Schnyder Corneal Dystrophy Clinical Presentation

Updated: Jun 10, 2020
  • Author: Ahmed Farghaly Abdelhameed Omar, MD, PhD; Chief Editor: Hampton Roy, Sr, MD  more...
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Presentation

History

The dystrophy can appear as early as the first year of life. Progression is slow. See the following image.

The natural history of Schnyder dystrophy with age The natural history of Schnyder dystrophy with age. Weiss JS: Schnyder's dystrophy of the cornea: a Swede-Finn connection. Cornea 1992; 11(2): 93-101.
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Physical

Typically, Schnyder corneal dystrophy can be diagnosed clinically. The diagnosis may be more difficult in patients without crystals.

The corneal findings are predictable on the basis of the patient's age. Loss of corneal sensation may be more profound in advanced cases.

Patients who are younger than 23 years demonstrate only a central corneal opacity (as depicted below), which may involve the entire stroma with or without central subepithelial cholesterol crystals. Central corneal mosaic opacities have been reported. These patients possess excellent visual acuity and normal corneal sensation.

A 22-year-old woman with circular corneal opacity A 22-year-old woman with circular corneal opacity best seen in retroillumination. Weiss JS: Schnyder's dystrophy of the cornea: a Swede-Finn connection. Cornea 1992; 11(2): 93-101.

Patients aged 23-39 years develop arcus lipoides as shown below. Snellen acuity may be diminished if measured under photopic conditions. Corneal sensation begins to decrease.

A 37-year-old man with central disclike opacity, a A 37-year-old man with central disclike opacity, affecting the entire stromal thickness, anterior stromal cholesterol crystals, and peripheral arcus lipoides. Weiss JS: Schnyder's dystrophy of the cornea: a Swede-Finn connection. Cornea 1992; 11(2): 93-101.

In patients older than 39 years, a midperipheral, panstromal corneal haze appears that fills in the area between the central opacity and the peripheral arcus.

Often, the arcus is dense enough to be seen without a slit lamp as depicted below.

A 78-year-old woman with dense arcus lipoides in t A 78-year-old woman with dense arcus lipoides in the corneal periphery, sparing the corneal scleral limbus. Weiss JS: Schnyder's dystrophy of the cornea: a Swede-Finn connection. Cornea 1992; 11(2): 93-101.

These patients usually demonstrate an objective loss of visual acuity (which appears worse under photopic conditions) and reduced corneal sensation.

In some members of families with this dystrophy, the presence of xanthelasma associated with elevated levels of serum cholesterol, triglycerides, and lipoproteins has been described.

While scotopic visual acuity may be good, a more accurate assessment of visual function can be obtained by measuring visual acuity under photopic conditions.

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Causes

See Pathophysiology.

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