RESULTS OF 15 YEARS OF COLLABORATION BETWEEN THE DEPARTMENTS OF OPHTHALMOLOGY AND STOMATOLOGY IN ONCOLOGICAL SURGERY OF THE ORBIT: A DIAGNOSTIC AND THERAPEUTIC APPROACH.

AIM: To report an overview of the most frequent tumors of the orbit, suggest diagnostic approach and possible solution according to experience with own cohort of patients. MATERIAL AND METHODS: From patients files from the Department of Ophthalmology and Department of Stomatology, First Medical Faculty, Charles University, and General Faculty Hospital in Prague, Czech Republic, there were selected patients, who underwent the surgery due to the suspicion of malignant development in the orbit during the period 2005 - 2019. From the surgical records we found information about 497 cases. At the Department of Stomatology, there were 282 surgeries under general anesthesia performed, and at the Department of Ophthalmology, there were 215 surgeries, mostly under local anesthesia performed. RESULTS: The number of surgeries in men and women was equal; patients of all ages were present. The median of patients age operated on at the Department of Stomatology was 53 years, and at the Department of Ophthalmology 63 years. The most common primary benign tumor was the cavernous hemangioma (9 %), the most common non-tumorous expansion was the dermoid cyst (7 %); the most common malignant tumor was the lymphoma (17,5 %). The last mentioned tumor was the most common diagnosis in the whole cohort as well. CONCLUSION: Our cohort of patients is comparable with large cohorts published in the literature concerning age and gender distributions. Differences in frequencies of some lesions may be explained by that our cohort includes patients after the surgery only. The malignant lymphoma is the most common diagnosis indicated to surgical procedure, mostly biopsy. Comparing the two cohorts from our departments 20 years apart, the malignant lymphoma remains the most common indication for surgery, but the incidence of adenomas and adenocarcinomas of the lacrimal gland decreased. It is not the goal of this paper to evaluate all possible orbital affections. Suggested surgical approaches are just recommendations according to years of experience; however, in some situations, to choose an individual surgical approach is necessary.

Macular corneal dystrophy and associated corneal thinning.

PURPOSE: To identify the molecular genetic cause of macular corneal dystrophy (MCD) in four probands, and characterize phenotypic similarities between MCD and keratoconus. METHODS: We performed ophthalmological examination, Scheimpflug imaging (Pentacam, Oculus Inc.), histopathological examination of excised corneal buttons, and direct sequencing of the CHST6 coding region. RESULTS: Pentacam measurements were taken in six eyes of three probands. All showed diffuse corneal thinning with paracentral steepening of the anterior corneal surface that was graded as keratoconus by the integrated software, but without associated ectasia of the posterior corneal surface or regional thinning. Homozygous or compound heterozygous CHST6 mutations were identified in all cases, including two novel mutations, c.13C>T; p.(Arg5Cys) and c.289C>T; p.(Arg97Cys). DISCUSSION: Localized elevation of the anterior corneal curvature can occur in MCD in the absence of other features of keratoconus. The identification of a further two Czech probands with the compound allele c.[484C>G; 599T>G] supports the enrichment of this allele in the study population.