Primary chondromesenchymal hamartoma of the orbit.

A 14-year-old girl presented with a 3-month history of proptosis of the left eye. Orbital MRI revealed a superiorly located, well-defined, large mass that was hyperintense on T1-weighted and T2-weighted images with heterogenous internal structure and contrast enhancement. The patient underwent superolateral orbitotomy with bone removal. The tumor was excised totally in a piecemeal fashion. Microscopic examination revealed hyaline cartilaginous nodules admixed with spindle cell stroma and bone formation. Immunohistochemically, the mesenchymal component was diffusely positive for vimentin, smooth muscle actin, and CD34. The cartilaginous nodules and stroma stained with S-100. Based on the histopathologic and immunohistochemical findings, a diagnosis of orbital chondromesenchymal hamartoma was made. The patient has been followed for 6 years with no sign of recurrence. A literature search from 1966 to present using the PubMed database yielded no prior reports of primary orbital chondromesenchymal hamartoma. This case demonstrates that chondromesenchymal hamartoma can occur as a primary orbital tumor in children. Awareness of the presence of this tumor is essential for correct diagnosis.

Macular vessel densities by optical coherence tomography angiography in patients with intermediate uveitis

Purpose: To evaluate the macular microvascular changes in patients with intermediate uveitis (IU) using optical coherence tomography angiography (OCT?A) and to determine the relationship of OCT?A measurements with disease duration and activity. Methods: This cross?sectional study was performed at the uvea department of a tertiary hospital. Patients with IU (8 women, 8 men; mean age: 28.80 ± 12.80 years) were included in the study. The macular thickness measurements obtained by optical coherence tomography (OCT) (Heidelberg Spectralis, Heidelberg Engineering, Heidelberg, Germany) and macular superficial and deep vessel density (VD) parameters obtained by OCT?A (Avanti RTVue?XR; Optovue, Fremont, CA, USA) were compared with healthy subjects (12 men, 22 women; mean age: 28.40 ± 8.32 years). Eyes with pathology including marked obvious edema in the macula were not included in the study. Results: The macular thickness was found to be higher in the patient group (p < 0.001). The deep and superficial VD measurements in almost all quadrants in both superficial and deep layers were significantly lower in the IU patient group (p < 0.05). Macular thickness was increased in the active state of IU (p = 0.03), however, none of the OCT?A parameters showed a significant difference between active and inactive IU patients (p > 0.05). No correlation was observed between OCT?A parameters and total IU disease duration. Conclusion: Quantitative analyses of macular vascular structures demonstrate significantly reduced VD in both superficial and deep retinal layers in IU patients. Considering the importance of the macula in visual prognosis, OCT?A can provide crucial data for the monitoring and follow?up of IU patients.