CONGENITAL MIDDLE-EAR DEAFNESS WITH ANOMALIES OF THE FACE.
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A periosteal pennant method of primary lateral canthoplasty is described, and it effectively maintains surgical correction of the axis of the palpebral fissure and the position of the lateral canthus. In using it more than 50 times, we have not had to do a secondary correction.
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Two patients with Goldenhar-Gorlin syndrome showed paralysis of one or more extraocular eye movements on neurologic examination. At autopsy, a third patient showed unilateral agenesis of trochlear and abducens nerves and corresponding brain stem nuclei. Congenital ophthalmoplegia is not infrequent in Goldenhar-Gorlin syndrome and may be due to hypoplasia or agenesis, or both, of extraocular muscles, extraocular nerves, and brain stem nuclei.
A standard method of treating syndromes with anomalies in the face does not exist. Not only are the differences between the symptoms of different syndromes very important. Also within one syndrome variations can be marked. A team of specialists supports the cranio-facial surgeon in his central role when treating these patients. Type of treatment, sequence, timing, et cetera, follow certain rules, which may be in part the same as for the treatment of 'ordinary' maxillo-mandibular disharmonies, but sometimes differ significantly from it. The most important aspects of treatment of three rather frequently seen cranio-facial syndromes are given. Incidence, symptoms, therapeutical possibilities and timing, and results are discussed as well as the necessity of this kind of surgery.
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