PURPOSE\nTo measure macular choroidal thickness in normal eyes at different points using enhanced depth imaging (EDI) optical coherence tomography (OCT) and to evaluate the association of choroidal thickness and age.\n\n\nDESIGN\nRetrospective, observational case series.\n\n\nMETHODS\nEDI OCT images were obtained in patients without significant retinal or choroidal pathologic features. The images were obtained by positioning a spectral-domain OCT device close enough to the eye to acquire an inverted image. Seven sections were obtained within a 5 x 30-degree area centered at the fovea, with 100 scans averaged for each section. The choroid was measured from the outer border of the retinal pigment epithelium to the inner scleral border at 500-microm intervals of a horizontal section from 3 mm temporal to the fovea to 3 mm nasal to the fovea. Statistical analysis was performed to evaluate variations of choroidal thickness at each location and to correlate choroidal thickness and patient age.\n\n\nRESULTS\nThe mean age of the 30 patients (54 eyes) was 50.4 years (range, 19 to 85 years), and 14 patients (46.7%) were female. The choroid was thickest underneath the fovea (mean, 287 microm; standard deviation, +/- 76 microm). Choroidal thickness decreased rapidly in the nasal direction and averaged 145 microm (+/- 57 microm) at 3 mm nasal to the fovea. Increasing age was correlated significantly with decreasing choroidal thickness at all points measured. Regression analysis suggested that the subfoveal choroidal thickness decreased by 15.6 microm for each decade of life.\n\n\nCONCLUSIONS\nChoroidal thickness seems to vary topographically within the posterior pole. The thickness of the choroid showed a negative correlation with age. The decrease in the thickness of the choroid may play a role in the pathophysiologic features of various age-related ocular conditions.

Enhanced Depth Imaging Optical Coherence Tomography of the Choroid in Normal Eyes

This case is submitted by Drs. Francisco J. Ascaso, Maria Rojo, and Enrique Minguez from the Department of Ophthalmology, “Lozano Blesa” University Clinic Hospital, Zaragoza, Spain, for the Diagnostic and Therapeutic Challenges Section of Retina; and commented by Dr. Steven M. Cohen, Clearwater, Flo

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Diagnostic and therapeutic challenges.

The authors report a case of Hypohidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome). Diagnosed at the age of 2 months.

Hypohidrotic ectodermal dysplasia

“Dedifferentiation” and/or high-grade transformation (HGT) has been described in a variety of salivary gland carcinomas, including acinic cell carcinoma, adenoid cystic carcinoma, epithelial-myoepithelial carcinoma, polymorphous low-grade adenocarcinoma, myoepithelial carcinoma, low-grade mucoepidermoid carcinoma and hyalinizing clear cell carcinoma, although the phenomenon is a rare event. Recent authors tend to preferably use the term HGT instead of “dedifferentiation” in these cases. HGT-tumors are composed of conventional carcinomas juxtaposed with areas of HG morphology, usually either poorly differentiated adenocarcinoma or “undifferentiated” carcinoma, in which the original line of differentiation is no longer evident. The HG component is generally composed of solid nests, sometimes occurring in cribriform pattern of anaplastic cells with large vesicular pleomorphic nuclei, prominent nucleoli and abundant cytoplasm. Frequent mitoses and extensive necrosis is evident. The Ki-67 labeling index is consistently higher in the HG component. p53 abnormalities have been demonstrated in the transformed component in a few examples, but the frequency varies by the histologic type. HER-2/neu overexpression and/or gene amplification is considerably exceptional. The molecular-genetic mechanisms responsible for the pathway of HGT in salivary gland carcinomas largely still remain to be elucidated. Salivary gland carcinomas with HGT have been shown to be more aggressive than conventional carcinomas with a poorer prognosis, accompanied by higher local recurrence rate and propensity for cervical lymph node metastasis, suggesting the need for wider resection and neck dissection.

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“Dedifferentiation” and High-Grade Transformation in Salivary Gland Carcinomas

Choroideremia (CHM) is an X-linked retinal dystrophy belonging to the family of blinding disorders. It is characterized by progressive degeneration of the choriocapillaris, retinal pigment epithelium and photoreceptors. CHM is caused by mutations in the Rab Escort Protein 1 (REP-1) gene, which encodes a protein involved in vesicular trafficking. This paper gives an overview of the clinical features, visual function, biochemistry, histology, molecular genetics, pathogenesis, diagnosis and treatment of CHM.

