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.