Cases reported "Vision Disorders"

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1/23. Orbital drainage from cerebral arteriovenous malformations.

    OBJECTIVE: To describe the neuro-ophthalmic findings in patients with orbital drainage from cerebral arteriovenous malformations (AVMs). methods: We reviewed the records of 100 consecutive adult patients with cerebral AVMs who presented to our institution during a 4-year period. All patients with orbital drainage were identified, and their neuro-ophthalmic evaluations were reviewed. RESULTS: Three patients (3%) were identified with orbital drainage from a cerebral AVM. The first patient presented with typical chiasmal syndrome (reduced visual acuity, bitemporal hemianopia, and optic atrophy). magnetic resonance imaging demonstrated a large left temporal and parietal lobe AVM with compression of the chiasm between a large pituitary gland and a markedly enlarged carotid artery. The second patient presented with headaches and postural monocular transient visual obscurations. Examination revealed normal visual function with minimal orbital congestion and asymmetrical disc edema, which was worse in the left eye. magnetic resonance imaging revealed a large right parietal and occipital lobe AVM without mass effect or hemorrhage and an enlarged left superior ophthalmic vein. The third patient had no visual symptoms and a normal neuro-ophthalmic examination; a right parietal lobe AVM was discovered during an examination for the cause of headaches. CONCLUSION: Orbital drainage from cerebral AVMs is rare. Manifestations may include anterior visual pathway compression, dilated conjunctival veins, orbital congestion, and asymmetrical disc swelling.
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2/23. Visual electrophysiology in children with tumours affecting the visual pathway. case reports.

    In 9 children (8-14 years of age) with orbital, suprasellar or postchiasmal tumours, visual loss was studied by visual electrophysiology in relation to ophthalmologic and neuroimaging findings. Pattern electroretinography (PERG) and pattern visual evoked potentials (PVEP) to full and half-field pattern-reversal stimulation were recorded and PERG and PVEP changes were related to the tumour location. PERG wave P50 attenuation was found associated with the central retinal dysfunction in the child with orbital rhabdomyosarcoma; PVEP wave P100 delay was associated with the optic nerve dysfunction in a child with retrobulbar chondrosarcoma and in a child with optic nerve glioma; PVEP wave P100 asymmetry was associated with the crossed fibers dysfunction in a child with hypothalamic germinoma, and PVEP wave P100 uncrossed asymmetry was associated with postchiasmal dysfunction in children with postchiasmal tumours (one with pilocytic astrocytoma and two with angioma). On the other hand, normal PERG suggested that there was no central retinal dysfunction in a child with pleomorphic adenoma of the lacrimal gland, and normal PVEP to full and half-field stimulation excluded visual pathway dysfunction at the chiasm in a child with suprasellar arachnoidal cyst. Follow-up was useful in indicating whether visual dysfunction was progressive or not. We conclude that PERG and PVEP findings contributed to understanding whether the dysfunction originated was at the retina, in the optic nerve, chiasm or postchiasmal pathway.
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3/23. The spectrum and significance of primary hypophysitis.

    Hypophysitis can present clinically as a mass lesion of the sella turcica. Secondary hypophysitis occurs in cases where a definite etiologic agent or process inciting the inflammatory reaction can be identified. In contrast, primary hypophysitis refers to inflammation confined to the pituitary gland with no identifiable etiologic associations. We report three cases of primary hypophysitis to illustrate the spectrum of three clinicopathological entities that encompass this disease: lymphocytic hypophysitis, granulomatous hypophysitis, and xanthomatous hypophysitis. Our three patients underwent surgery, with variable response. However, conservative, supportive treatment with or without surgical decompression is generally favored over aggressive and extensive surgical resection that results in hypopituitarism. We conclude that the optimal management of patients with hyophysitis requires a high index of suspicion before extensive surgical resection. Histological confirmation of the diagnosis of hypophysitis can be obtained by performing a biopsy or by requesting an intraoperative frozen section consultation.
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4/23. Intra-sellar salivary gland-like pleomorphic adenoma arising within the wall of a Rathke's cleft cyst.

    Salivary gland rests occur in the posterior lobe of the pituitary gland near or often communicating with the Rathke's cleft or its cystic subdivisions, and are usually incidental autopsy findings. They are attributed to the oropharyngeal development of the Rathke's pouch and may rarely give rise to salivary gland-like tumors in the sella. We present a pleomorphic adenoma, a rare tumor of the sellar region, that has not been previously recognized in association with Rathke's cleft cyst. It occurred in a 44-year-old man who presented with hypopituitarism and reduced vision. magnetic resonance imaging showed a sellar mass with suprasellar extension which was totally removed. It consisted of segments of a cyst wall lined by focally ciliated columnar of cuboid epithelium containing goblet cells. An eosinophilic granular material with cholesterol clefts represented the contents of the cyst. Within its wall there was a tumor with ductal structures and non-ductal varied cellular components including hypercellular areas of spindle and ovoid cells forming interlacing fascicles. Individual cells appeared to float in abundant mucinous material. The appearances were those of a salivary gland pleomorphic adenoma arising within the wall of a Rathke's cleft cyst. The myoepithelial nature of non-ductal tumor cells was confirmed with immunocytochemistry. The existence of seromucous glands communicating with the Rathke's cleft remnants, explains the concomitant occurrence of the tumor and the cyst. This rare neoplasm from salivary gland rest should be considered in the differential diagnosis of unusual sellar and suprasellar tumors.
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5/23. Effect of prostaglandin E1 on ophthalmic artery velocimetry in a pre-eclamptic woman with visual disturbance caused by retinal arterial narrowing.

