Clinical Manifestations

It is estimated that 80 percent of people with Paget's disease are asymptomatic and have no clinical findings suggestive of the disease. When symptomatic, the clinical manifestations are often defined by the complications of the disease. Clinical findings may include some or all of the following: frontal bossing, scalp vein dilatation, angioid streaks, simian pos-

Figure VIII. 100.1. Severe Paget's disease of the bone in a 66-year-old male. Note the anthropoid appearance and simian posturing, with the anterior and lateral bow to the arms, thighs, and legs. There are functional flexion contractures of the hips and knees.

ture, short stature, flexion contractures at hips and knees, anterior and lateral bowing of long bones, warmth of involved extremity, periosteal tenderness, and compressive neuropathy. Figure VIII. 100.1 shows a man with severe Paget's disease of bone.

Pagetic bone pain is uncommon, but when present it is aching, deep, poorly described, and occasionally continuous at night. It is often aggravated by pressure or weight bearing.

The following deformities may develop: (1) The involved skull may become soft, thickened, and enlarged; (2) the femora tend to migrate medially and superiorly deforming the softened pelvis; (3) enlargement of vertebrae alters the spine, resulting in an anthropoid appearance; (4) the affected long bones soften and bow from weight-bearing or from the force of the surrounding muscles.

Pathological fractures can also occur in the form of compression fractures of vertebrae, incomplete or stress fractures of the long bones, or completed fractures of long bones.

The relation of secondary osteoarthritis to Paget's disease has recently been emphasized. Low back pain is the most common clinical presentation of Paget's disease and is often related to secondary osteoarthritis.

Sarcomatous degeneration, although uncommon, does occur in approximately 1 percent of patients with Paget's disease, representing a 40-fold increase over that of the general adult population. It is most often heralded by increasing pain.

Skull symptoms may include headaches, vertigo, rushing sensations, and noise in the head. Maxillary exceeds mandibular involvement by a 2:1 ratio, and both may disrupt the lamina dura or cause unsightly deformity. Hearing loss may be severe but is most often moderate.

Neural compression may occur from spinal stenosis or a spinal lateral recess syndrome. Paraparesis -paraplegia in Paget's disease - is most often related to Paget's disease of dorsal or cervical vertebrae.

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