Chest Wall Deformities

Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal defects 5. Miscellaneous

Congenital Heart Disease Associated with Pectus Excavatum and Pectus Carinatum Aortic ring AR ASD VSD TOF PDA TGA Ebstein’s malformation TAPVR

Pectus Excavatum( Funnel Chest ) Pectus excavatum is posterior depression of the sternum and costal cartilage due to over grow of costal cartilage. The 1st and 2nd ribs, manubrium are in normal position. Asymmetrical of the depression may be present and the right is often more depressed than the left.

Pectus Excavatum Pectus excavatum is present at birth or within the 1st year of life in most patients. It may worsen at adolescent. It may be associated with scoliosis, asthma.

Etiology and Incidence of Pectus Excavatum It is reported 1/( 300 to 400) of lives birth and rare in blacks. M:F=4:1 Etiology is unknown.

M-S Abnormalities with Pectus Excavatum Scoliosis Kyphosis Myopathy Marfan’s syndrome Cerebral palsy Prune-belly syndrome Tuberous sclerosis

Symptoms of Pectus Excavatum It is well tolerated in infants and children. Older child may have precordial pain after exercise Palpitation may be present due to atrial arrythmia.

Pathophysiology of Pectus Excavatum Systolic murmur results from close proximity of the sternum and the pulmonary artery. EKG abnormality results from displacement of and rotation of the heart into the left thoracic cavity.

Pulmonary Function Study of Pectus Excavatum The maximum voluntary ventilation is lower than normal. Some studies shows deterioration in pulmonary function after surgery due to increased rigidity of chest wall. Exercise tolerance is improved after surgery.

Cardiovascular Study of Pectus Excavatum Anterior identation of right ventricle is present. Elevated right heart pressure is present. In pectus excavatum, increased cardiac output on exertion is due to increased heart rate rather than stroke volume. Echo reveals mitral valve prolapse, which can subside after surgery.

Sugical Repair of Pectus Excavatum

Complications of Surgery of Pectus Excavatum Pneumothorax Wound infection Wound hematoma Pneumonia Seroma Recurrence Hemopericardium Hemoptysis Wound dehescence

Complications of Surgery of Pectus Excavatum The most distressing complication is major recurrence. Major recurrence is present frequently with poor muscular and asthenic or marfanoid habitus.

Complications of Surgery of Pectus Excavatum Chest growth impairment may be present if costal cartilage is resected too much. Delaying the surgery until the child is older and preserving costochondral junction with a segemnt on the rib can avoid delayed thoracic growth.

Pectus Carinatum ( Pigeon Chest ) It refers anterior protrusion of the sternum. It is less common than pectus excavatum.

Categories of Pectus Carinatum 1. Chondrogladiolar (1) It is most common pectus carinatum . (2) It consists of anterior protrusion of the body of sternum and lower costal cartilages.

Categories of Pectus Carinatum 2. Mixed with excavatum and carinatum It consists carinatum on one side and excavatum on another side.

Categories of Pectus Carinatum 3. Chondromanubrial (1) It is the most uncommon pectus carinatum. (2) It consists protrusion of manubrium, 2nd and 3rd costal cartilages with relative depression of the body and sternum.

Etiology of Pectus Carinatum The etiology is unknown and genetic basis is favored. It is more common in boys than girls. It may be associated with M-S abnormality and congenital heart disease.

Surgical Repair of Pectus Carinatum

Poland’s Syndrome It refers congenital absence of the pectoralis major and minor muscles, ribs, breast abnormality, chest wall depression and syndactyly( or brachydactyly, ectromelia ). It is present in 1/30000. The etiology is unknown.

Surgical Repair of Poland’s Syndrome

Sternal Defects 1. Cleft sternum 2. Ectopia cordis

Thoracic Deformities in Diffuse Skeletal Disorders 1. Asphyxiating thoracic dystrophy( Jeune’s syndrome ) 2. Spondylothoracic dysplasia( Jarcho-Levin syndrome)