Case #18 – Marfan Syndrome

Marfan Syndrome – Page 40 of 50

Orbital Disorders Since the orbit, unlike the retina, cannot be viewed directly, ultrasonography is invaluable in the detection of abnormalities that may be overlooked by ophthalmoscopy. In orbital diagnosis, the B-mode is ideal for the identification and delineation of orbital anomalies. A-mode analysis and dynamic scanning add information to the pattern obtained from

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Marfan Syndrome – Page 39 of 50

Variations in Globe Shape and Size Phthisis Bulbi Eyes with short axial lengths, as in microphthalmos, can also be displayed ultrasonically. Another condition of diagnostic importance revealed ultrasonically in phthisis bulbi, which is shrinkage of the globe. This shrinkage results in disorganization of intraocular tissue such that an amorphous mass of tissue is seen on

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Marfan Syndrome – Page 38 of 50

Variations in Globe Shape and Size Staphylomas A common finding in eyes with long axial lengths, as in high myopia, is posterior staphyloma. Although staphylomas can be detected ophthalmoscopically, they can be dramatically displayed by B-mode ultrasonography. Staphylomas appear as a large, concave depression in the retina-choroid-sclera complex. If the staphyloma is present

Marfan Syndrome – Page 38 of 502021-11-30T22:35:36+00:00

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Peripapillary staphyloma Peripapillary staphyloma or scleral ectasia is a rare congenital anomaly of the optic nerve. It occurs when the most posterior portion of the sclera fails to develop during embryogenesis.4 Patients with this condition have a normal or nearly normal optic nerve head that lies at the bottom of a

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Marfan Syndrome – Page 36 of 50

Drusen of the Optic Nerve Head (Cont'd) At a high-sensitivity setting (see top image) the nerve head appears to be slightly elevated, but no significant information is obtained. At a lower sensitivity setting (see bottom image), all other ocular tissues "drop-out" because they can no longer reflect sound at that sensitivity. The only objects

Marfan Syndrome – Page 36 of 502021-11-30T22:35:21+00:00

Marfan Syndrome – Page 35 of 50

Drusen of the Optic Nerve Head Drusen bodies of the optic nerve head are often visible ophthalmoscopically and can give the optic nerve head the appearance of a cluster of grapes. Buried drusen, however, are not visible to the eye and are perhaps the most common cause of pseudopapilledema. When drusen are buried,

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"Morning Glory" Disc Coloboma DGH B-Scan Scanmate The DGH B-scan Scanmate below is a vertical slices through the optic nerve head and the retinoschisis inferior to the optic nerve head.

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"Morning Glory" Disc Coloboma DGH B-Scan Scanmate The B-scan movie represents a series of vertical slices through the optic nerve head and the retinoschisis inferior to the optic nerve head.

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Marfan Syndrome – Page 32 of 50

"Morning Glory" Disc ColobomaA 49-year old male with history of a unilateral congenital optic nerve abnormality detected five days after birth. The patient is aware of an enlarged blind spot in the left eye which has not changed over the years. Vision is reported to be excellent in each eye although the patient is strongly

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Marfan Syndrome – Page 31 of 50

Retinoschisis Retinoschisis is defined as a splitting of the sensory retina into two or more layers, which most often results in an elevated lesion that resembles an intra-retinal blister. It is typically an acquired disorder that is nearly always found in the peripheral temporal fundus, either superior or inferior. The split is usually

Marfan Syndrome – Page 31 of 502021-11-30T22:34:47+00:00