Table 1 of Deng, Mol Vis 2008; 14:1229-1233.

Table 1. Clinical details of three affected members and three carriers in the ectopia lentis family.


II:1 II:8 II:11 III:1 III:6 III:7
Age 67 61 49 36 34 21
Onset age 37 54 42 - - -
Ocular features
Best corrected Visual Acuity (R/L) LP*/1.5 1.0/1.0 1.0/1.0 1.0/1.0 1.0/1.0 1.0/1.0
Ectopia lentis + + + - - -
Secondary glaucoma + + + - - -
Eye operation (R) LE ILV ILV - - -
Eye operation (L) PI ILV ILV - - -
Cornea CL (R) - - - - -
Myopia ? + + + + +
Retina detachment - - - - - -
Skeletal features
Height (cm) 168 160 165 174 165 162
Pectus carinatum - - - - - -
Pectus excavatum - - - - - -
Scoliosis (>20°) - - - - - -
Arachnodactyly - - - - - -
Joint hypermobility - - - - - -
Cardiovascular features
Mitralvalve prolapse - - - - - -
Aortic ascendens dilatation/dissection - - - - - -

R represents right eye; L represents left eye; + represents positive syndrome; - represents negative syndrome; ? represents syndrome is unknown; LP represents light perception; LE represents lensectomy; PI represents peripheral iridotomy; CL represent corneal leukoma; ILS represent intraocular lens suspension; The asterisk indicates that the lens was completely dislocated into the anterior chamber, which induced corneal endothelial decompensation and corneal opacity.

Deng, Mol Vis 2008; 14:1229-1233. http://www.molvis.org/molvis/v14/a144

©2008 Molecular Vision http://www.molvis.org/molvis/

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