Berçin Tarlan

Subconjunctival hemorrhage: risk factors and potential indicators

Subconjunctival hemorrhage is a benign disorder that is a common cause of acute ocular redness. The major risk factors include trauma and contact lens usage in younger patients, whereas among the elderly, systemic vascular diseases such as hypertension, diabetes, and arteriosclerosis are more common. In patients in whom subconjunctival hemorrhage is recurrent or persistent, further evaluation, including workup for systemic hypertension, bleeding disorders, systemic and ocular malignancies, and drug side effects, is warranted.

Papillary Thyroid Carcinoma: Bilateral Choroidal Metastases with Extrascleral Extension

Here, we present the case of a patient with bilateral choroidal metastases with extraocular extension in one eye. Metastasis of papillary thyroid carcinoma to the uvea is extremely rare, with only 6 patients reported in the literature. A 62-year-old man with a prior history of papillary thyroid carcinoma suffered the rapid loss of vision in his right eye. He had neovascular glaucoma, total retinal detachment, and a solitary choroidal mass. A month later, his left visual acuity also decreased because of a small macular choroidal mass. The right eye was enucleated and a nodular lesion over the sclera representing extraocular extension was observed. This tumor and the intraocular lesion were composed of papillary excrescences and cystic spaces and stained positively for thyroid transcription factor 1 and thyroglobulin, all confirming the diagnosis of metastatic papillary thyroid carcinoma. The tumor in the left eye was successfully treated with diode laser transpupillary thermotherapy. The patient expired within a month as a result of widespread pulmonary metastases. Papillary thyroid carcinoma may metastasize to the uvea bilaterally, cause rapid visual function loss, erode the sclera, and may extend outside the globe similar to choroidal melanoma. This aggressive ocular involvement was associated with a dismal prognosis in our patient.

The treatment of retinoblastoma with four-drug regimen including cisplatin, etoposide, vincristine, and cyclophosphamide.

Over an 11-year period, 59 patients (83 eyes) were treated with four-drug chemotherapy (cisplatin, etoposide, cyclophosphamide, and vincristine) at Hacettepe University, Departments of Ophthalmology and Pediatric Oncology. We evaluated the clinical features, treatment modalities, and outcome of these patients with a median follow-up of 55 months (range 9–130 months). Enucleation was performed as a first-line treatment for 30 eyes due to iris neovascularization and neovascular glaucoma, tumor in the anterior chamber regardless of the tumor stage, and for the patients with the Reese–Ellsworth (RE) group Vb. Chemotherapy was given regardless of tumor stages according to the RE groups in all 59 patients (83 eyes). Fifty-three eyes were treated with chemoreduction (CRD) and focal treatment. The rates of globe preservation were 87% for bilateral tumors and 35% for unilateral tumors in the CRD group. The 5-year overall (OS) and enucleation-free survival (EnFS) was 86.9% and 40%, respectively, for the whole group. At 3rd year, ocular survival rate for the eyes with vitreal or subretinal seeding was 58% and without seeding was 66% (P = .78). Seeding or subretinal collection may not indicate poor prognosis under intensive chemotherapy. The intensive four-drug chemotherapy protocol might have satisfactory results in the retinoblastoma (RBL) patients.

Orbital richter syndrome.

Abstract We report two patients with previously diagnosed chronic lymphocytic leukemia who developed Richter syndrome in the orbit as the sole extranodal site. The medical history, clinical findings, orbital imaging and histopathological features of the patients were reviewed. Treatment protocols and the outcomes were also assessed. The first patient developed Richter syndrome at the age of 64 years, 3 years after the diagnosis of chronic lymphocytic leukemia. The tumor was located at the inferotemporal quadrant of the orbit. The second patient was 59 years old when Richter syndrome arose in the lacrimal gland, 4 years after the diagnosis of chronic lymphocytic leukemia. Incisional biopsy from the orbital tumors were performed. Histopathological findings included diffuse CD20, CD 23, CD5, bcl2, bcl6 positive lymphocytic infiltration. Both patients were treated with chemotherapy and rituximab. During 3 years of follow-up, there was no orbital or systemic recurrence of the disease. Richter syndrome may develop in the orbital soft tissue and the lacrimal gland, and the orbital disease appears to have a better prognosis compared to patients with systemic involvement.

