Alejandra A. Valenzuela

Orbital Solitary Fibrous Tumor: A Case Series with Review of the Literature

Abstract 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.

Primary Apocrine Adenocarcinoma of the Eyelid

Primary apocrine adenocarcinoma (PAA) of the eyelid is a rare and potentially life-threatening disease with only 12 cases described in the literature. An aggressive course with metastatic spread at the time of diagnosis is not uncommon and early diagnosis is crucial. We report a case of a PAA with intraorbital extension in a 78-year-old male who was treated successfully with orbital exenteration and radiation.

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.

Septic Cavernous Sinus Thrombosis with Diffuse Spread Leading to Cerebral Ischemia

Abstract 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.

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.

I feel it in my bones: a rare presentation of idiopathic sclerosing orbital inflammation with hyperostosis

Idiopathic sclerosing orbital inflammation (ISOI) is a unique clinicopathological subset of orbital inflammation that is poorly understood and is described in the literature only in case reports or small series. The incidence has been estimated at 5–7.8% of inflammatory orbital lesions, and it is characterized by an insidious, chronic and progressive fibrosing process that damages the orbit and surrounding structures. ISOI has been associated with bone destruction; however, there are no reports with an association of hyperostosis. An 80-year-old African American man presented with orbital pain, diplopia and proptosis of the left eye for several months. On examination, visual acuity was 6/9 in both eyes. Extraocular movements were intact, and slitlamp and fundus examinations were normal bilaterally. Exophthalmometry showed 2-mm left proptosis, with no other abnormalities except for age-related inferior orbital fat and lacrimal gland prolapse bilaterally (Fig. 1a). A computed tomography of the orbits showed an ill-defined left superolateral extraconal mass, along with thickening of the left greater wing of the sphenoid (Fig. 1b). The patient underwent a left lateral orbitotomy without bone removal for an excisional biopsy of the lesion. During surgery, a thick and hyperostotic segment of the left greater wing of the sphenoid was encountered, and could be clearly distinguished by its whiter colour and smooth appearance among the surrounding normal bone. Histopathology revealed the typical features of an ISOI, including densely sclerotic collagenous tissue with a patchy nodular chronic inflammatory infiltrate (Fig. 2). Immunohistochemical analysis revealed a mixture of small CD20positive B cells, small CD3-positive T cells and rare CD138-positive plasma cells. Flow cytometry and B-cell gene arrangement studies showed no evidence of a monoclonal immunoglobulin heavy chain gene rearrangement by polymerase chain reaction. Sections of bone showed sclerotic and fibrotic collagenous tissue with associated patchy nodular lymphoid aggregates suggestive of osteitis. Considering the patient’s advanced age and the discovery of a hyperostotic lesion, a concurrent work-up for an occult malignancy was also performed. The work-up led to an incidental finding of a pleural effusion on chest X-ray, which turned out to be an advanced mesothelioma. The intended immunosuppressive treatment for ISOI was not initiated due to his newly diagnosed lung malignancy, and the patient died 3 months later from his cancer. ISOI is a distinct form of orbital inflammatory disease that typically exhibits fewer inflammatory signs, and has a more chronic and insidious onset. The signs and symptoms, which can include dull pain, proptosis, mild to moderate inflammation and diplopia, are dictated mainly by the anatomical location within the orbit, rather than by the specific nature of the disease. Histologically, the lesion typically depicts paucicellular chronic infiltrate of lymphocytes, plasma cells, histiocytes, neutrophils, eosinophils and a variable amount of fibrosis depending on the chronicity of the lesion. Although ISOI is thought to be limited to the orbit, extraorbital extension and other associated fibrosclerosis disorders have been reported. The sclerosing inflammation occurring in the orbit and in other organs has been linked to immunoglobulin G4-related disease. On imaging, sclerosing inflammation of the orbit generally appears as a homogeneously enhancing soft tissue lesion with irregular yet distinct borders. The involvement of bone in ISOI has not been well documented or described. Hyperostosis is defined as excessive bone growth and can be caused by defective osteoclast function or stimulation of osteoblast, which can be triggered by trauma, infection, vascular problems, metastatic tumours, inflammation, and hormonal and metabolic disorders. In our patient, the most concerning association of hyperostosis was malignancy, prompting a work-up for cancer concomitantly with the surgery. Hyperostosis in the orbit has been associated with metastatic disease, secondary tumours and sphenoid wing meningioma; however, none of these entities were detected in our patient. In regard to the diagnosis of mesothelioma in our patient, there are a few case reports documenting bone metastases to the spine and femur, but no reports of orbital invasion or associated

Multicentred international review of orbital exenteration and reconstruction in oculoplastic and orbit practice

Background Orbital exenteration is a disfiguring procedure reserved for life-threatening malignancies. This study examines the clinical course and outcomes of a large series of patients who underwent orbital exenteration for malignant periocular neoplasms. Methods This is a retrospective review of patients who underwent orbital exenteration from 1 July 2005 to 30 June 2015 at four tertiary referral centres in the USA, Australia and Canada. Demographics, indication for surgery, pathology, surgical technique, reconstruction type and outcomes were reviewed. Results Orbital exenteration was performed on 102 patients. The mean age at surgery was 67.5 years. The most common malignant tumours encountered were squamous cell carcinoma, melanoma and basal cell carcinoma. Seventy-six patients (75%) underwent reconstruction with a local myocutaneous flap, twelve with partial-thickness skin grafts (PTSG), or split skin graft, two had a free flap, and one had a dermis fat graft. Sixteen patients had combined procedures of two of the above. Complete removal of the tumour was achieved with clear margins in 81 cases. Of all patients, 72% were alive at 48 months or more. Conclusion The majority of orbital exenterations performed in this series were secondary to periocular malignancies with unsuccessful/insufficient previous treatments. Regional myocutaneous flaps, PTSG, full-thickness skin grafts and dermis fat grafts were all highly effective and durable reconstructive options, and were able to withstand radiation therapy without complications.

Glasses: Hiding or causing skin cancer?

ABSTRACT 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.

ABSTRACT 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.