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Surgical Results in Congenital Jaensch-Brown-Syndrome

Schmitz N. G., Schworm H. D.,
Universitätsklinikum Hamburg-Eppendorf, Klinik und Poliklinik für Augenheilkunde, Bereich Orthoptik und Pleoptik (Hamburg)

Background: Essential feature of the congenital Jaensch-Brown-Syndrome is a uni- or bilateral restriction of active and passive upgaze in adduction. The outcome of surgical treatment is usually unsatisfactory. Mühlendyck recently described a stiff cord at the posterior part of the insertion of the superior oblique muscle as the underlying cause and recommended its resection. This new technique is compared to the common ways of surgery.
Methods: The pre- and postoperative results of 13 patients at a mean age of 6.2 years (range, 3 - 9 years) were compared retrospectively with regards to active and passive motility. A resection of the posterior cord had been carried out in 2 patients. A total recession of the superior oblique by 8 - 9 mm had been performed in 10 patients, one patient underwent a Z -plasty of the tendon.
Results: All patients showed unrestricted passive motility postoperatively. In contrast, improvement of active motility was less pronounced. In 4 patients who underwent the common way of surgery, a further recession was necessary. Among the 2 patients with resection of the posterior cord, one patient with unilateral disease showed an improvement of active motility leading to a vertical increase of nearly 20° of the field of binocular single vision. The second patient presenting with bilateral disease revealed a major reduction of chin elevation postoperatively, long term results are yet to be investigated.
Conclusions: Our study confirms the limited improvement of active motility despite unrestricted passive motility after surgery. Resection of the posterior cord as recommended by Mühlendyck compared to total recession of the superior oblique muscle showed at least an equal improvement of active upgaze without the risk of cycloductional interference. The cause for the discrepancy between the unrestricted passive motility and the limited improvement of active motility is not yet clearly understood.