The Clinical Spectrum and a New Theory of Pathogenesis of True Exfoliation Syndrome.
To describe the clinical spectrum and a new theory of pathogenesis of true exfoliation syndrome.



Cross-sectional and prospective, observational case series.



Consecutive patients with characteristic peeling of the anterior lens capsule.



After maximal mydriasis, slit-lamp biomicroscopy, and photography, imaging of the anterior capsule and zonules was performed. The condition was classified into 4 clinical stages: annular anterior capsule thickening with a distinct splitting margin (stage 1), an inward detached crescentic flap lying on the anterior lens (stage 2), a floating and folding translucent membrane behind the iris (stage 3), and a broad membrane within the pupil (stage 4). Serial photography was performed at each 3-month follow-up visit. Ultrastructural examination of dislocated lenses and excised anterior capsules was performed.



Detached membrane morphologic features, zonular defects, pigment deposition, glaucoma, phacodonesis, and cataract.



We enrolled 259 patients (424 eyes). Ages ranged from 52 to 97 years (mean age, 75.2±7.1 years). Eleven patients were associated with trauma (n = 1) or intense heat (n = 10), whereas 248 were idiopathic. Two hundred ten patients were followed up every 3 months, with a mean follow-up of 9.6±6.1 months (range, 3-50 months). The detachment started along the anterior zonular insertions in association with zonular disruption. It progressed centrally to higher stages, manifesting a spectrum of disease. Several stages coexisted in a single eye. At the final visit, including 49 patients who were examined once, there were 70, 87, 85, and 17 patients in stages 1, 2, 3, and 4, respectively. All stages shared common histologic findings consisting of diffuse capsular lamellar separation and anterior zonular disruption. All developed cataract. Pigment deposition on the membrane was present in 178 patients (68.7%). Twenty-six patients (10%) had spontaneous phacodonesis. Eighteen eyes (4.2%) demonstrated secondary delamination.



Capsular lamellar separation and anterior zonular disruption are characteristic findings. Aging, heat exposure, and trauma are risk factors. Initial capsular splits occur along the insertions of disrupted anterior zonules. The peeling progresses centrally in association with iris movement and aqueous flow. A second detachment can occur.
Department of Ophthalmology, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. Electronic address: chai1391@gmail.com.
Chaiwat
C
Teekhasaenee
Department of Ophthalmology, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Yanin
Y
Suwan
Department of Ophthalmology, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Wasu
W
Supakontanasan
Department of Ophthalmology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
Wasee
W
Tulvatana
Einhorn Clinical Research Center, New York Eye and Ear Infirmary of Mount Sinai, New York, New York.
Robert
R
Ritch
D000368:Aged; D000369:Aged, 80 and over; D003315:Cornea / Q000000981:diagnostic imaging*; D003430:Cross-Sectional Studies; D017889:Exfoliation Syndrome / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007903:Lens Capsule, Crystalline / Q000000981:diagnostic imaging*; D008297:Male; D033401:Microscopy, Acoustic; D008875:Middle Aged; D008962:Models, Theoretical; D011446:Prospective Studies; D041623:Tomography, Optical Coherence