Optical coherence tomography findings in a child with posterior scleritis.

PURPOSE: To present the findings of optical coherence tomography (OCT) in a child with posterior scleritis. METHODS: A 13-year-old boy was admitted to our clinic with symptoms of low degree of vision and ocular pain. Ophthalmologic examination, ocular ultrasonography, magnetic resonance imaging, fundus fluorescein angiography (FFA), and OCT investigations were carried out and the results were evaluated. RESULTS: Visual acuity was 10/10 in the right eye, and finger counting at 1 meter in the left eye. Anterior segments and vitreous were normal in both eyes. Fundus examination revealed the edema on the macula and the normal disc margins on the left eye, while the right eye was normal. The FFA revealed the leakage on the macula. Posterior scleral thickening, choroidal effusion, and serous retinal detachment were determined in ocular ultrasonography, suggesting posterior scleritis. There were interesting cystic forms underneath the retina and serous macular detachment (SMD) in the first OCT examination. The patient was treated with oral prednisolone for 10 days and then with oral indomethacin for 20 days. Following the systemic treatment the visual acuity of the left eye improved, subjective signs disappeared, and the OCT manifestations returned to normal. CONCLUSIONS: In this case report, OCT provided new information about the retinochoroidal structure in posterior scleritis. These findings may be helpful for the diagnosis of this rare condition, but further studies are needed.

Ultrasound biomicroscopy and OCT findings in posterior microphthalmos.

PURPOSE: Posterior microphthalmos is a rare ocular abnormality. The authors report two siblings with bilateral posterior microphthalmos. METHODS: Two siblings (aged 10 and 13) were admitted with a history of low degree of vision. Ophthalmologic examinations, ocular ultrasonography, ultrasonic biomicroscopy (UBM), and optical coherence tomography (OCT) investigations were carried out and the results were evaluated. All results were also compared with their normal sibling. RESULTS: Two siblings with posterior microphthalmos have normal and near normal anterior segment dimensions, shortened axial lengths (smaller than 17 mm), low vision with high hyperopias, papillomacular folds, and crowded optic discs. OCT investigation showed bilateral papillomacular retinal fold and UBM examination showed that ciliary body moved behind the iris towards the pupilla and the iridocorneal angle anomalies in two siblings. These findings were not observed in the normal sibling. CONCLUSIONS: Posterior microphthalmos can exist in the presence of some ocular anomalies. In this case report, UBM provided new information about the structure of iridocorneal angle and ciliary body.

An unusual cause of central retinal artery occlusion: acquired immunodeficiency syndrome.

PURPOSE: In patients with acquired immunodeficiency syndrome (AIDS), disturbances in the circulation of retinal vessels are mostly encountered at the microvascular level. Rarely observed large retinal vessel occlusions frequently affect retinal veins. METHODS: A 32-year-old woman was admitted to the authors' clinic with sudden loss of vision. Her clinical and ophthalmologic examinations and laboratory tests were carried out and the results were evaluated. RESULTS: The patient's history revealed a diagnosis of AIDS established 5 years ago. Her corrected visual acuity was limited to light perception in the right eye and 20/60 in the left eye. There was afferent pupillary defect in the right eye. Posterior segment examination demonstrated central retinal artery occlusion in the right eye and cotton-wool spots in the left eye. The clinical examination and laboratory test results did not reveal any comorbid disease state that can contribute to this presentation. CONCLUSIONS: As thrombi may develop in patients with human immunodeficiency virus infection, they should be closely followed up for the development of vasoocclusive disease.

Neurofibromatosis type 1 associated with pheochromocytoma: a case report and a review of the literature.

Pheochromocytoma (PHEO) occurs in 0.1-5.7% of patients with neurofibromatosis type 1 (NF1). We report a case of adrenal PHEO in a patient with NF1. A 30-yr-old Turkish man was admitted to our hospital for further examinations of a right adrenal mass, that was incidentally discovered by abdominal ultrasonography during examinations for acute hepatitis B infection in another hospital. In his past medical history, the patient had only had one palpitation, sweating and headache episode 4 yr before. On admission, his blood pressure was 110/70 mmHg. Physical examination revealed signs of NF1. He had multiple neurofibromas over the entire skin, café-au-lait spots on the trunk and extremities and skinfold freckling. Bilateral opthalmic examination revealed multiple Lisch nodules. The 24-h ambulatory blood pressure monitoring revealed paroximal hypertension attacks (190/148 mmHg). Urinary catecholamines were markedly increased. Magnetic resonance imaging (MRI) revealed a solid round tumor approximately 5 cm in diameter, located in right adrenal gland. A 131Iodine-metaiodobenzylguanidine (131I-MIBG) scan showed uptake in the right adrenal gland. The pre-operative treatment with an alpha-blocker (phenoxybenzamine) was performed. Right adrenalectomy was performed; the surgical specimen revealed PHEO. Urine catecholamines and their metabolites returned to normal ranges on post-operative day 7. In conclusion, an adrenal mass can be incidentally discovered in any patient. After diagnosis of NF1, patients who have episodes of hypertension, sweating, headache and palpitation should be evaluated for PHEO.

