Congenital Pseudoarthrosis of Clavicle

Summary

Congenital Pseudoarthrosis of Clavicle (CPC) is a rare condition in which the two ossification centers of the clavicle fail to fuse. This can result in a painless, non-tender mass on the clavicle, and in some cases, winging of the scapula.

CPC is usually diagnosed with radiographs of the clavicle, which will show a rounded sclerotic bone at the pseudoarthrosis site. Treatment for CPC is typically observation for patients with minimal symptoms and cosmetic deformity. Surgery may be necessary for patients with persistent pain or functional limitations.

Epidemiology

CPC is a rare condition, with an estimated incidence of 1 in 200,000 live births. It is more common in males than females, and the right side is more commonly affected than the left. Bilateral CPC is rare.

Etiology

The exact cause of CPC is unknown, but it is thought to be due to extrinsic compression of the clavicle by the subclavian artery. There is no clear genetic link to CPC.

Presentation

Most patients with CPC are asymptomatic. On physical exam, there may be a painless, non-tender mass on the clavicle. In some cases, there may be winging of the scapula.

Imaging

Radiographs of the clavicle are the recommended imaging study for CPC. Radiographs will show a rounded sclerotic bone at the pseudoarthrosis site.

Treatment

Treatment for CPC is typically observation for patients with minimal symptoms and cosmetic deformity. Surgery may be necessary for patients with persistent pain or functional limitations.

Nonoperative treatment

Nonoperative treatment for CPC includes observation and pain management. Patients should avoid activities that may aggravate their symptoms.

Operative treatment

Operative treatment for CPC is typically open reduction and internal fixation (ORIF) with iliac crest bone grafting. Surgery is typically performed between the ages of 3 and 6 years.

Prognosis

The prognosis for patients with CPC is generally good. Most patients are asymptomatic and do not require treatment. If surgery is necessary, successful union of the clavicle is usually obtained.