Article
Results of surgery for congenital Jaensch-Brown syndrome
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Published: | September 22, 2004 |
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Outline
Text
Objective
Reports on the effects of surgery for congenital Jaensch-Brown-syndrome are not unequivocal. They are restricted to relatively small case series. We have evaluated the effects of a superior oblique tendon recession in a group of 23 patients with a congenital Jaensch-Brown-syndrome.
Methods
The patients received surgery from 1985 to 2002 in our department. The goal of surgery was to improve the monocular motility, the gaze area of binocular fusion, and the abnormal head posture. The squint angles were measured in degrees by the alternate prism and cover test, the monocular motility by pursuit movement. The abnormal head posture was measured with distance fixation. The measurements were performed 1 day before, 3 months after and more than 2 years after surgery.
Results
At surgery, the patients were 4-17 years (median 7 years). Thirteen patients were male, in 15 patients, the right eye was affected. Preoperatively, the vertical deviation in straight gaze was 0-12° (median 7°). The elevation of the affected eye was restricted to -10 (below horizontal) to 15° (median 0°) in adduction and 10-35° (median 25°) in abduction. Seven patients did not show any abnormal head posture, 9 patients had an elevation of 5-20°, 3 patients a depression of 10-30°, and 4 a head-tilt of 10-15° to the affected side. Regularly, the superior oblique tendon was recessed by 10 mm, in some cases with an additional polyester loop. Three months postoperatively, the vertical deviation was reduced to 0-6° (median 1°) and the monocular elevation in adduction was improved to -5 bis 15° (median 5°). Twelve patients did not show any abnormal head posture, 7 had an elevation of 5-10°, two a depression of 5°, and 1 patient tilted her head by 15°. The evaluation of the long term results was not yet finished at the time of abstract submission. Half of the patients already examined showed further significant improvement of motility.
Conclusions
The effects of surgery for congenital Jaensch-Brown-syndrome differ individually. In spite of free passive motility after the recession of the superior oblique tendon, there is usually a restriction of the elevation in adduction. The abnormal head posture can usually be improved.