A novel D458V mutation in the SANS PDZ binding motif causes atypical Usher syndrome.

Homozygosity mapping and linkage analysis in a Turkish family with autosomal recessive prelingual sensorineural hearing loss revealed a 15-cM critical region at 17q25.1-25.3 flanked by the polymorphic markers D17S1807 and D17S1806. The maximum two-point lod score was 4.07 at theta=0.0 for the marker D17S801. The linkage interval contains the Usher syndrome 1G gene (USH1G) that is mutated in patients with Usher syndrome (USH) type 1g and encodes the SANS protein. Mutation analysis of USH1G led to the identification of a homozygous missense mutation D458V at the -3 position of the PDZ binding motif of SANS. This mutation was also present homozygously in one out of 64 additional families from Turkey with autosomal recessive nonsyndromic hearing loss and heterozygously in one out of 498 control chromosomes. By molecular modeling, we provide evidence that this mutation impairs the interaction of SANS with harmonin. Ophthalmologic examination and vestibular evaluation of patients from both families revealed mild retinitis pigmentosa and normal vestibular function. These results suggest that these patients suffer from atypical USH.

Parry-Romberg syndrome associated with Adie's pupil and radiologic findings.

We describe Adie's pupil and radiologic changes related to Parry-Romberg syndrome in a child who presented with facial hemiatrophy with no neurologic deficit. We suggest that cerebral lesions in Parry-Romberg syndrome without neurologic symptoms must be carefully investigated.

The one-month effects of topical betaxolol, dorzolamide and apraclonidine on ocular blood flow velocities in patients with newly diagnosed primary open-angle glaucoma.

PURPOSE: This double-masked, prospective and randomized clinical trial was planned to investigate with color Doppler imaging the 1-month vascular effects of betaxolol, dorzolamide and apraclonidine treatment on patients with newly diagnosed primary open-angle glaucoma (POAG). METHODS: 22 consecutive patients with newly diagnosed POAG between the ages of 46 and 72 years were enrolled in this study. All patients were newly diagnosed cases and had not received any antiglaucoma medication before. Patients who had a systemic vascular disease (including systemic hypertension) or were taking beta-blockers, nitrates or calcium channel blockers were excluded from the study. The patients were randomly divided into three groups. Groups A and B contained 7 patients, group C contained 8 patients. Group A patients were treated with topical betaxolol, group B patients received topical dorzolamide eye drops, and group C patients were treated with topical apraclonidine eye drops. Peak systolic velocities (PSV), end-diastolic velocities (EDV) and resistive indices (RI) in the right ophthalmic arteries (OA), central retinal arteries (CRA) and posterior ciliary arteries (PCA) were measured at baseline by using color Doppler imaging on a masked basis. On days 15 and 30 of treatment, the same measurements were repeated. The inter- and intragroup results were compared statistically. RESULTS: Compared to pretreatment measurements, topical betaxolol therapy significantly decreased PSV only in the PCA and only on day 30 of treatment (p = 0.011). On days 15 and 30, dorzolamide decreased RI measurements in the PCA compared to pretreatment measurement (p = 0.013 and p = 0.011, respectively). Apraclonidine also decreased PSV in the OA on days 15 and 30 of treatment when compared to pretreatment values (p = 0.013 and p = 0.012, respectively). When 15-day measurements were compared between the groups, PSV in the OA were significantly higher in dorzolamide-treated patients compared to other groups (p = 0.01 and p = 0.011). On day 30 of treatment, PSV in the OA was also higher in the dorzolamide-treated group than the other groups (p = 0.012 and p = 0.01). Additionally, apraclonidine-treated patients had a significantly lower EDV in the OA than the other groups (p = 0.013 and p = 0.01). The RI in the OA was also significantly lower in the apraclonidine-treated group compared to the other groups (p = 0.01 and p = 0.011). CONCLUSION: Our study suggests that dorzolamide has the most advantageous 1-month effects on blood flow velocity in the retrobulbar arterial circulation of POAG patients. Betaxolol seems superior to apraclonidine in this regard. Our data may help the clinician when treating patients with POAG medically. Further studies using a larger population size may clarify our results.

Cyclosporine effects on clinical findings and impression cytology specimens in severe vernal keratoconjunctivitis.

