We report a case of insufficiency of bilateral shoulder function associated with Noonan's syndrome. Noonan's syndrome is characterized by multiple congenital abonormalities similar to those of Turner's syndrome and by normal karyotype.
A 4-year-old male infant was presented to Nagasaki University Hospital for deformities around neck and shoulders. These deformities had been noted since birth but no treatment had been sought. His father was noticed to have similar deformities of neck and both shoulders but is not alive now.
Physical examination revealed obvious superior migration of both scapulae, and active abduction of bilateral shoulders were only 80°. And he also had webbed neck, hypertelorism, antimongolid slant, low hair line, low set ears, high arched palate and shield like chest. Though there were no signs of cardiac abnormalities. Chromosomal analysis demonstrated the normal male karyotype; Noonan syndrome was compatible. Roentgenograms revealed bilateral elevated scapulae with each shape of equilateral triangle and no omovertebral bone. Abduction of bilateral scaplae on A-P view were under 6° in active aboduction of shoulders.
We resected scaplae 1cm in width along the vertebral margin, released rhomboid muscles and lower part of trapezius muscles and corrected the webbed neck deformity in the first and second operation. Although the mobilitiy of scaplae increased, active shoulder abduction decreased to 70° after these operation. Defect of scapular abductor muscles was suspected. Then we transferred pectoralis major muscles to both scaplae. Abduction of the right scapula was improved to 28° after 2 years and abduction of right shoulder increased to 150°. Abduction of left scapula was 4° after 3 months. The obtainment of scapular abducting power and stability of scapulae may have contributed the improvement of shoulder abduction.
