Marfan syndrome with ocular manifestations

This code identifies Marfan syndrome, a genetic connective tissue disorder, specifically when it presents with ophthalmologic involvement. Ocular manifestations can include lens dislocation (ectopia lentis), myopia, retinal detachment, or glaucoma, which are characteristic features of the syndrome.

Apply this code when documentation clearly states a diagnosis of Marfan syndrome and explicitly details the presence of ocular complications. This is appropriate when the physician has diagnosed Marfan syndrome and linked it to specific eye conditions.

• Ensure documentation specifies which ocular manifestations are present, even if not required for this specific code.
• Do not use if Marfan syndrome is present but without any documented eye involvement; use Q87.40 or Q87.41 instead.
• Code any specific ocular manifestations (e.g., H27.1x for lens subluxation) as secondary diagnoses to provide greater specificity.