KYPHOSCOLIOSIS

KYPHOSCOLIOSIS

Kyphoscoliosis is a disorder characterized by progressive deformity of Spine consisting of lateral and posterior curvatures.

Clinical Picture

• In majority it is of idiopathic etiology.
• Deformity results in shortening of height.
• Patients can be asymptomatic.
• Mobility of chest wall is impaired, the chest wall is stiff and lung volumes are restricted.
• Hypoventilation can occur due to small tidal volumes and increased dead space ventilation.
• V/Q mismatch leads to significant hypoxia, and can progress to symptoms of Cor pulmonale.
• CXR shows significant distortion of spine and thorax.
• Mediastinal position is altered and tremendous regional variation in intensity of breath sounds can be noted on physical exam.
• PFT will show a restrictive defect. Hypoxemia is common.

Therapy

• Milwaukee brace controls moderate deformities.
• Surgical correction is attempted in some to fix spine and arrest progression of the deformity.
• Oxygen on long term may be necessary in patients with significant hypoxemia.