PURPOSE: To investigate the possible effects of topical cyclosporine eye drops 2% (CsA) on conjunctival immune cells obtained by impression cytology technique and to determine the clinical relevance of this effect if present. METHODS: Ten consecutive patients with severe, resistant, and active vernal keratoconjunctivitis (VKC) were included in this study. All of them were treated with 2% of topical CsA drops. Symptom and clinical scores and conjunctival impression cytology specimens were obtained both before and following treatment. Impression cytology specimens were stained with anti-CD8+, CD4+, CD45RA+, and CD23+ antibodies and the percentages of positive cells were counted. The results were compared and correlation analyses were performed between clinical and laboratory data. RESULTS: Significant reductions were observed with respect to the percentages of CD4+ and CD23+ cells in the conjunctival impression cytology specimens and clinical and symptom scores following treatment with topical CsA, while no change occurred in the percentages of CD8+ and CD45RA+ cells. The percentages of CD4+ and CD23+ cells showed good correlations with symptom and clinical scores. CONCLUSION: Topical CsA treatment is a very effective alternative in severe VKC cases in clinical ground and clinical efficacy of topical CsA treatment in severe, resistant VKC cases can be (at least partly) related to reduction of the CD23+ and CD4+ cell populations on the conjunctival surface.

Marfan's syndrome, dextrocardia and situs inversus associated with discrete subaortic stenosis and aortic insufficiency in an adult female: case report.

Marfan's syndrome is an inherited connective tissue defect that affects many organs, especially of the musculoskeletal, ophthalmic and cardiovascular systems, and may be associated with some rare conditions. Here, we report the first known case of Marfan's syndrome, combined with situs inversus totalis with dextrocardia and discrete subaortic stenosis and aortic insufficiency in a 22-year-old woman.

Behçet's disease with a large intracardiac thrombus: a case report.

Behçet's disease is recognised as a chronic multisystem disorder with vasculitis as its underlying pathological process. Cardiac involvement is rare and often associated with poor prognosis. A case of a 33 year old man with Behçet's disease, presenting with a large right ventricle and right atrial thrombus, is reported. Two dimensional (cross sectional), colour Doppler, and transoesophageal echocardiography, angiography, computed tomography, and magnetic resonance imaging were used to diagnose the disease. With cyclophosphamide and dexamethasone treatment, the cardiac lesions progressively resolved.

Arterial crimping in branch retinal vein occlusion with macular edema.

PURPOSE: To evaluate the effects of arterial crimping plus grid pattern laser photocoagulation on the macular edema due to branch retinal vein occlusion. PATIENTS AND METHODS: Seventy two eyes (70 patients) with macular edema due to BRVO were divided into two similar groups according to age, gender, location and extent of the occluded area. Grid pattern laser photocoagulation alone was applied in 34 eyes (group A), whereas arterial crimping plus grid pattern laser was used in 38 eyes (group B). Visual acuity, central 30 degree perimetry, and fundus fluorescein angiography were carried out in all patients at each examination. Patients were followed up for an average of 23 mo (ranging from 11 to 34 mo). FINDINGS: A rate of 62% and 79% improvement in macular edema was recorded in groups A and B, respectively (p=0.057). The perimetric performance value improved more in group B than in group A (p=0.028). In addition, visual acuity improved more in group B than group A (p=0.041). CONCLUSION: In patients with BRVO, grid pattern retinal photocoagulation treatment plus arterial crimping resulted in better visual performance two years after treatment than did grid pattern photocoagulation alone.

Tolosa Hunt syndrome: a case report. Clinical and magnetic resonance imaging findings.

A 36-year-old woman was admitted with a left abducens nerve palsy. MR showed enlargement of the left cavernous sinus. The patient was treated with 80 mg oral methyl prednisolone. Clinical findings improved within a month. Two months later, she was readmitted with left oculomotor and right abducens nerve palsy. MR showed significant increase in the volume of the abnormal area in the left cavernous sinus and a new lesion within the right cavernous sinus. After intravenous gadolinium DTPA, there was enhancement in both cavernous sinuses. Methyl prednisolone therapy was again started. After one month of treatment neurological examination was normal. Follow-up MR findings were similar to previous ones.

The importance of plasma polymorphonuclear (PMN) elastase determination in patients with uveitis.

PURPOSE: Polymorphonuclear elastase is used as an important marker for inflammatory disease. We wanted to show whether polymorphonuclear elastase could be a marker in uveitis. METHODS: Twenty-two patients with uveitis and 30 healthy control subjects were included in the study. Polymorphonuclear elastase levels were determined by immunoactivation method before and after periods of corticosteroid and/or cyclosporin treatments. RESULTS: The levels were high in all of the patients before treatment; the levels reached normal range with treatment. In some cases, however, uveitis could not be suppressed in spite of treatment; these patients had high polymorphonuclear elastase levels. CONCLUSION: It was concluded that determination of polymorphonuclear elastase level may be used for monitoring of uveitis.