Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum...

25
Chest Wall Deformities

Transcript of Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum...

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

Chest Wall Deformities

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

Categories of Congenital Anterior Chest Wall Deformities

• 1. Pectus excavatum

• 2. Pectus carinatum

• 3. Poland’s syndrome

• 4. Sternal defects

• 5. Miscellaneous

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

Congenital Heart Disease Associated with Pectus Excavatum and Pectus Carinatum

• Aortic ring

• AR

• ASD

• VSD

• TOF

• PDA

• TGA

• Ebstein’s malformation

• TAPVR

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

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.

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

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.

Page 6: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

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.

Page 7: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

M-S Abnormalities with Pectus Excavatum

• Scoliosis

• Kyphosis

• Myopathy

• Marfan’s syndrome

• Cerebral palsy

• Prune-belly syndrome

• Tuberous sclerosis

Page 8: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

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.

Page 9: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

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.

Page 10: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

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.

Page 11: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

Cardiovascular Study of Pectus Excavatum

• Anterior identation of right ventricle is present.

• Elevated right heart pressure is present.• In pectus excavatum, increased cardiac outp

ut on exertion is due to increased heart rate rather than stroke volume.

• Echo reveals mitral valve prolapse, which can subside after surgery.

Page 12: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

Sugical Repair of Pectus Excavatum

Page 13: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

Complications of Surgery of Pectus Excavatum

• Pneumothorax

• Wound infection

• Wound hematoma

• Pneumonia

• Seroma

• Recurrence

• Hemopericardium

• Hemoptysis

• Wound dehescence

Page 14: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

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.

Page 15: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

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.

Page 16: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

Pectus Carinatum( Pigeon Chest )

• It refers anterior protrusion of the sternum.

• It is less common than pectus excavatum.

Page 17: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

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.

Page 18: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

Categories of Pectus Carinatum

• 2. Mixed with excavatum and carinatum

It consists carinatum on one side and

excavatum on another side.

Page 19: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

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.

Page 20: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

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.

Page 21: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

Surgical Repair of Pectus Carinatum

Page 22: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

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.

Page 23: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

Surgical Repair of Poland’s Syndrome

Page 24: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

Sternal Defects

• 1. Cleft sternum

• 2. Ectopia cordis

Page 25: Chest Wall Deformities Categories of Congenital Anterior Chest Wall Deformities 1. Pectus excavatum 2. Pectus carinatum 3. Poland’s syndrome 4. Sternal.

Thoracic Deformities in Diffuse Skeletal Disorders

1. Asphyxiating thoracic dystrophy( Jeune’s

syndrome )

2. Spondylothoracic dysplasia( Jarcho-Levin

syndrome)