Rita Van Ginderdeuren

Sub-inner limiting membrane haemorrhage: causes and treatment with vitrectomy

Background: Preretinal haemorrhages usually occur at the interface between the posterior hyaloid and inner limiting membrane (ILM). Less frequently, they are located between the ILM and the retinal nerve fibre layer. Sub-ILM haemorrhages have been described in a variety of clinical settings and often lead to severe visual impairment because of their predilection for the macular region. Methods: A consecutive series of five cases in which sub-ILM haemorrhages were clinically suspected and confirmed during early vitrectomy with ILM peeling were reviewed. Results: Sub-ILM haemorrhages were clinically suspected in five patients (median age 32 years) based on the fundoscopic appearance and clinical setting of Terson’s syndrome (n = 1), Valsalva retinopathy (n = 2), blood dyscrasia (n = 1) and blunt facial trauma (n = 1). Vision was severely impaired in all patients (to hand movements in four of five) because of a premacular location of the haemorrhage. All patients were treated with early pars plana vitrectomy because of insufficient spontaneous visual recovery after a median of 6 weeks. The sub-ILM location of the haemorrhage could be confirmed intraoperatively in all patients by biostaining of the membrane overlying the haemorrhage. ILM peeling and aspiration of the haemorrhage resulted in excellent visual recovery in all patients. No procedure-related complications were observed. Conclusions: Sub-ILM haemorrhages often occur in a specific clinical context and can lead to severe visual impairment in young patients. Given the excellent results and low complication rates, timely surgical intervention is justified when spontaneous resorption is insufficient.

Report of a new family with dominant congenital heredity stromal dystrophy of the cornea

Purpose. To report a new family with the rare form of congenital and hereditary stromal dystrophy of the cornea. Methods. A mother and son, showing a bilateral congenital clouding of the cornea, were studied clinically and by biomicroscopy. After corneal transplantation, light microscopy and electron microscopy were performed. Results. The stroma of the cornea was bilaterally and symmetrically thickened with diffuse and homogeneous small opacities. The opacities were present at birth and slowly progressive. Visual acuity was reduced to 2/10. Electron microscopy of the excised corneas showed a thickened stroma owing to cleaving of the lamellae by alternating layers of small-diameter collagen fibrils arranged in a random fashion. The epithelium, Bowmans membrane, the endothelium, and Descemets membrane were normal. Conclusions. This family presents with a congenital stromal dystrophy of the cornea not linked to endothelial defects and thus differs from the more common form of congenital hereditary corneal endothelial dystrophy.

Clinical Presentation, Pathological Features and Natural Course of Metastatic Uveal Melanoma, an Orphan and Commonly Fatal Disease

Background: Uveal melanoma (UM) is a rare disease characterized by an unpredictable course and a variable outcome. We assessed the natural course of patients with metastatic uveal melanoma (MUM). Methods: Data from 76 patients with MUM who were treated in Leuven between 1957 and 2008 were examined retrospectively. Statistical analysis involved the nonparametric, Kaplan-Meier and log rank tests. Results: The median age at diagnosis of UM was 58 years (range 30-94). Synchronous metastases were found in 9% of the cases; all other patients had metachronous disease after a median interval of 40 months (range 7-420). Statistical analysis failed to identify factors related to the time until metastatic spread with the exception of a significant negative correlation with age at UM diagnosis (Spearman: ρ = -0.4, p < 0.001). The most frequent metastatic site was the liver (in 96% of the patients). The median time from diagnosis of UM until death was 47 months (range 2-236) and only 5 months in patients with metastatic disease (range 1-128). Conclusions: MUM is an orphan disease. Advances in MUM can only be achieved by the networking of sites interested in this rare tumor type, with systematic collection of data and tumor tissue for improving our understanding of the molecular biology of this disease.

A new and standardized method to sample and analyse vitreous samples by the Cellient automated cell block system.

In this prospective study, a universal protocol for sampling and analysing vitreous material was investigated. Vitreous biopsies are difficult to handle because of the paucity of cells and the gelatinous structure of the vitreous. Histopathological analysis of the vitreous is useful in difficult uveitis cases to differentiate uveitis from lymphoma or infection and to define the type of cellular reaction.

Precipitation of calcium salts on a hydrophilic acrylic intraocular lens after a vitreous hemorrhage : Case report and histopathologic correlation

&NA; We describe the formation of calcium phosphate deposits on the external anterior surface of a single‐piece foldable 26% hydrophilic acrylic intraocular lens (IOL) after vitreal bleeding. The IOL was explanted 20 months after uneventful phacotrabeculectomy because opacification was causing significant visual disturbance. Light microscopy and Raman spectroscopy of the explanted IOL showed that the opacification consisted of calcium phosphate on the IOL surface.

