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Lens dislocation in homocystinuria: about 2 cases

Poster Details

First Author: M.Ez-zahraoui MOROCCO

Co Author(s):    O. Lezrek   C. Daoudi   I. Ben Dali   N. Boutimzine   O. Cherkaoui   M. Laghmari     

Abstract Details

Purpose:

Homocystinuria is an autosomal recessive disorder of methionine metabolism due to cystathionine-beta-synthetase deficiency. Ocular complications are the most constant, and are usually inaugural. Systemic complications involve over all skeletal, central nervous and vascular systems.

Setting:

Department A of Ophthalmology, Teaching Hospital of Rabat, Mohammed V Souissi University, Rabat, Morocco.

Methods:

A 24-year-old patient presented for bilateral redness and painful eye. Slit lamp examination of the left eye showed a temporal scleromalacia and a dislocated cataracted lens in anterior chamber(AC).Intraocular pressure(IOP) was 50mmhg. Examination of the right eye showed a discolted clear lens in AC. Somatic examination revealed a mental retardation. An 8-year-old patient presented for decreased visual acuity. Slit lamp examination showed a clear dislocated lens in AC in both eyes, with an upper scleromalacia and a high intraocular pressure in the right eye. Somatic examination found a mental retardation, a scoliosis and a pectus carinatum

Results:

The management of lens dislocation in the anterior chamber in homocystinuria usually consists in an anterior approach including lens phacophagia and anterior vitrectomy after an appropriate preparation for the anesthesia. Eyeglasses equipment, scleral-fixated Intraocular lens (IOL) and iris-fixated IOL remain methods for correction of aphakia. Our preference is for eyeglasses. Unfortunately the first patient completely refused surgery. For the second patient, the operating follow ups remain simple and quasi painless.

Conclusions:

The management of homocystinuria is not easy. Screening and early treatment are necessary to improve prognosis. Prenatal diagnosis should be suggested in case of family history. Vascular accidents can be life-threatening.

Financial Disclosure:

NONE

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