Key result
Flatter corneal curvature is linked to ~3.4-fold higher odds of Marfan syndrome in ectopia lentis.
Why the study?
Differentiating Marfan syndrome from non-Marfan causes of congenital ectopia lentis in children is challenging and requires improved diagnostic tools.
Case-Control (n=103)
No
Answers are grounded in this paper and cite their sources.
May aid Marfan diagnosis in pediatric ectopia lentis; hypothesis-generating and requires prospective validation.
Marfan syndrome is an autosomal dominant disorder of the connective tissues. Its major manifestations are in the cardiovascular, musculoskeletal, and ocular systems. Recognizing the phenotypic presentation of tall stature, long limbs and fingers, chest deformity, myopia, midsystolic click, and systolic or diastolic murmur can lead to early diagnosis. Morbidity and mortality are primarily caused by cardiovascular involvement. The goal of medical therapy is to retard the aortic root dilation that leads to sudden death from dissection or rupture. Surgical interventions for mitral valve regurgitation and resection of aortic aneurysms are highly effective. In addition, individuals with Marfan syndrome should be restricted from participation in certain sports.
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Salim et al. (2001) conducted a case-control in Marfan syndrome and congenital ectopia lentis (n=103). Corneal curvature radius z-score (z-CCR) vs. Reference z-CCR was evaluated on Diagnosis of Marfan syndrome (OR 3.40, 95% CI 1.83-6.31, p=<0.001). Flatter corneal curvature (OR 3.40), lower body mass index (OR 0.66), and larger aortic root diameter (OR 2.34) were identified as significant independent predictors for diagnosing Marfan syndrome in children with congenital ectopia lentis.
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