Choroideremia: A review of general findings and pathogenesis

Stellate Nonhereditary Idiopathic Foveomacular Retinoschisis

A rare variant of adenoid cystic carcinoma is the dedifferentiated sarcomatoid form, which has previously been reported in the hard and soft palate, maxillary sinus, submandibular glands, and nasal cavity. The authors report the first case of a dedifferentiated sarcomatoid adenoid cystic carcinoma occurring in the lacrimal gland, that of a 52-year-old man. The patient presented with a 4-month history of diplopia, decreased vision, and right upper eyelid swelling. Radiographic imaging showed a soft tissue mass in the extraconal compartment superolateral to the right eye. The patient subsequently underwent surgical debulking. Histologic examination of the tissue revealed classic cribiform adenoid cystic carcinoma and a sarcomatous component consisting of malignant spindle cells and fusiform cells arranged in whorls. Dedifferentiation is a well-established phenomenon in salivary gland tumors that is associated with aggressive behavior and poor prognosis; however, the exact nature of such dedifferentiated neoplasms remains unclear.

Dedifferentiated adenoid cystic carcinoma of the lacrimal gland.

Purpose
The purpose of this study is to report a case of Kyrieleis plaques (segmental retinal periarteritis) associated with cytomegalovirus (CMV) retinitis.

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Kyrieleis plaques in cytomegalovirus retinitis

PURPOSE The purpose of this study was to report recurrent hemolacria, or "bloody tears," as a sign of scleral buckle (SB) infection. METHODS This is an interventional case series of three eyes of three patients with hemolacria after SB placement. RESULTS Two men and one woman were treated for recurrent hemolacria after SB placement for a rhegmatogenous retinal detachment. Two patients had an encircling silicone sponge placed, one 6 years and the other 3 years before presentation. The third patient had a segmental solid silicone element placed 3 months before presentation. Two of the patients reported between 6 to 10 episodes of hemolacria occurring for "years" before referral and diagnosis. In all three patients, hemolacria originated from an occult conjunctival fistula overlying or adjacent to an exposed SB. Microbiological cultures grew Staphylococcus aureus in two eyes and polymicrobial growth in the other. Hemolacria resolved with explantation of the SB in two patients and with long-term continuous topical antibiotics in the other patient. CONCLUSION Hemolacria can be a sign of a SB infection and should raise a high level of suspicion for the presence of an occult conjunctival fistula with exposure of the underlying scleral buckling element when frank exposure is not seen.

Recurrent hemolacria: a sign of scleral buckle infection.

Background: Choroideremia is an X-linked recessive disorder characterized by vision loss with progressive atrophy of the retinal photoreceptors, retinal pigment epithelium (RPE), and choriocapillaris. Ectodermal dysplasia is a heterogeneous group of disorders characterized by a deficiency of two or more ectodermal derivatives. We report on the phenotypic and genetic characteristics of a 29-year-old woman with both choroideremia and ectodermal dysplasia.Materials and Methods: Observational case report with physical and ophthalmic examination, fluorescein angiography (FA), visual field testing, electroretinography, and cytogenetic analysis. This study adhered to the tenets of the Declaration of Helsinki and The New York Eye and Ear Infirmary Institutional Review Board guidelines.Results: Physical and ocular examination revealed hypotrichosis, hypohidrosis, full dentures, meibomian gland hypoplasia, and a decrease in corneal tear film. Visual acuity was hand motions in the right eye and 20/50 in the left eye...

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Krishna Mukkamala

Papers

Stellate Nonhereditary Idiopathic Foveomacular Retinoschisis

Dedifferentiated adenoid cystic carcinoma of the lacrimal gland.

Kyrieleis plaques in cytomegalovirus retinitis

Recurrent hemolacria: a sign of scleral buckle infection.

Choroideremia in a woman with ectodermal dysplasia and complex translocations involving chromosomes X, 1, and 3.