To report unilateral anterior dislocation of crystalline lens into the anterior chamber with diffuse corneal edema in a patient with homocystinuria

Homocystinuria is a metabolic disorder caused by deficiency of cystathionine beta synthase with autosomal recessive inheritance. Clinically it is characterized by lens subluxation, skeletal abnormalities and thromboembolic accidents. The patient was a 6-year-old boy, who was a known case of homocystinuria with high serum homocystein level and positive urine homocysteine. The patient had a previous history of thrombotic cerebrovascular infarction at three years old age. He complained from ocular pain prior two weeks in his left eye. In ophthalmic examinations, dislocation of crystalline lens into the anterior chamber with diffuse corneal stromal edema was presented in the affected eye

At initially the patient was treated with topical eye drops of Atropine and Betamethasone, but After 48 hours, due to no response to these medications and corneo-lenticular contact with corneal edema, the patient underwent lens extraction and iris fixated intra ocular lens in the affected eye. In follow-up visits, complaints of the patient including ocular pain and corneal edema was improved

In patients with homocystinuria administration of cycloplegics are the treatment of choice, but clear lens extraction must be indicated if corneo-lenticular contact or pupillary block is presented