Atlas of Ophthalmology

Patient: 45 years of age, male, IOP 16/16 mmHg, BCVA -6.75-7.75/157° = 0.25 at OD, -7.75-0.5/175°=0.32 at OS. Ocular Medical History: progressive dislocation of the crystalline lens in both eyes, no history of blunt ocular trauma, no signs of Marfan syndrome. General Medical History: diabetes, level of plasma was Hba1c 8%, level of plasma homocysteine was 11.5 µmol/l. Main Complaint: decrease in vision. Purpose: to present a bilateral partial dislocation of the crystalline lens . Methods: Colour Photography Anterior Segment Findings: Colour Photography Anterior Segment: partial dislocation of the crystalline lens in right eye. Colour Photography Anterior Segment: partial dislocation of the crystalline lens in left eye. Discussion: Ectopia lentis classically presents with a preceding history of blunt ocular trauma, or it may be associated with other ocular disorders such as congenital glaucoma and aniridia, or concomitant hereditary systemic diseases such as Marfan syndrome and homocystinuria. Ectopia lentis is a condition in which the crystalline lens is displaced from its normal position in the eye. A zonular weakening occurs in conjunction with a systemic disease like Homocystinuria or Marfan syndrome. Normal levels of plasma homocysteine are between 5–15 µmol/l l and concentrations between 16–30 µmol/l is mild, 31- 100nmol/ml is moderate, and greater than 100 µmol/l is severe hyperhomocystinuria. Homocystinuria is a rare autosomal recessive disorder characterized by inborn error of metabolism due to deficiency of cystathionine beta synthase resulting in systemic accumulation of homocysteine and methionine and causing multisystem disorder of connective tissues, muscles, nervous system, and cardiovascular system. In European populations, prevalence of homocystinuria is around 1 in 10.000. In the presented case we found a level of plasma homocysteine of 11.5 µmol/l. Literatur: (1) Gilbert AL. Bilateral lens dislocation in a patient with homocystinuria. JAMA Ophthalmol. 2015 Mar;133(3):e143538. doi: 10.1001/jamaophthalmol.2014.3538. Epub 2015 Mar 12.

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