OCULAR MANIFESTATIONS OF WEILL-MARCHESANI -SYNDROME - A REVIEW OF 10 CASES

Weill-Marchesani syndrome is an uncommon congenital disease inherited on an autosomal recessive basis. Brachymorphic dwarfism is extremely s uggestive. The severity of the condition is due to its ocular manifest ations, which include a small spherical lens, dislocation of the lens, and glaucoma. Ten cases including four from the same family are revie wed. Dysmorphism and glaucoma were consistent features. Surgery was pe rformed in most cases. An evaluation of medium-and long-term outcomes showed that the prognosis is guarded in the absence of early surgical treatment.