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
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