    We reported the ophthalmic arterial velocimetry and the effect of prostaglandin E1 on that using color Doppler ultrasonography in a 29-year-old pre-eclamptic woman with postpartum weakness of vision due to retinal arterial narrowing. In this case, we found higher pulsatility index and lower mean velocity than that in normal pregnancy. The 30.8% reduction of ophthalmic artery pulsatility index and 53.9% acceleration of mean velocity were observed at 25 min after intravenous administration of prostaglandin E1. Normalization of these values preceded the recovery of vision. These findings suggest a vasodilated effect of prostaglandin E1 on orbital circulation in a pre-eclamptic woman with weakness of vision due to retinal arterial narrowing.
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6/23. Primary adrenal lymphoma with choroidal metastases.

    PURPOSE: To describe a 71-year-old man with bilateral primary adrenal lymphoma with metastases to the choroid. DESIGN: Interventional case report. methods: A 71-year-old man presented with decreased visual acuity in his left eye secondary to an exudative retinal detachment. Abdominal computed tomography and percutaneous biopsy revealed non-Hodgkin large-cell lymphoma involving both adrenal glands. RESULTS: Ocular and adrenal lesions resolved after six cycles of chemotherapy with cytoxan, adriamycin, vincristine, and prednisone. Seven years after his initial diagnosis, the patient reported no recurrence of either ocular or systemic lymphoma. CONCLUSIONS: To our knowledge, this is the first case report of bilateral primary adrenal lymphoma with apparent metastases exclusively to the eye.
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7/23. central serous chorioretinopathy associated with adrenal myelolipoma.

    BACKGROUND: We describe a case of central serous chorioretinopathy associated with a large adrenal myelolipoma. history AND SIGNS: A 55-year old man was admitted to our clinic complaining for metamorphopsia and blurred vision in his left eye. Standard ophthalmologic examination and fluorescein angiography established the diagnosis of central serous chorioretinopathy (CSC). Due to the presence of arterial hypertension, we proceeded to a thorough systemic clinical and laboratory investigation. THERAPY AND OUTCOME: Clinical and laboratory investigation disclosed a large mass in the right abdominal region. magnetic resonance imaging showed that this mass was located superior to the right kidney, in the right adrenal gland, compressing the kidney and the liver. Surgical excision of the mass was done one month later. Histological examination revealed an adrenal myelolipoma. Improvement of CSC was recorded one month after surgery with complete remission two months later. Additionally, systemic blood pressure and increased urinary steroids concentration before the operation returned to normal in the late postoperative period. CONCLUSIONS: CSC pathogenesis is not well understood. Many factors are implicated in this disease. Hypercortisolism and sympathetic activity play a crucial role in the pathogenesis of CSC. This is the first report of CSC in a patient with a benign tumor of the adrenal gland without Cushing's syndrome.
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8/23. pituitary apoplexy causing optic neuropathy and horner syndrome without ophthalmoplegia.

    A 47-year-old woman presented with headache, acute monocular vision loss, and ipsilateral horner syndrome. Apart from the optic neuropathy, all cranial nerve function was intact. magnetic resonance imaging revealed an enlarged pituitary gland with compression of the orbital apex. The surgical specimen was consistent with pituitary apoplexy. The combination of headache, acute visual loss, and ipsilateral horner syndrome without ophthalmoplegia, which may suggest carotid artery dissection, is evidently an unusual manifestation of pituitary apoplexy.
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9/23. Vision impairment as presenting symptom of a sphenoidal mucocele.

    We present a case of a 27-year-old woman with a sphenoid mucocele, presenting with bilateral visual disturbance and pituitary gland dysfunction but without nasal or sinus complaints. We would like to emphasize the importance of early diagnosis and prompt surgical treatment.
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10/23. Rapid response of cystoid macular edema related to Nd:YAG laser capsulotomy to 0.5% ketorolac.

    A 68-year-old woman who had had cataract surgery several years previously presented with decreasing visual acuity and posterior capsular opacification. She underwent an Nd:YAG laser capsulotomy, but developed decreased visual acuity secondary to cystoid macular edema 2 months after the procedure. She was immediately administered 0.5% ketorolac drops. Several days after initiating treatment, she noted an improvement in her vision. Eight days later, her vision had improved from 20/400 to 20/60. Three weeks later, her vision returned to her baseline of 20/30 and a fluorescein angiogram showed resolution of the edema. This may indicate that cystoid macular edema related to Nd:YAG laser capsulotomy is caused by prostaglandin release, and that it may be responsive to topical nonsteroidal drops.
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