Congenital pseudarthrosis of the clavicle is a rare condition, most often presenting as an isolated anatomic variation.4^, 5^, 6 and 7 Unilateral cases almost universally affect the right clavicle. There can be an associated high first rib and/or cervical rib. Left-sided presentation does occur8 and is generally associated with dextrocardia and situs inversus.

The theory is that the failure of consolidation of the ossification centers is related to the high subclavian artery on the right side (or left side in dextrocardia).5 Bilateral cases do occur. There is a familial incidence.9 Associated conditions include cleidocranial dysostosis, neurofibromatosis, trisomy 21, and other rarer syndromes (Goltz, Floating Harbor, Prader Willi, chromosome 10p deletion). Histologic evaluation of the pseudarthroses indicates enchondral ossification on both sides of the resected specimens, indicative of failure of coalescence of the two primary centers of ossification.10^,11

These children generally present with a right-sided prominence of their diaphyseal clavicle. There may be a foreshortened shoulder girth. Initially, this foreshortening is usually asymptomatic. The children may present at birth (Figure 18-2). In some respects, early presentation is most fortunate because surgery is less invasive in infancy.12 The prominence often increases in size with growth. Range of motion, strength, and sensibility testing are generally normal at presentation. Later, patients may develop signs and symptoms of thoracic outlet syndrome due to the compressive effect of the pseudarthrosis on the underlying neurovascular structures in certain positions. This effect includes numbness in C8-T1-ulnar nerve distribution; loss of radial pulse in abduction and external rotation of the shoulder; and, at times, weakness of the hand and arm, especially with overhead activities.13^,14 Radiographs at any
age will reveal the obvious pseudarthrosis.15^,16 Magnetic resonance imaging or CT scan may help better define neurovascular compromise or bony anatomy.

Associated conditions such as cleidocranial dysostosis are evaluated. Generally, pseudarthrosis of the clavicle is an isolated anatomic variant. It can be post-traumatic or associated with neurofibromatosis. Either way, the treatment is the same: observation17 or surgical intervention. Attempts can be made to lessen the thoracic outlet symptoms with postural and scapular stabilization exercises, but this condition usually comes to surgery. There are reports of vascular lesions including aneurysm of the subclavian artery or vein, acute ischemia, and subclavian vein thrombosis in adulthood in association with congenital pseudarthrosis of the clavicle.18

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