Oncocytic lesions of the ocular adnexa: A review of the histopathology with a brief discussion of the pathogenesis.

Oncocytic lesions may be metaplastic, hyperplastic, or neoplastic and occur in a variety of tissues, including those of the ocular adnexa. Oncocytes are enlarged epithelial cells with abundant eosinophilic granules in the cytoplasm, which represent large mitochondria with distorted cristae. The causes of oncocytic lesions remain uncertain, although in some sites such as the lacrimal sac, chronic inflammation may be a factor. Oncocytic neoplasms in all adnexal sites are generally benign (oncocytoma/oncocytic adenoma) and oncocytic adenocarcinomas are uncommon. Research into oncocytic neoplasms, particularly of the kidney and thyroid, has shed some light on the complicated genomic and metabolic changes that are associated with mitochondrial dysfunction in such neoplasms. The major driver event is mutation of mitochondrial DNA-encoding subunits of complex I in the respiratory chain. The subsequent metabolic events may promote tumorigenesis and inhibit malignant transformation. This review discusses the histopathology and histogenesis of two examples of oncocytoma in the ocular adnexa and presents a simplified synopsis of the genomic and metabolic changes that are significant in the pathogenesis of these neoplasms.

Glasses: Hiding or causing skin cancer?

This article evaluates malignant transformation of lesions presenting in the periocular skin under the eye spectacle nose pad. A non-comparative retrospective chart review of clinical features and pathological findings of patients presenting with periocular malignancies in the exact vicinity where the nose pads of their eye spectacles rested was completed. The study took place in one tertiary oculoplastic referral center between 2007-2013. Ten patients were included, six of whom were male. All subjects wore eye spectacles while awake for at least 15 years, and had an evident suspicious lesion in the exact area that coincided with the resting place of the nose pad. The mean age was 73.5 years (range 65-85 years) and all patients had the lesion present for at least one year. Most cases were squamous skin malignancies (five squamous cell carcinomas [SCC], 2 intra-epidermal carcinomas [IEC], while 3 basal cell carcinomas [BCC]). Treatment involved surgical excision of the lesion with frozen section for margin control and reconstruction with a myocutaneous flap. Periocular malignancies of the inferior medial canthal area, where the nose pad of eye spectacle places pressure, can be easily missed or misdiagnosed. Marjolin ulcers (MU) classically present as an aggressive SCC in area of chronic inflammation, which has been previously correlated to constant pressure, repetitive trauma, or non-healing wounds in other areas of the body. We propose that the traumatic chronic pressure in the infero-medial canthal region from long-term eye spectacle nose pad use, may induce poor lymphatic regeneration leading to an immune system deficiency that predisposes this skin to a malignant transformation. The presence of chronic eye spectacle nose pads also prevents proper and timely detection of such malignancies. Complete excision of these lesions with margin control, adequate follow-up for possible recurrence, and surveillance for new lesions on the patient's contralateral side, is crucial for adequate management.

A Cyst You Can't Miss: A Rare Presentation of an Orbital Apocrine Hidrocystoma.

Apocrine hidrocystomas are benign cystic lesions derived from the sweat glands of Moll and seldom found in the orbit. The authors present a case of a 41-year-old healthy man, with no prior medical history, referred for a painless enlarging mass, medial to his right upper eyelid for the past 3 months. Computed tomography showed a well-defined cystic lesion localized in the supero-medial anterior orbit. Following complete excision of the lesion, histopathology revealed an apocrine hidrocystoma. Although rare, apocrine hidrocystomas should be considered in the differential diagnosis for cystic mass of the orbit at any age group.

Diagnostic challenges in primary orbital fibrosarcoma: a case report.

Fibrosarcoma is a rare and malignant spindle cell tumor of mesenchymal origin that infrequently presents in the orbit. Evolving diagnostic criteria confound the identification of these tumors, as well as the interpretation of previous studies on this unusual entity. We report a case of a 66-year-old man with a mass on his left inferior orbit, with associated paresthesia. A computed tomography (CT) scan showed a lesion on the left anteroinferomedial orbit, with bone erosion. An en bloc surgical excision followed by a thorough immunohistologic evaluation allowed diagnosis of an orbital fibrosarcoma. The patient has had no recurrence after 14 months of follow up. Once a commonly identified soft tissue malignancy, fibrosarcoma has become a diagnosis of exclusion as a result of improved diagnostic and classification techniques, such as immunohistochemistry and fluorescence in situ hybridization (FISH). This type of soft tissue tumor is now an uncommon entity, and we report the first case of a primary orbital fibrosarcoma in an adult, using modern diagnostic and classification methods.

Sphenoid sinus dehiscence as a risk for visual consequences in an immunocompromised patient.

