[Abnormal eyelid positions in Brown syndrome]

To describe the possible abnormal upper eyelid positions in congenital Brown syndrome (BS) and discuss physiopathology.

A typical symptom of BS, the incapacity to raise the eye in adduction, is usually accompanied by a retraction of the upper eyelid. However, as the abnormal position of the eye often refers to a patient looking straight ahead into the distance, abnormal eyelid positions are possible but are on the whole unknown.

The study investigated 82 patients with unilateral congenital BS, who consulted between 1989 and 2001. The primary position of the upper eyelid concerned was carefully measured, each side separately. The patients were then classified into three groups according to the type of difficulty encountered when raising the upper eyelid: group 1--those with a raising deficiency in adduction (42 cases), group 2--those with a raising deficiency in adduction and in primary position (26 cases), group 3--those with a global raising deficiency with predominance in adduction (14 cases). Fifty patients were operated on for strabismus, after which the position of the upper eyelid was remeasured.

An abnormal position of the upper eyelid was noted in 23 patients (28%) and the distribution between the different groups was as follows: group 1, 14%; group 2, 30%; group 3, 63%. The following malpositions were found: pseudoptosis with hypotropia, eight cases; ptosis, six cases; pseudoretraction with vertical oculomotor disorder, five cases; real upper eyelid retraction, four cases. Ptosis and pseudoptosis were observed more often when BS involved the nondominant eye; retraction or pseudoretraction was observed more often when BS involved the dominant eye. After surgery for vertical strabismus, the abnormal position of the upper eyelid had remarkably decreased and only three cases required specific surgery for ptosis.

The abnormal position of the upper eyelid in primary position was found in nearly one-third (28%) of the patients with BS, therefore a quite frequent occurrence. It sometimes involved associated congenital ptosis. However, the most frequent symptom was a deficiency of the eyelid that was caused either by the patient being incapable of preventing the eyelid from falling (ptosis and pseudoptosis) or the patient making a compensatory effort to raise the eyelid (retraction and pseudoretraction).