Evaluation of axial length/total corneal refractive power ratio as a potential marker for ocular diagnosis of Marfan’s syndrome in children
Authors:   Department of Ophthalmology and Vision Science, Eye and ENT Hospital of Fudan University, Shanghai 200031, China; NHC Key Laboratory of Myopia (Fudan University), and Key Laboratory of Visual Impairment and Restoration of Shanghai, Shanghai 200031, China, Chen Tian-Hui , Wang Yu-Liang ,   Department of Ophthalmology and Vision Science, Eye and ENT Hospital of Fudan University, Shanghai 200031, China; NHC Key Laboratory of Myopia (Fudan University), and Key Laboratory of Visual Impairment and Restoration of Shanghai, Shanghai 200031, China, Zhang Min ,   Department of Ophthalmology and Vision Science, Eye and ENT Hospital of Fudan University, Shanghai 200031, China; NHC Key Laboratory of Myopia (Fudan University), and Key Laboratory of Visual Impairment and Restoration of Shanghai, Shanghai 200031, China, Chen Jia-Hui ,   Department of Ophthalmology and Vision Science, Eye and ENT Hospital of Fudan University, Shanghai 200031, China; NHC Key Laboratory of Myopia (Fudan University), and Key Laboratory of Visual Impairment and Restoration of Shanghai, Shanghai 200031, China, Zheng Jia-Lei ,   Department of Ophthalmology and Vision Science, Eye and ENT Hospital of Fudan University, Shanghai 200031, China; NHC Key Laboratory of Myopia (Fudan University), and Key Laboratory of Visual Impairment and Restoration of Shanghai, Shanghai 200031, China, Deng Michael ,   Department of Ophthalmology and Vision Science, Eye and ENT Hospital of Fudan University, Shanghai 200031, China; NHC Key Laboratory of Myopia (Fudan University), and Key Laboratory of Visual Impairment and Restoration of Shanghai, Shanghai 200031, China, Ji Ying-Hong ,   Department of Ophthalmology and Vision Science, Eye and ENT Hospital of Fudan University, Shanghai 200031, China; NHC Key Laboratory of Myopia (Fudan University), and Key Laboratory of Visual Impairment and Restoration of Shanghai, Shanghai 200031, China, Jiang Yong-Xiang ,   Department of Ophthalmology and Vision Science, Eye and ENT Hospital of Fudan University, Shanghai 200031, China; NHC Key Laboratory of Myopia (Fudan University), and Key Laboratory of Visual Impairment and Restoration of Shanghai, Shanghai 200031, China
Published: July 2021

AIM: To investigate whether the axial length (AL)/total corneal refractive power (TCRP) ratio is a sensitive and simple factor that can be used for the early diagnosis of Marfan’s syndrome (MFS) in children.
METHODS: The relationship between the AL/TCRP ratio and the diagnosis of MFS for 192 eyes in 97 children were evaluate. The biological characteristics, including age, sex, AL, and TCRP, were collected from medical records. Receiver operating characteristic (ROC) curve analysis was performed to investigate whether the AL/TCRP ratio effectively distinguishes MFS from other subjects. The Youden index was used to re-divide the whole population into two groups according to an AL/TCRP ratio of 0.59.
RESULTS: Of 96 subjects (mean age 7.46±3.28y) evaluated, 56 (110 eyes) had a definite diagnosis of MFS in childhood based on the revised Ghent criteria, 41 (82 eyes) with diagnosis of congenital ectopia lentis (EL) were included as a control group. AL was negatively correlated with TCRP, with a linear regression coefficient of -0.36 (R2=0.08). A significant correlation was found between age and the AL/TCRP ratio (P=0.023). ROC curve analysis showed that the AL/TCRP ratio distinguished MFS from the other patients at a threshold of 0.59. MFS patients were present in 24/58 (41.38%) patients with an AL/TCRP ratio of ≤0.59 and in 34/39 (87.18%) patients with an AL/TCRP ratio of >0.59.
CONCLUSION: An AL/TCRP ratio of >0.59 is significantly associated with the risk of MFS. The AL/TCRP ratio should be measured as a promising marker for the prognosis of children MFS. Changes in the AL/TCRP ratio should be monitored over time.