9 year old with IOP of 30 OS and 24 OD

Posted by - Posted on 04/18/2016 00:02:16 Glaucoma

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The most common corneal abnormaility associated with this condition is what ?

Diagnosis:  Axenfeld-Rieger syndrome

The most common corneal abnormality associated with A-R syndrome is Posterior embryotoxon (prominent and anteriorly displaced Schwalbe line)

Fun facts

Autosomal Dominant disorder

Adnormal development of neural crest cells leading to abnormal development of anterior chamber angle, iris and trabecular meshwork.

50% develop glaucoma

A-R syndrome systemic associations: defects of teeth, maxillary hypoplasia, redundant periumbical skin, pituitary abnormalities, hypospadias.

DD include

Peters anamoly: corneal opacity with iris adhesions to central cornea. Usually sporadic. 50% develop glaucoma; Assc with central defect in corneal endothelium and descemet's membrane..

ICE or Iridocorneal endothelial syndrome: Unilateral condition, 20-40 year old women,, abnormal corneal endothelium is basic pathology; High PAS with elevated IOP; Iris atrophy with holes; Glaucoma in 50%; 

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