Spherophakia

Spherophakia is a rare, congenital ocular anomaly characterized by a small, spherical lens. This abnormal shape can lead to myopia, lenticular astigmatism, and an increased risk of lens dislocation due to weakened or absent zonular fibers. It is often associated with systemic conditions such as Marfan syndrome, Weill-Marchesani syndrome, and Alport syndrome.

This code is used when documentation specifies a diagnosis of spherophakia. It is appropriate for patients presenting with symptoms related to the abnormally shaped lens, such as severe myopia or lens subluxation/dislocation. Documentation should clearly state "spherophakia" or describe the lens as being small and spherical.

• Always review the medical record for any associated systemic syndromes, which should be coded first if they are the underlying cause.
• Differentiate from other lens anomalies like microphakia (small lens but normal shape) or aphakia (absence of lens).
• Look for documentation of visual disturbances or complications directly attributable to the spherophakia.