Isolated sphenoid sinus disease is a rare entity with severe and potentially life threatening sequela. Because of the proximity of the sinus to the orbit, anatomical defects within the surrounding bony structures can facilitate communication with orbital content, predisposing the patient to substantial visual consequences. We report a case of a 51-year-old immunocompromised male who presented with headache and gradual unilateral decreases in vision. Computed tomography revealed opacification of the left sphenoid sinus accompanied by unusual bony dehiscence of the proximal optic canal. Early recognition and treatment of sphenoid sinusitis requires urgent surgical intervention with delay of treatment potentially leading to irreversible blindness or other devastating consequences. Bony dehiscence of the sphenoid sinus overlying the optic nerve has only been found in 4% of cadavers. It is associated with increased risk of orbital complications and predicts a poor prognosis. Immediate intervention is particularly important in immunocompromised individuals who are at greater risk of these severe complications.

Orbital solitary fibrous tumor: a case series with review of the literature.

PURPOSE: To report the clinical features, patient demographics, management, and outcomes of a series of patients with orbital solitary fibrous tumors (OSFTs) and provide a review of the English literature describing this rare entity. DESIGN: A review of patient demographics, clinical presentations, imaging, histopathology, surgical management, and outcomes were analyzed. METHODS: A non-comparative retrospective chart review of the demographics, clinical presentations, imaging, histopathological features, management, and disease outcomes of patients presenting to a tertiary orbital center with a tissue diagnosis of OSFT between 2007-2012 was performed along with a review of the English-language literature. RESULTS: Our study included four male patients, with a mean age of 48 years at referral. The most common presentations included a slowly growing mass, globe displacement, diplopia on extreme gazes, and/or facial disfiguration. All tumors were analyzed with histopathology and immunohistochemistry. Treatment involved the complete surgical excision of the lesion, obtaining clear margins. All patients remain alive and well with no evidence of recurrence after a minimum follow-up of 15 months (range 15 months-5 years). CONCLUSION: SFTs should be considered when confronting a painless slowly growing orbital mass that induces globe displacement and/or facial deformity. Imaging will show a well-defined lesion that enhances with contrast. A careful initial surgical excision with clear margins is required for adequate local control of the tumor, avoiding recurrence and potential malignant transformation.

Septic cavernous sinus thrombosis with diffuse spread leading to cerebral ischemia.

INTRODUCTION: Septic cavernous sinus thrombosis (CST) is a rare disease with many etiologies and a diffuse array of initial presentation leading to high mortality. CASE: A 25-year-old male with a history of a nasal furuncle presents with an acute onset of fixed and dilated pupils, bilateral exophthalmos, hemorrhagic chemosis, elevated intraocular pressures, restricted extraocular motility, and unresponsiveness. A retinal septic emboli in the left eye is present on dilated fundoscopic exam while an exudative retinal detachment is present in the right eye. MRI/MRV revealed extensive thrombosis of cavernous sinuses, distal sigmoid dural sinuses, and proximal internal jugular veins with associated cerebral edema and multifocal areas of venous infarction. Blood cultures grew Methicillin-resistant Staphylococcus aureus and the patient suffered multi-system organ failure. Despite treatment with full dose systemic anticoagulation and broad spectrum antibiotics the patient suffered a cardiopulmonary arrest and expired. COMMENT: The acuity of onset coupled with the multiple risk factors for septic cavernous sinus thrombosis in this patient led to a fulminant presentation of this disease and ultimate poor outcome.

Combined surgical and sclerotherapy for an extensive venous-lymphatic orbital anomaly.

Combined venous-lymphatic anomalies (lymphangiomas) of the orbit are nonhereditary, congenital lesions. We report the case of a 6-year-old boy with an extensive right orbital venous-lymphatic anomaly and severe facial deformity who developed 3 intraconal hemorrhages over 3 months. Although the visual acuity was recovered in the first two surgical interventions, the last episode of bleeding resulted in permanent visual acuity loss. At that stage, adjunctive therapy with n-butyl-cyanoacrylate allowed for greater surgical excision. No further episodes of recurrent hemorrhage occurred and the exophthalmos was corrected, thereby improving his overall cosmesis.

Traumatic superior ophthalmic vein thrombosis in a child.

Superior ophthalmic vein thrombosis (SOVT) is a rare condition of multiple etiologies that generally presents with proptosis, ophthalmoplegia, periorbital edema, and occasionally decreased visual acuity. We describe a unique case of a two-year-old child presenting with extensive superior forniceal chemosis obstructing the visual axis after trauma in a motor vehicle accident. Angiography revealed complete thrombosis of the superior ophthalmic vein. Management and outcome are also discussed.

Acute orbital inflammatory syndrome secondary to an ANCA-positive small-vessel vasculitis: a case report and review of the literature.

