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Title | Creator | Description |
226 |
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Ocular Manifestations of Congenital/Inherited Diseases | Jacqueline A. Leavitt, MD | This 22-year-old woman has neurofibromatosis, type 2. Acuity, color plates, pupillary responses, slit-lamp examination, IOP, fields, and funduscopy are all normal. There is a 3 mm proptosis OS. The patient has recently undergone gamma knife for the acoustic tumor, and she has residual facial nerve p... |
227 |
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Ocular Manifestations of Congenital/Inherited Diseases | Larry P. Frohman, MD | This 14-year-old boy presented with sudden visual loss of the right eye that occurred 3 weeks before and due to a central retinal vein occlusion. His ocular history was quite complicated. He had had a resection of a lymphangioma of the left upper lid at age 7 months and underwent left orbitotomy for... |
228 |
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Ocular Manifestations of Congenital/Inherited Diseases | Larry P. Frohman, MD | This 14-year-old boy presented with sudden visual loss of the right eye that occurred 3 weeks before and due to a central retinal vein occlusion. His ocular history was quite complicated. He had had a resection of a lymphangioma of the left upper lid at age 7 months and underwent left orbitotomy for... |
229 |
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Ocular Manifestations of Congenital/Inherited Diseases | Mark J. Kupersmith, MD | This 9-year-old girl, who had complained of recurrent spontaneous bleeding from the palate and slight swelling and increased warmth over the left cheek, was found to have Wyburn-Mason syndrome. Image 1993_16 shows a small area of arteriovenous shunt on the left optic disc in this patient, who has no... |
230 |
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Ocular Manifestations of Congenital/Inherited Diseases | Daniel M. Jacobson MD | This 38-year-old man had unexplained poor vision in his left eye all his life and was told that he had some type of congenital vascular anomaly. He had no neurocutaneous problems. The photographs demonstrate a complex arteriovenous malformation with dilated loops of veins extending out from the nerv... |
231 |
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Ocular Manifestations of Congenital/Inherited Diseases | William Fletcher Hoyt, MD | Image shows a patient with the Laurence-Moon_Biedl syndrome, an autosomal recessive syndrome characterized by obesity, mental retardation, retinitis pigmentosa, polydactyly, and hypogonadism. Pair with 91_06. |
232 |
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Ocular Manifestations of Congenital/Inherited Diseases | William Fletcher Hoyt, MD | Neurofibromatosis, type 1, is an autosomal dominant phakomatosis characterized by Lisch nodules of the iris (hamartomas) plexiform neurofibromas, café-au-lait spots on the skin, and axillary freckling. Intracranial tumors such as optic pathway gliomas may occur. Disease/Diagnosis: Neurofibromatosis... |
233 |
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Ocular Manifestations of Congenital/Inherited Diseases | Larry P. Frohman, MD | On optic nerve CT scan, this patient with neurofibromatosis, type 1, shows the classic railroad-track sign of optic nerve meningioma and the kink sign of optic nerve glioma. Disease/Diagnosis: Neurofibromatosis, Type 1. |
234 |
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Ocular Manifestations of Congenital/Inherited Diseases | William Fletcher Hoyt, MD | Tuberous sclerosis is an autosomal dominant phakomatosis characterized by astrocytic hamartomas of the retina or optic nerve, adenoma sebaceum of the face, periungual fibromas, and astrocytic hamartomas of the brain, with secondary seizures and mental retardation. Disease/Diagnosis: Tuberous Scleros... |
235 |
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Ocular Manifestations of Congenital/Inherited Diseases | Anthony C. Arnold, MD | Note the retinal/optic nerve head astrocytoma in a 12-year-old boy with tuberous sclerosis. Disease/Diagnosis: Tuberous Sclerosis. |
236 |
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Ocular Manifestations of Congenital/Inherited Diseases | Larry P. Frohman, MD | This girl was 8 years old when these photographs were taken. She had been referred by pediatric neurology with ataxia. When seen, the findings of ataxia, poor convergence, early head thrusting maneuvers, and relatively preserved motility, in conjunction with the conjunctival findings as seen in the ... |
237 |
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Ocular Manifestations of Congenital/Inherited Diseases | Larry P. Frohman, MD | This girl was 8 years old when these photographs were taken. She had been referred by pediatric neurology with ataxia. When seen, the findings of ataxia, poor convergence, early head thrusting maneuvers, and relatively preserved motility, in conjunction with the conjunctival findings as seen in the ... |
238 |
