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A very rare syndrome characterized by costocoracoid ligament shortness resulting in cosmetic deformity with limitation of the rotation movements of the shoulder.

Described in one large Canadian kindred. Autosomal dominant transmission with variable expression.

The scapula is fixed to the first rib by a congenitally short costocoracoid ligament. The rib cage may be narrow and the shoulders sloping. The contour of the anterior clavicle is lost, the shoulders rounded, and the scapular motion limited. Movements depending on rotation or retraction of the scapula are limited, but usually do not interfere with normal activities. Surgical excision of the costocoracoid ligament results in some correction of the cosmetic deformity.

No literature regarding anesthesia in these patients is available. Careful intraoperative positioning is needed, particularly in the prone position because of limited shoulder mobility. The subclavian approach for central venous line insertion or axillary plexus blockade probably should be avoided because of altered landmarks (loss of anterior clavicle contour). Chest narrowing theoretically can result in decreased chest wall compliance, but this problem has not been reported.

Bamforth JS, Bell MH, Hall JG, et al: Congenital shortness of the costocoracoid ligament. Am J Med Genet 33:444, 1989.  [PubMed: 2596500]

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