Oil Drop Cataract

Figure 2.79. The cataract shown on the left is a classical "oil drop" cataract present at birth in an infant with galactosemia which is an autosomal recessive (galactoki-nase deficiency; 9p13) disorder. On the right, the same eye is shown several years later with the cataract almost completely gone after years of good dietary control.

Figure 2.80. The

Hallermann-Streiff syndrome is an autosomal dominant syndrome which is associated with hypotrichosis, mandibular hypoplasia, beaked nose, and endocephaly. The figure on the left demonstrates an early cataract in a child with the Hallermann-Streiff syndrome. The figure on the right is of die same eye a few weeks later showing progression to a dense cataract requiring surgery.

Figure 2.81. Dense bilateral cataracts represent an "ocular emergency" since immediate referral can result in normal vision (20/20) and stereoacuity. This perfect result cannot be obtained if surgery with optical correction has not been completed by the age of 6 weeks following birth.

Figure 2.79. The cataract shown on the left is a classical "oil drop" cataract present at birth in an infant with galactosemia which is an autosomal recessive (galactoki-nase deficiency; 9p13) disorder. On the right, the same eye is shown several years later with the cataract almost completely gone after years of good dietary control.

Galactosemia Oil Droplet Cataract

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