Epitheloid Hemangioendothelioma of the Palpebral Lobe of the Lacrimal Gland

ABSTRACT Purpose: To describe a case of an epitheloid hemangioendothelioma which is a tumor of endothelial origin with borderline malignancy between hemangioma and angiosarcoma and which is very rare in the orbit. Methods: Clinical features, results of imaging and histopathological studies and postoperative clinical course of a 22-year-old female patient who presented with a gradual swelling of the left upper eyelid were reviewed. Results: Magnetic resonance imaging studies suggested a solid lesion with moderate contrast enhancement in the palpebral lobe of the lacrimal gland. A short course of systemic corticosteroid therapy failed to resolve the lesion. Excisional biopsy of the tumor yielded the diagnosis of epitheloid hemangioendothelioma of the lacrimal gland. The tumor was composed of nests of epitheloid cells, some forming CD31 positive intracytoplasmic vascular channels containing erythrocytes. CD34 and EMA were also positive and desmin, SMA, p63, S100, Factor VIII and HHV-8 were negative on immunohistochemical studies. During 44 months of follow-up, there has been no recurrence or systemic metastasis. Conclusion: Epitheloid hemangioendothelioma can occur in the palpebral lobe of the lacrimal gland as a hard, painless, immobile mass. Simple excisional biopsy of the tumor, assumed to have an intermediate malignancy grade, without further local or systemic therapy provided a disease-free 3-year survival.

Langerhans cell histiocytosis of the orbit.

Purpose The management of Langerhans cell histiocytosis is controversial. This study evaluated our clinical experience and therapeutic results in orbital Langerhans cell histiocytosis. Methods This is a retrospective, noncomparative interventional case series involving 17 consecutive patients with biopsy-proven orbital Langerhans cell histiocytosis. Response to surgery and chemotherapy and development of diabetes insipidus were the main outcome measures. Results Thirteen (76.5%) of the patients were male and the mean age at diagnosis was 10.7 years (range 2–39 years). The most frequent presenting sign was proptosis (8 patients) and upper eyelid edema (4 patients). Pain was present in 5 cases and periocular redness in 6. No patient reported a history of trauma. The frontal bone was involved in 16 patients followed by the zygomatic in 9 cases. Five patients also had lesions in the calvarium, femur, facial, temporal, and parietal bones. No patients had systemic disease. Ten patients were managed with vinblastine (0.2 mg/kg, 6–12 months) chemotherapy because of major residual tumor burden (5 cases) and multi-bone involvement (5 cases). Four patients were observed following macroscopically complete tumor removal. Three patients with limited anterior orbital soft tissue tumors and single bone involvement received systemic corticosteroids (40 mg/m2/d, 6–10 weeks). No patients developed diabetes insipidus after a median follow-up of 46 months. Conclusions Macroscopically complete excision of the unifocal tumors may not necessitate any further treatment. Vinblastine chemotherapy following incomplete tumor removal and in patients with multifocal bone disease resulted in recurrence-free survival at 3 years in 90% of patients.