Juvenile xanthogranuloma of the corneoscleral limbus: report of two cases

Purpose: To report the clinical and histopathologic findings of limbal juvenile xanthogranuloma (JXG) in two children. Methods: Case report. Results: Two children (a 9-month old boy and a 4-year old girl) presented with an enlarging yellowish mass at the corneoscleral limbus. The girl had been diagnosed with neurofibromatosis 1 (NF1). Both lesions underwent simple excision. Histopathologic examination revealed foamy histiocytes within an inflammatory infiltrate with the presence of multiple multinucleated giant cells. In both cases intraoperative subconjunctival steroids and postoperative topical steroids were administered during 1 month. Conclusions: Juvenile xanthogranuloma may rarely present as a mass occurring at the corneoscleral limbus. Therefore it should be considered in the differential diagnosis of any corneoscleral limbal mass lesion, particularly in children. Most are isolated lesions without systemic involvement which are treated by simple excision. But association with NF1 is possible and in that case screening for hematological malignancies is recommended.

Orbital tumor associated with a microphthalmic eye and colobomatous cleft: Pilocytic astrocytoma (glioma) or massive retinal gliosis

A 11-year-old boy with congenital microphthalmos of the right eye presented with gradual protrusion of his ocular prosthesis. MRI showed an orbital mass adjacent to the microphthalmic eye. After removal of the eye and the orbital soft tissue mass a gliotic mass, resembling a pilocytic astrocytoma WHO grade 1 (glioma) was diagnosed. Through a colobomatous cleft in the eye the tumour spread in the orbit. There were no clinical signs of neurofibromatosis 1. This case showed a very rare association between a microphthalmic and colobomatous eye and pilocytic astrocytoma, grade 1. However a far advanced and infiltrative massive retinal gliosis cannot be definitively excluded as differential diagnosis.

The Conjunctiva in Normal Tension Glaucoma Patients is Thinner Than in Primary Open-Angle Glaucoma Patients: A Comparative Histologic Study.

Purpose:To compare histologically the thickness of conjunctival specimens of normal tension glaucoma (NTG) patients with primary open-angle glaucoma (POAG) patients. Materials and Methods:In this prospective study, 54 patients scheduled for trabeculectomy were categorized into NTG and POAG based on their maximum untreated intraocular pressure at any time (IOPmax) as measured by Goldmann applanation tonometry. Sixteen patients with NTG (IOPmax⩽21 mm Hg) and 36 patients with high tension POAG (IOPmax>21 mm Hg) were included in the study. Biopsies were taken from the superior bulbar conjunctiva during trabeculectomy. The specimens were fixed in formalin, embedded in methacrylate, histologically sectioned, stained with toluidine blue, and analyzed with a light microscope. The stromal conjunctival thickness (CT) was measured in a standardized way and compared between the 2 groups. Intergroup comparisons were performed using the Mann-Whitney U test for continuous variables and the Fisher exact test for categorical variables. The correlation between the central cornea thickness (CCT) and the CT was investigated by the Spearman test. Results:The stromal CT was significantly thinner in NTG compared with POAG (64±31 vs. 103±44 µm, respectively; P=0.002). Stromal CT of the whole group was positively correlated with IOPmax (r=0.41; P=0.002; 95% confidence interval, 0.15-0.62) but not with central cornea thickness (r=−0.005; P=0.97; 95% confidence interval, −0.28 to 0.27). Conclusions:The conjunctiva in patients with NTG was thinner than in POAG patients. This finding is an additional feature in the pattern of thinner ocular structures in patients with NTG.

A safety evaluation of the intravitreal use of a beta-2 agonist in rabbit eyes.

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Choroidal Paraganglioma with Metastases to the Fellow Eye

Purpose: To report a case of a paraganglioma in the right eye with metastatic disease in the fellow eye 3 years later. Methods: A 70-year-old man presented with a painful amblyopic right eye; rubeosis iridis and a large choroidal tumor were found. The tumor was treated by enucleation. Pathology diagnosed the tumor as a paraganglioma. Screening for other tumors or metastatic disease was negative at that moment. After 3 years, a paraganglioma skin metastasis was detected, and screening revealed metastatic disease in the liver. Another 6 months later he was referred for tumors in the left eye, which were treated by radiotherapy. He succumbed 6 months later. Results: Histopathology of the right eye revealed the typical image of a paraganglioma, with expression of synaptophysin, neuron-specific enolase and chromogranin. S-100 staining was positive in the sustentacular cells; staining for HMB-45, SME, EMA and pan-keratin was negative. Microscopy of the tumors in the skin and liver 3 years later showed a dedifferentiated tumor with the same immunological characteristics, but with higher Ki67 expression and more mitoses. Conclusions: This report documents a very rare choroidal paraganglioma which presented clinically as a melanoma. The patient succumbed 4 years later to generalized metastatic disease. No other primary paraganglioma was found; however, paraganglion cells in the eye have never been described.