Case #18 – Marfan Syndrome

Marfan Syndrome – Page 20 of 50

Retinal Detachments A shallow retinal detachment appears as a thin white band (retina), separated from the choroid tissue by a dark space. Normally the retina appears on a B-mode ultrasonogram as a concave, smooth white surface, which is indistinguishable and inseparable from the choroid and sclera. In retinal detachment, however, the retina

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Marfan Syndrome – Page 19 of 50

Intraocular Foreign Bodies (Cont'd) Intraocular metallic foreign body imbedded in the inferior posterior eye wall. Note the "acoustic shadowing" in the orbital fat pads. (Modified from William Jones, OD). The search for and identification of foreign bodies is extremely time-consuming compared with routine ocular diagnosis. Sometimes, foreign bodies are surrounded by hemorrhage. The

Marfan Syndrome – Page 19 of 502021-11-30T22:33:29+00:00

Marfan Syndrome – Page 18 of 50

intraocular Foreign Bodies A steel BB pellet underlies a retinal detachment in this ultrasonogram. The lair or replication echoes seen behind the BB are caused by reflections from the BB pellet itself. Another clinical application of ultrasonography has been the localization of intraocular foreign bodies and the assessment of their metallic characteristics.

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Marfan Syndrome – Page 17 of 50

Asteroid Hyalosis  Asteroid hyaloids or calcium soaps in the vitreous are easy to detect in B-scan ultrasonography. The reflections remain long after the sensitivity is reduced and other ocular structures disappear due to their acoustical density. Asteroid hyalosis is a vitreal condition in which calcium soaps accumulate and disperse throughout the vitreous.

Marfan Syndrome – Page 17 of 502021-11-30T22:33:13+00:00

Marfan Syndrome – Page 16 of 50

Vitreal hemorrhage Vitreal hemorrhage due to ocular trauma. Note, that the retina is attached. Hemorrhages of the vitreous are second only to cataracts as the cause of opaque media. While dense hemorrhages can preclude funduscopic evaluation, ultrasonography is not affected. Dense hemorrhage appears as irregular white or gray areas on the screen. If

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Marfan Syndrome – Page 15 of 50

Vitreal Abnormalities The vitreous is normally acoustically silent, and therefore, appears dark or clear on an ultrasonogram. Besides retinal detachments and fibroproliferative membranes, vitreal abnormalities will result in white reflections on the screen. These include: vitreous hemorrhage asteroid hyalosis inflammatory debris vitreal membranes Not all of these are easily discernible. B-scan ultrasonogram

Marfan Syndrome – Page 15 of 502021-11-30T22:33:01+00:00

Marfan Syndrome – Page 14 of 50

B-scan for Beginners B-mode ultrasonogram of a normal eye. Note the corneal echo on the extreme left. The vitreous is sonolucent and appears dark. The retina-choroid-sclera complex is on the right. Note the sonolucent V-shaped optic nerve and superior and inferior extraocular muscles. Orbital fat, surrounding the optic nerve, reflects sound well.

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Marfan Syndrome – Page 13 of 50

Findings in Marfan syndrome Eye RelatedDislocated lens (in 50-80% of affected individuals, progressive)Refractive error - usually very high myopiaStrabismusGlaucomaRetinal Detachment Non-eye Related Bone overgrowth Loose joints Pectus excavatum (funnel chest) Pectus carinatum (pigeon breast) Dilated aorta Mitral valve prolapsed Tricuspid prolapse Enlargement of the proximal pulmonary artery Aortic tear and rupture (aortic dissection)

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Marfan Syndrome – Page 12 of 50

B-scan Image of Right Eye at Most Recent Visit A retinal detachment (red arrow) utilizing B-scan was imaged as well as an intra-retinal macrocyst (white arrow) which signifies that the retinal detachment was long standing.

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Marfan Syndrome – Page 11 of 50

B-scan Image of Right Eye at Most Recent Visit In the right eye following a different episode of trauma, the decision was to perform a scleral buckle and leave the dislocated lens in place. Note the dislocated lens found in the vitreal cavity OD.

Marfan Syndrome – Page 11 of 502021-11-30T22:32:35+00:00