounting for only 1% to 2% of all lymphomas. Diffuse large B-cell lymphoma (DLBCL) is a high-grade lymphoma that commonly presents with systemic involvement. Most ocular adnexal lymphomas are low-grade extranodal marginal zone lymphomas of B-cell type. Morphologic variants of DLBCL include centroblastic, immunoblastic, T-cell/histiocyte-rich and anaplastic subtypes. SRCL, most commonly described as a variant of follicular lymphoma, has been rarely described in DLBCL. To date, only about 50 cases of SRCL have been described in the literature. Most commonly affecting the lymph nodes, SRCLs have been described to involve the skin, stomach, thyroid, small bowel, and bone marrow. Orbital SRCL is rare, with only 1 such case described previously in the literature. Our case represents the second reported case of orbital SRCL. Based on the morphologic and immunohistochemical features, SRCLs are classified into 3 subtypes: clear vacuole type, Russell

Unilateral optic disc pit associated with orbital cyst in a child

Optic disc pit and disc coloboma are common congenital anomalies. Both have been known to be associated with an orbital cyst. We report a case of a 6 month-old child who presented with unilateral progressive proptosis. Imaging investigations revealed a well-defined, intraconal, orbital cyst located close to the posterior surface of the globe displacing the optic nerve laterally. The cyst was excised and histopathological examination showed the cyst to have a lining of glial tissue, a thin epithelial lining, over a layer of collagenous connective tissue matrix. We hypothesize that colobomatous disc abnormalities and optic disc pits are different points on the same disease spectrum and can be associated with orbital cysts. Furthermore, our case points to the possible role of imaging the optic nerve in all cases of optic disc anomalies.

Life-Threatening Infections

There are very few potentially fatal clinical conditions that present primarily to the ophthalmologist. Majority of these conditions are infective in nature and may have an acute, subacute, or even a chronic course. Three such conditions, cavernous sinus thrombosis, periocular necrotizing fasciitis, and invasive fungal sinusitis, have been reviewed in this chapter. This chapter aims to cover the clinical features, diagnosis, and management of these three clinical entities.

Sebaceous Gland Carcinoma

Sebaceous gland carcinoma (SGC) is a malignant neoplasm of sebaceous origin, which is commonly found in the eyelid and ocular adnexa. The tumor is notorious, for mimicking various benign and malignant clinical entities and for its aggressive local behavior and the potential to metastasize regionally to lymph nodes and to distant organs. Greater awareness among clinicians in the recent years and resection of tumor with margin control, along with modalities like topical Mitomycin-C (MMC) and neoadjuvant chemotherapy, have resulted in better treatment outcomes.