Strabismus surgery for diplopia in chronic progressive external ophthalmoplegia.

BACKGROUND: To report midterm outcomes of strabismus strategy for management of diplopia in chronic progressive external ophthalmoplegia and specific surgical planning rationale. DESIGN: Retrospective interventional case series. RESULTS: Two patients, a 26-year-old male and a 36-year-old female, diagnosed with chronic progressive external ophthalmoplegia presented with blepharoptosis and intermittent diplopia. Ocular motility examination was significant for bilateral profound impairment of adduction with relative preservation of abduction, infraduction and elevation. Control of intermittent exotropia gradually worsened over 3 and 1.5 years of follow-up, respectively, in the presence of documented stability of the angle of exodeviation. Strabismus surgery involving modest amounts of bilateral medial rectus resection and lateral rectus recessions was undertaken. Surgical intervention was successful in controlling alignment in primary position and alleviating diplopia and asthenopia after 9 and 8 years of follow-up time, respectively, despite slow progression of ophthalmoplegia. CONCLUSION: Bilateral selective impairment of adduction and intermittent exotropia may be the presenting ocular motility disturbance in chronic progressive external ophthalmoplegia. Properly designed strabismus surgery may provide sustainable, in the midterm, control of alignment and symptomatic relief in selected patients with CPEO.

Unilateral Recession-Resection Surgery for Infantile Esotropia: Survival of Motor Outcomes and Postoperative Drifts.

PURPOSE: To outline the short- and long-term motor outcomes of unilateral medial rectus muscle recession and lateral rectus muscle resection for the correction of moderate angle infantile esotropia. METHODS: A retrospective study of 109 consecutive patients with moderate angle infantile esotropia treated with graded unilateral recession-resection surgery. Criteria for successful motor outcome included alignment ±10Δ from orthophoria. Outcome evaluation was a comparison of successful alignment versus an overcorrection or undercorrection at eight weeks postoperatively as well as on the final follow-up examination. RESULTS: The mean preoperative deviation was 35.5 prism diopters (Δ) and mean follow-up time was 4.9 years. At the eight-week postoperative examination, 99 patients (89.9%) were successfully aligned, as opposed to 75 of 95 patients (78.9%) at the final postoperative visit (P=0.041). There was no statistically significant difference between the rate of early versus late undercorrections (7.3% versus 12.5%, P=0.267) or overcorrections (2.7% versus 8.3%, P=0.125). Ten patients had an esotropic drift over time and 10 patients had an exotropic drift. Recurrent esotropia was associated with high hyperopia and presumed infantile esotropia diagnostic entity. The Kaplan-Meier estimate of survivorship of a successful motor outcome was 75.5% at five years and 71% at 15 years postoperatively. The mean response to surgery was 2.9Δ per mm of muscle recessed and resected and was positively related to the preoperative angle of deviation (R=0.615). CONCLUSIONS: The unilateral recession-resection procedure for the correction of infantile esotropia is shown to be associated with a favorable survival of motor outcomes and a relatively balanced rate of undercorrections versus overcorrections tending to be maintained through the follow-up period.

Polymorphism analysis of COL4A3 and COL4A4 genes in Greek patients with keratoconus.

BACKGROUND: In this study, we conducted the genotyping of D326Y in COL4A3 and M1327V, as well as F1644F in COL4A4 polymorphisms, in a case-control sample panel of Greek origin population. MATERIALS AND METHODS: A case-control panel, with 45 keratoconus (KC) patients and 78 healthy controls, were surveyed. DNA from each individual was tested for the D326Y in COL4A3 and M1327V, as well as F1644F in COL4A4 polymorphisms by direct sequencing. RESULTS: When analyzing the Hardy-Weinberg equilibrium, we observed no significant deviation from expected numbers in both KC patients and controls. The genotype frequencies in the polymorphisms tested were not found to be significantly associated with KC development risk. The M1327V AA and F1644F TT genotypes were significantly over-represented in healthy individuals. CONCLUSIONS: We could hypothesize that mutations in COL4A3 and COL4A4 genes are not involved in KC risk in Greek population. Nevertheless, the M1327V AA and F1644F TT genotypes were significantly over-represented in healthy individuals, suggesting a protective role of these genotypes in KC development risk in our population.

Three horizontal muscle surgery for large-angle infantile or presumed infantile esotropia: long-term motor outcomes.

IMPORTANCE: Bilateral medial rectus muscle recession and one lateral rectus muscle resection surgery for the correction of large-angle infantile esotropia may be associated with a favorable long-term motor outcome. A consecutive exotropic drift was encountered more commonly than a recurrent esotropic drift in the long run, especially in the smaller (50-69 prism diopters [Δ]) range of preoperative esodeviation. OBJECTIVE: To outline the short- and long-term motor outcomes of graded bilateral medial rectus muscle recession and one lateral rectus muscle resection for the correction of large-angle esotropia (≥50Δ). DESIGN: Retrospective analysis. SETTING: Strabismus service, tertiary care university referral center. PARTICIPANTS: A total of 194 consecutive patients with infantile or presumed infantile esotropia. EXPOSURE: All patients underwent bilateral medial rectus recession and a lateral rectus resection in the nondominant eye by the same surgeon. MAIN OUTCOMES AND MEASURES: Short-term (8 weeks) and long-term rates of postoperative successful alignment (±10Δ), undercorrection, and overcorrection. RESULTS: The median age of patients at surgery was 2.7 years (range, 20 months-36 years). The median follow-up time was 4.5 years (range, 6 weeks-25 years). The mean preoperative deviation was 68.2Δ. Of the 194 patients, 121 (62.4%) were successfully aligned at the last follow-up visit or prior to reoperation, and 154 (79.4%) were successfully aligned at the 8-week postoperative evaluation. A comparison of early vs late outcomes revealed a higher rate of late overcorrections (5.15% vs 24.1%, respectively; P = .001) but the same rate of undercorrections (15.4% vs 15.1%; P = .85). The outcome of surgery was not associated with the presence of amblyopia, high hyperopia, or the total amount of millimeters of surgery but was adversely influenced by the presence of inferior oblique overaction and the magnitude of the preoperative esodeviation. Delayed consecutive exotropia was more prevalent in the 50Δ to 69Δ range of preoperative esodeviation. CONCLUSIONS AND RELEVANCE: Three horizontal muscle surgery for the correction of large-angle esotropia is associated with a high success rate. Long-term follow-up indicated that an exotropic drift may be expected 3 times more often than an esotropic drift.