Anterior axial lens subluxation, progressive myopia, and angle-closure glaucoma: recognition and treatment of atypical presentation of ectopia lentis.
OBJECTIVE
To describe the clinical features of an atypical presentation of ectopia lentis consisting of primary anterior axial lens subluxation in childhood, associated progressive myopia, and complicating angle-closure glaucoma; to facilitate early diagnosis of this triad; and to report the results of lensectomy for treatment.


METHODS
A retrospective case review of eight patients. Clinical course, visual acuity, gonioscopy, axial length, refraction, fundus examination, associated systemic features, and results of treatment are provided.


RESULTS
This subgroup of patients with ectopia lentis is at risk for progressive, synechial, angle-closure glaucoma. Identification of a clinical triad consisting of rapidly increasing myopia, normal axial length, and progressive axial subluxation leads to earlier diagnosis. Prophylactic lensectomy provides a safe and sight-saving treatment that arrests and, at times, reverses the progression of angle-closure glaucoma.


CONCLUSIONS
Ectopia lentis with anterior axial subluxation and progressive myopia can be a sight-threatening condition when significant forward mobilization of the lens results in synechial angle-closure glaucoma. Recognition of this clinical entity can expedite diagnosis and prevent irreversible loss of vision secondary to glaucoma. Because the primary mechanism of angle closure is angle crowding, peripheral iridotomy does little to control the rise in intraocular pressure. Prophylactic lensectomy has proven to be the most reliable treatment in our experience.
Dagi
Linda R
LR
Children's Hospital Boston, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA. linda.dagi@childrens.harvard.edu
Walton
David S
DS
D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004479:Ectopia Lentis / Q000175:diagnosis* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D015812:Glaucoma, Angle-Closure / Q000175:diagnosis*; D006068:Gonioscopy; D006801:Humans; D007223:Infant; D007429:Intraocular Pressure; D007906:Lens Subluxation / Q000175:diagnosis* / Q000601:surgery; D007908:Lens, Crystalline / Q000601:surgery*; D008297:Male; D009216:Myopia / Q000175:diagnosis*; D012189:Retrospective Studies; D014792:Visual Acuity