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Ocular Manifestations of Systemic Disorders | Rosa A. Tang, MD | Myasthenia gravis should be considered in any patient with painless, pupil-spared, nonapoptotic ophthalmoplegia. It may mimic any ophthalmoparesis. Involvement of the medical rectus may result in a pseudointernuclear ophthalmoplegia. Pair with 96_24 and 96_25. |
239 |
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Ocular Manifestations of Systemic Disorders | Larry P. Frohman, MD | A 17-year-old girl had undergone multiple superficial biopsies of the orbit for what was felt to be refractory orbital pseudotumor. Initial evaluation revealed the saddle-nose deformity, which the patient confirmed was acquired. More extensive biopsy was consistent with lymphomatoid granulomatosis. ... |
240 |
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Ocular Manifestations of Systemic Disorders | Rosa A. Tang, MD | Myasthenia gravis should be considered in any patient with painless, pupil-spared, nonapoptotic ophthalmoplegia. It may mimic any ophthalmoparesis. Involvement of the medical rectus may result in a pseudointernuclear ophthalmoplegia. Pair with 96_23 and 96_25. |
241 |
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Ocular Manifestations of Systemic Disorders | Rosa A. Tang, MD | Myasthenia gravis should be considered in any patient with painless, pupil-spared, nonapoptotic ophthalmoplegia. It may mimic any ophthalmoparesis. Involvement of the medical rectus may result in a pseudointernuclear ophthalmoplegia. Pair with 96_23 and 96_24. |
242 |
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Ocular Manifestations of Systemic Disorders | Daniel M. Jacobson MD | This 59-year-old man had a 4-week history of malaise, myalgias, night sweats, weight loss, fatigue, and generalized headaches, and a few days before admission he developed a red eye OS secondary to exposure keratitis associated with a left-sided seventh nerve palsy. Subsequent evaluation disclosed a... |
243 |
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Ocular Manifestations of Systemic Disorders | Larry P. Frohman, MD | The same young woman from Case 77 (Image 92_57) presented 8 years after the orbital biopsy with this retinal lesion, presumably from her underlying disorder. The eye has 20/20 vision and no other disturbances. This is a fundus photo. |
244 |
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Ocular Manifestations of Systemic Disorders | Rosa A. Tang, MD | Systemic lymphoma may occur in the orbit and may involve the lacrimal gland. Patients usually present with a painless, progressive proptosis or a mass. CT scan usually demonstrates an irregularly shaped lesion conforming to the globe or lacrimal fossa, and bone erosion is not usually found. Pair wit... |
245 |
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Ocular Manifestations of Systemic Disorders | Rosa A. Tang, MD | Systemic lymphoma may occur in the orbit and may involve the lacrimal gland. Patients usually present with a painless, progressive proptosis or a mass. CT scan usually demonstrates an irregularly shaped lesion conforming to the globe or lacrimal fossa, and bone erosion is not usually found. Pair wit... |
246 |
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Ocular Manifestations of Systemic Disorders | Rosa A. Tang, MD | Thyroid eye disease may cause proptosis and extraocular muscle enlargement that may be seen on orbital imaging studies. In general, coronal images allow the best visualization of the extraocular muscle enlargement. Pair with 94_44 and 94_46. |
247 |
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Ocular Manifestations of Systemic Disorders | Rosa A. Tang, MD | Thyroid eye disease may cause proptosis and extraocular muscle enlargement that may be seen on orbital imaging studies. In general, coronal images allow the best visualization of the extraocular muscle enlargement. Pair with 94_44 and 94_45. |
248 |
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Ocular Manifestations of Systemic Disorders | Robert F. Saul, MD | Wilson's disease (hepatolenticular degeneration) is a progressive autosomal recessive multisystem disease that may result in cirrhosis of the liver, renal dysfunction, and motor neurologic disease. A Kayser-Fleischer ring may occur as a green-brown band at the level of Descemet's membrane in the cor... |
249 |
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Ocular Manifestations of Systemic Disorders | Mitchell J. Wolin, MD | Thyroid eye disease is the most common cause of unilateral or bilateral proptosis in the adult patient. Other signs of thyroid eye disease should be sought, including lid retraction, inferior scleral show, and lid lag. Patients with markedly asymmetric or strictly unilateral proptosis should probabl... |
250 |
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Ocular Manifestations of Systemic Disorders | Mitchell J. Wolin, MD | Thyroid eye disease is the most common cause of unilateral or bilateral proptosis in the adult patient. Other signs of thyroid eye disease should be sought, including lid retraction, inferior scleral show, and lid lag. Patients with markedly asymmetric or strictly unilateral proptosis should probabl... |