Uveal Melanoma: Current Trends in Diagnosis and Management

Uveal melanoma, which is the most common primary intraocular malignancy in adults, arises from melanocytes within the iris, ciliary body and choroid. The diagnosis is based principally on clinical examination of the tumor with biomicroscopy and indirect ophthalmoscopy and confirmed by diagnostic techniques such as ultrasonography, fundus fluorescein angiography and optical coherence tomography. The clinical diagnosis of posterior uveal melanomas can be made when the classical appearance of a pigmented dome-shaped mass is detected on dilated fundus exam. Uveal melanomas classically show low to medium reflectivity on A-scan ultrasonography and on B-scan ultrasonography the tumor appears as a hyperechoic, acoustically hollow intraocular mass. Management of a suspicious pigmented lesion is determined by its risk factors of transforming into a choroidal melanoma, such as documentation of growth, thickness greater than 2 mm, presence of subretinal fluid, symptoms and orange pigment, margin within 3 mm of the optic disc, and absence of halo and drusen. Advances in the diagnosis and local and systemic treatment of uveal melanoma have caused a shift from enucleation to eye-conserving treatment modalities including transpupillary thermotherapy and radiotherapy over the past few decades. Prognosis can be most accurately predicted by genetic profiling of fine needle aspiration biopsy of the tumor before the treatment, and high-risk patients can now be identified for clinical trials that may lead to target-based therapies for metastatic disease and adjuvant therapy which aims to prevent metastatic disease.

Current treatment of choroidal melanoma

Choroidal melanoma is the most common primary intraocular malignancy in adults. Alternative treatment modalities have been proposed in recent years including enucleation, local resection, plaque brachytherapy, charged-particle radiotherapy, stereotactic photon beam irradiation therapy, transpupillary thermotherapy and photodynamic therapy. This review aims to focus on all the available therapeutic options in choroidal melanoma and update the reader on the current status of each modality. Treatment of choroidal melanomas should be directed towards reducing the risk of recurrences, as it is known that recurrent tumors are associated with an increased rate of metastatic disease and poor prognosis. Advances in genetics and cytogenetics can enhance the accuracy of patient prognostication.

Recurrent subconjunctival hemorrhage due to cavernous hemangioma of the conjunctiva

OBJECTIVE To report 3 patients with conjunctival cavernous hemangioma in association with multiple recurrent episodes of subconjunctival hemorrhage. DESIGN Retrospective clinical case series. PARTICIPANTS We studied 3 patients, 11, 13, and 21 years of age, respectively, each of whom had a small, solitary, and irregular multiloculated vascular mass on the temporal bulbar conjunctiva of the right eye. The tumours were excised for cosmetic reasons. METHODS The medical records and histopathologic slides of the 3 patients were reviewed. RESULTS Histopathologic examination showed that the tumours were composed of large, congested blood vessels separated by thin connective tissue. The patients did not have any recurrence of the tumour or hemorrhage. CONCLUSIONS Cavernous hemangioma had a tendency to develop rapidly in young people on the temporal bulbar conjunctiva of the right eye, causing recurrent subconjunctival hemorrhages.

Adult orbital xanthogranulomas: clinical features and management.

Purpose Adult-onset asthma with periocular xanthogranuloma and adult-onset xanthogranuloma are 2 rare subtypes of non-Langerhans cell histiocytic disorder and much remains unknown regarding optimal treatment. The authors describe their experience in the management of these 2 disease subtypes. Methods This is a retrospective case series with histopathologically proven orbital xanthogranuloma over a period of 12 years. Clinical, imaging, and histopathologic features; associated systemic conditions; treatment modality; and outcome during follow-up of 6 adult patients who had adult-onset asthma with periocular xanthogranuloma and adult-onset xanthogranuloma were reviewed. Results The age range was 29-75 years (median 56 years). The duration of symptoms and signs varied from 10 months to 9 years. All patients had bilateral and asymmetric involvement. Palpebral swelling with yellow discoloration and upper eyelid ptosis were the most common signs. Adult-onset asthma was present in 2 patients. Imaging studies demonstrated ill-defined infiltrative lesions involving the preseptal area, lacrimal glands, extraocular muscles, retrobulbar fat, and optic nerves. The median follow-up was 50 months. Complete regression of all clinical signs was obtained at 8 months, whereas imaging findings disappeared at 18 months with treatment. No recurrence was observed. Conclusions Treatment consisting of debulking as much affected soft tissue as possible followed by a 3-day course of intravenous pulse methylprednisolone administration and then by oral prednisone for at least 6 months may provide adequate regression of the granulomas without recurrence and satisfactory cosmesis in patients with adult orbital xanthogranuloma with and without asthma.