Systemic diseases with ectopia lentis

Ectopia lentis refers to the displacement / malposition of the eye's crystalline lens from its normal location.

Systemic diseases with ectopia lentis are given below table-

Disease	Ectopia lentis	Systemic features
Marfan syndrome (Autosomal dominant) Mnemonic: Mast	Superior and temporally	1. Arachnodactyly (spider finger) 2. Hyperextensibility of joint 3. Long extrimities 4. High arch palate 5. Dissecting aortic aneurysm
Homocystinuria	Inferior and nasally	-
Weill Marchesani syndrome	Anterior	1. Anterior dislocation of lens cause pupil block glucoma
Ehlers-Danlos syndrome	Present	1. Blue sclera 2. Hyperextensibility of joint 3. Loose skin with fold
Hyperlysinaemia (deficiency of lysine alpha ketone glutarate reductase)	Occasionally associated	1. Lax ligament 2. Hypotonic muscles 3. Seizures 4. Mental handicap
Sticker syndrome	Occasionally associated	-
Sulphite oxidase deficiency	Present	1. Progressive muscle rigidity 2. Decerebrate posture 3. Mental handicap 4. Fatal disease (death occur before 5 year of age)

Reference:

1. Comprehensive ophthalmology, A K Khurana, 7th edition, P. No. 222-23