A disorder characterized by costocoracoid ligament shortness resulting in cosmetic deformity with limitation of the rotation movements of the shoulder.
Unknown. Described in one large Canadian kindred.
Autosomal dominant with variable expression.
Based on the clinical findings.
The scapula is fixed to the first rib by a congenitally short costocoracoid ligament. The anomaly may be uni- or bilateral. 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.
Precautions before anesthesia
Routine preoperative assessment is considered adequate.
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 infraclavicular plexus blockade should best be avoided because of altered landmarks (loss of anterior clavicle contour) or ultrasound used for guidance. Chest narrowing theoretically could result in decreased chest wall compliance, but this problem has not been reported.
None specific to this disorder.
et al: Congenital shortness of the costocoracoid ligament. Am J Med Genet 33:444–446, 1989.