A 61-year-old man presents with sequential painful bilateral proptosis within 36 h and orbital compartment syndrome resulting in complete loss of vision bilaterally. Sequential urgent lateral canthotomy and cantholysis were performed to reverse the compartment syndrome. Orbital imaging showed non-specific orbital inflammation. Biopsies showed necrotizing inflammation and bloodwork was positive for c-ANCA. The patient was therefore treated with prednisone and cyclophosphomide and showed good recovery of vision in one eye, and had no recurrence of orbital inflammation. ANCA-associated orbital vasculitides are rare, but must be kept in mind in the differential diagnosis of acute orbital inflammatory syndromes.

Eye Popping Disease: Common Characteristics and Management of Spontaneous Globe Subluxation.

PURPOSE: The objective of this study was to describe the clinical features, predisposing factors, and management of 8 new cases of spontaneous globe subluxation (SGS) and provide a review of the relevant literature. DESIGN: Patient demographics, clinical presentations, investigations, management, complications, and outcomes were measured. METHODS: This was a retrospective, case-note analysis of 8 patients with SGS and a major review of the English-language literature. RESULTS: Eight new cases of spontaneous globe subluxation were identified with varying clinical manifestations and treatments. Literature review has shown less than 50 cases. Space-occupying lesions such as thyroid eye disease, shallow orbits, and floppy eyelids were major contributors to predisposition to SGS. CONCLUSIONS: Orbital congestion, shallow orbits, and floppy eyelids can all play a role in predisposing patients to SGS. Successful surgical management of SGS, when required, should be tailored to the individual patient profile for best results.

Apocrine hidrocystoma of the orbit.

Apocrine hidrocystomas are benign cysts of sweat duct origin, originating mainly from the apocrine secretory glands of Moll. They are typically encountered in the head and neck, particularly around the inner canthus of the eyelid. An intraorbital location of this lesion is extremely rare but should be considered in the differential diagnosis of a painless cystic lesion in the ocular adnexa at any age.

Lacrimal caruncle: continuation to the lower eyelid retractors.

PURPOSE: To examine the relationship between the lacrimal caruncle and the lower eyelid retractors and to study the shape and position of the lacrimal caruncle in relation to the lower eyelid margin. METHODS: A transcaruncular approach was used to investigate the direction of the lacrimal caruncle shift in 14 medial orbital decompressions (7 patients) and 10 medial orbital wall fracture reductions (10 patients). Eighty lacrimal caruncles in 40 normal volunteers were examined regarding the position of this structure in relation to the lower eyelid retractors and its shape in connection to the lower eyelid margin. Direct investigation of whether the lower eyelid retractors continued to the lacrimal caruncle was performed during 4 lazy-T procedures (2 patients). RESULTS: All the severed lacrimal caruncles moved inferolaterally. The volunteers' lacrimal caruncles were situated adjacent to the lower eyelid retractors, had an ovoid shape, and were positioned obliquely with an inferolateral direction. In the normal eyelid position, the superior border of the lacrimal caruncle was aligned with the lower eyelid margin. During the lazy-T procedures, the lower eyelid retractors were shown to have direct connection to the lacrimal caruncle. CONCLUSION: The lacrimal caruncle has a direct connection to the lower eyelid retractors that plays an antagonistic role to the medial rectus caspulopalpebral fascia in the movement of the lacrimal caruncle, and it is positioned obliquely, directing inferolaterally with its superior border in level with the lower eyelid margin.

Orbital metastasis: clinical features, management and outcome.

PURPOSE: To review the clinical features, treatment, outcome and survival of metastatic tumors of the orbit. MATERIALS AND METHODS: Retrospective, non-comparative, chart review of 80 patients with orbital metastasis treated in four tertiary orbital centres in Australia. RESULTS: The study included 80 patients of which, 44 were male with a mean age of 60 years. Orbital involvement commonly presented late in a multisystemic disease; however, the orbit was the first presentation in 15% of the cases. Diplopia (48%), pain (42%), and visual loss (30%) were the commonest symptoms at presentation; whereas proptosis (63%), strabismus (62%), and visual loss (41%) were the most frequent clinical signs. Computed tomography commonly showed a solid enhancing mass (42 cases) located within the orbital fat (43%), or enlarging an extraocular muscle (28%). Breast carcinoma (29%), melanoma (20%), and prostatic cancer (13%) were the most frequent histological types. Treatment was often multi-disciplinary and modalities included radiotherapy, chemotherapy, hormone therapy, surgery, and immunotherapy. Survival was limited to 1.5 years after diagnosis independent of the histological type, with 29% of patients alive after 17 months follow-up. CONCLUSIONS: A high index of suspicion and appropriate intervention with histological diagnosis can help in the management and quality of life in patients with metastatic orbital disease. Overall survival is limited and we encountered statistical limitations proving differences in the survival based on the sub-type of primary tumour involved. Metastatic orbital melanoma presented a higher incidence when compared with previous studies, probably due to the increase frequency of skin found in the Australian population.