Clinical Picture
Hanging by threads: ectopia lentis Dipika V Patel, Charles N J McGhee
A 24-year-old man presented with increasingly blurred vision in his right eye. He had previously been diagnosed with bilateral, subluxated crystalline lenses at age 3 years, with subsequent dense amblyopia in the left eye. Visual acuity was 20/40 in the right eye and less than 20/200 in the left eye. After pupil dilation, examination showed that the right lens had posterior subcapsular cataract and modest, inferior, subluxation. By contrast, the left crystalline lens was superiorly subluxated, by approximately 50%, with a dramatic view
of the stretched lens zonules and areas of zonule deficiency (figure). Ectopia lentis is associated with direct trauma and several systemic disorders, most commonly Marfan syndrome, homocystinuria, Weill-Marchesani syndrome, and EhlersDanlos syndrome. The patient was healthy and had no clinical features of Marfan syndrome or other associated diseases. Assessment by a clinical geneticist led to a diagnosis of isolated ectopia lentis. Lens surgery improved visual acuity in the right eye to 20/20.
Published Online April 11, 2014 http://dx.doi.org/10.1016/ S0140-6736(13)61838-3 Department of Ophthalmology, New Zealand National Eye Centre, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand (D V Patel PhD, Prof C N J McGhee DSc) Correspondence to: Dr Dipika V Patel, Department of Ophthalmology, New Zealand National Eye Centre, Faculty of Medical and Health Sciences, University of Auckland, Private Bag 92019, Auckland, New Zealand [email protected]
Figure: Superiorly subluxated lens, associated with stretched zonules and areas of zonule deficiency
www.thelancet.com Published online April 11, 2014 http://dx.doi.org/10.1016/S0140-6736(13)61838-3
1
Hanging by threads: ectopia lentis Dipika V Patel, Charles N J McGhee
A 24-year-old man presented with increasingly blurred vision in his right eye. He had previously been diagnosed with bilateral, subluxated crystalline lenses at age 3 years, with subsequent dense amblyopia in the left eye. Visual acuity was 20/40 in the right eye and less than 20/200 in the left eye. After pupil dilation, examination showed that the right lens had posterior subcapsular cataract and modest, inferior, subluxation. By contrast, the left crystalline lens was superiorly subluxated, by approximately 50%, with a dramatic view
of the stretched lens zonules and areas of zonule deficiency (figure). Ectopia lentis is associated with direct trauma and several systemic disorders, most commonly Marfan syndrome, homocystinuria, Weill-Marchesani syndrome, and EhlersDanlos syndrome. The patient was healthy and had no clinical features of Marfan syndrome or other associated diseases. Assessment by a clinical geneticist led to a diagnosis of isolated ectopia lentis. Lens surgery improved visual acuity in the right eye to 20/20.
Published Online April 11, 2014 http://dx.doi.org/10.1016/ S0140-6736(13)61838-3 Department of Ophthalmology, New Zealand National Eye Centre, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand (D V Patel PhD, Prof C N J McGhee DSc) Correspondence to: Dr Dipika V Patel, Department of Ophthalmology, New Zealand National Eye Centre, Faculty of Medical and Health Sciences, University of Auckland, Private Bag 92019, Auckland, New Zealand [email protected]
Figure: Superiorly subluxated lens, associated with stretched zonules and areas of zonule deficiency
www.thelancet.com Published online April 11, 2014 http://dx.doi.org/10.1016/S0140-6736(13)61838-3
1
![[Hereditary ectopia lentis].](/assets/img/hold.png)
