Bilateral acquired Brown syndrome in systemic scleroderma.
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Biomedical subjects
Publications and source records attributed to Samuray Tuncer.
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PURPOSE: We sought to report the incidence of visual axis opacification and to evaluate the complication and reoperation rates after intraocular lens implantation in infants. METHODS: Twenty-one infants (31 eyes) who had cataract extraction with primary hydrophobic acrylic IOL implantation between October 1996 and May 2002 were reviewed. Posterior capsule was left intact in 14 eyes (group A); posterior capsulorrhexis or capsulotomy with anterior vitrectomy was performed in 17 eyes (group B). Complication and reoperation rates were compared with an age-matched control group of 17 patients (33 eyes) who were left aphakic after pars plicata lensectomy. Mean follow-up period was 41 months (range, 22-75 months) in group A, 37 months (range, 10-75 months) in group B, and 52 months (range, 7-97 months) in the control group. RESULTS: Mean age of the patients was 6.8 months (range, 3-10 months) in group A, 8.9 months (range, 3-18 months) in group B, and 4.9 months (range, 1-15 months) in the control group. Visual axis opacification was significantly higher in group A (86%) when compared with group B (17.6%; P < 0.0001). No significant difference was found in terms of pupillary irregularities and peripheral anterior synechiae formation between pseudophakic and aphakic group ( P = 0.43 and P = 0.306, respectively), whereas pigment dispersion and fibrinous reaction were significantly more common in the pseudophakic group ( P = 0.002). Serious complications, such as retinal detachment, pseudophakic bullous keratopathy, and secondary glaucoma, did not develop in any eye. Reoperation rate was significantly higher in group A (78%) when compared with group B (17%) and the control group (12%; P = 0.0011 and P < 0.0001, respectively). CONCLUSIONS: Visual axis opacification requiring a reoperation was significantly more common in patients with an intact posterior capsule. To decrease the reoperation rate and maintain a clear visual axis, posterior capsulorrhexis with anterior vitrectomy should be performed. Even although early complications were quite frequent, serious late complications were not encountered in any eye. Therefore, under appropriate conditions, IOL implantation is a suitable alternative in infants.
PURPOSE: To report the first case of congenital extraskeletal mesenchymal chondrosarcoma arising in the orbit. DESIGN: Interventional case report and review of the literature. METHODS: Ophthalmologic examination and computed tomography scan of the orbit were performed. Histologic and histochemical examination and immunohistochemistry of the biopsy specimen were evaluated. MAIN OUTCOME MEASURES: Ocular and systemic disease control. RESULTS: A 5-month-old girl was seen with a papillomatous lesion in the right lower tarsal conjunctiva present from birth. Histologic examination demonstrated an admixture of undifferentiated mesenchymal cells and islands of mature hyaline cartilage, and immunohistochemistry studies revealing positivity for vimentin and S-100 were consistent with the diagnosis of mesenchymal chondrosarcoma. The patient was treated with combined chemotherapy (ifosfamide, epirubicin, and cisplatin), radiotherapy, and surgery (exenteration). She is alive with no evidence of disease after 4 years of follow-up. Facial asymmetry and dental malformations have developed as late effects. Further reconstructive surgery is planned for the malformations. CONCLUSIONS: Extraskeletal mesenchymal chondrosarcoma in the orbit is extremely rare. This study reports the first case of congenital extraskeletal mesenchymal chondrosarcoma arising in the orbit (also the youngest patient) both in skeletal and extraskeletal sites. Multimodality treatment (surgery, chemotherapy, and radiotherapy) may lead to long-term survival.
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PURPOSE: To compare the clinical features and outcomes of HLA-B27-positive and HLA-B27-negative acute anterior uveitis (AAU) in a large Turkish patient population. METHODS: Forty-four patients with HLA-B27-positive AAU and 65 with HLA-B27-negative AAU were reviewed retrospectively. HLA-B27 typing, gender, laterality, clinical features (fibrinous reaction, hypopyon formation), ocular complications, medical treatment, and final visual acuity were recorded. Statistical analyses included Student's t-test, Fisher's exact test, Yate's corrected Chi2 test, and Mann-Whitney U test. A p-value of <0.05 was considered statistically significant. RESULTS: Forty percent of all patients with AAU were found to be positive for HLA-B27 antigen. HLA-B27-positive AAU was significantly more common in males and HLA-B27-negative AAU in females. Unilateral or bilateral alternating AAU as well as fibrinous reaction and hypopyon formation were more common in HLA-B27-positive patients. Posterior iris synechiae and cataract formation were more common and systemic immunosuppressive agents were exclusively required in patients with HLA-B27-negative AAU. Visual outcome did not differ significantly between the HLA-B27-positive and HLA-B27-negative groups. CONCLUSIONS: HLA-B27 association is less common in Turkish patients with AAU than in other populations reported in the literature. Although distinct features of HLA-B27 uveitis were confirmed in our study, visual outcome did not differ from idiopathic AAU. Therefore, we believe that HLA-B27 typing in patients with AAU helps the clinician with the diagnosis. However, it has a limited value in determining the prognosis.