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chestwall - Chest wall disorders the lung A group of...

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Chest wall disorders & the lung: A group of conditions affecting the Inspiratory pump and potentially leading to respiratory failure. Disorders may affect all mechanical components of the Inspiratory pump. Respiratory muscles Bony rib cage The spine and its articulations. Soft tissue compromising the abdomen Lead to a mechanical disadvantage to the Inspiratory pump and an increased work of breathing. Characterized usually by a restrictive defect. Share the potential of long term hypercapnic respiratory failure
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1.Spine & articulations: Kyphoscoliosis Thoracoplasty 2.Rib cage: flail chest( esp with pulmonary contusion.) Pectus excavatum/ Pectus carinatum Ankylosing spondylitis. 3. Respiratory muscle involvement: Central Peripheral: nerve/ neuromuscular junction/ muscle. 4.Soft tissue: obesity. Etiology of chest wall disorders:
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Kyphosis may exist as an isolated entity. Osteoporosis. post-radiation. Estimated prevalence: mild deformity 1 in 1000 severe deformity 1 in 10000 in U.S Scoliosis invariably associated with Kyphosis. A. Congenital Isolated Associated syndromes: NF/ Marfan’s /EDS. B. Paralytic/ Secondary Neuromuscular: poliomyelitis/ M. dystrophy /CP/CMT syndromes. connective tissue related: EDS/ Morquio’s. vertebral disease: osteoporosis/ osteomalacia/ spina bifida. compensatory: post Thoracoplasty. C. Idiopathic .
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Often begins in early adolescence a female predominance (4 to 1) May account for as many as 80% of cases of Kyphoscoliosis The Cobb angle is associated with the degree of restriction. The relationship is however more complex. Other factors involved are: 1. Number Of Vertebrae 2. Location of curve 3. Patient’s age. 4. Presence of Kyphosis 5. Degree of rotation Idiopathic Kyphoscoliosis
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Congenital Kyphoscoliosis : Isolated or Syndromic/may be genetic present at birth Malformations of vertebrate( during ontogeny) loss of VC 15% greater than in idiopathic scoliosis . Paralytic Kyphoscoliosis : loss of muscular tone primary secondary unequal resting tone & remodeling. should be ruled before other 2 categories considered. association between scoliosis & restriction not strong. reduction of VC primarily assoc with weakness. early onset of scoliosis . faster progression.
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Pathophysiology of Kyphoscoliosis Maximum restriction among chest wall disorders. Pulmonary function testing : TLC VC N/ RV N FEV1/FVC FEV1 RV/TLC. FEV1 reduced proportional to the restriction. Specific airway conductance is actually increased( radial traction because of recoil.) Mimics obstructive disease.
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