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Chest Wall Anomalies And Tumors

Chest Wall Anomalies and Tumors

1. Pectus Excavatum

A. Most common congenital sternal deformity, occurring in 1 in 400 children
B. Excessive growth of lower costal cartilage results in sternal depression
C. Usually causes a deeper depression on the right, pushing heart to the left
D. Congenital with progressive worsening over time
E. Rarely familial

2. Physiologic Manifestations

A. Usually asymptomatic
B. Subjective decrease in respiratory reserve with exercise
C. Scoliosis and mitral valve prolapse have been associated with pectus excavatum
D. Decreased maximal voluntary ventilation and a mild restrictive pattern on PFTs has been documented in some studies
E. Decreased SV and CO during upright exercise has also been demonstrated

3. Operative Indications

A. Cosmetic correction is the most common reason
B. Psycho-social factors, however, may be quite limiting, particularly in older children
C. Respiratory insufficiency and recurrent pulmonary infections
D. Best results are obtained in patients between the ages of 3 and 5

4. Operative Technique

A. Ravitch repair
1) Midline or transverse inframammary incision
2) Pectoralis reflected bilaterally to expose costal cartilages
3) Subperichondrial resection of all deformed costal segments
4) Elevate sternum from underlying structures and separate from cartilage
5) Transverse sternal osteotomy and fixation with pin or cartilage support
B. Sternal eversion
1) En bloc excision of sternum and associated deformed cartilages
2) Free graft everted and fixated
3) Alternatively, the graft can be mobilized on an internal mammary artery pedicle
4) New anterior surface of the sternum shaped to form proper contour
C. Prosthetic implants
1) Silastic or other prosthetic molds generally give poor results

5. Results

A. Cosmetic results are good in 80-90%
B. Recurrence occurs in about 10-20% of patients
C. Return of normal respiratory function and improvement in exercise capacity is possible

6. Other Deformities

A. Pectus Carinatum
1) More common in males and is associated with scoliosis
2) Usually presents as anterior sternal displacement with symmetric costal cartilage concavity
3) Costal cartilage resection gives excellent results
B. Poland's syndrome
1) Unilateral absence of pectoralis major with hypoplasia or aplasia of ipsilateral breast and ribs, and bradysyndactyly
2) More common in males, usually occurs on the right side, and is most often sporadic
3) Operative repair involves rib grafts and prosthetic patching of the chest wall
C. Sternal fissure
1) Complete, upper, or distal varieties occur
2) Narrow clefts can be closed primarily after mobilization by oblique chrondotomies
3) Broader clefts may require a prosthesis to avoid compressing the heart
D. Cantrell's Pentalogy
1) Characterized by a distal cleft, omphalocele, diaphragmatic cleft, pericardial defect, and congenital heart defect (usually VSD or TOF)
2) One-stage repair is usually possible

CHEST WALL TUMORS

1. Incidence

A. Comprise 7-8% of all bony tumors
B. Most primary chest wall tumors are malignant
C. 85-90% occur in the ribs (50% malignant)
D. 10-15% occur in the sternum (95% malignant)
E. Male:female = 2:1

2. Clinical Presentation

A. Slowly enlarging mass eventually causes pain and presence of mass
B. Pain is more common in malignant tumors, but 20-25% are asymptomatic
C. Tumors occur at any age and are more likely to be malignant in older patients
D. CXR with rib detail films and CT scan are usually adequate and can evaluate associated pulmonary nodules
E. MRI distinguishes nerve and vascular invasion

3. Etiology

MalignantBenign
ChondrosarcomaFibrous dysplasia (40%)
MyelomaChondroma (30%)
Osteogenic sarcomaOsteochondroma
Ewing's sarcomaDesmoid

4. Principles of Treatment

A. Excisional rather than incisional biopsy should be peformed if a primary chest wall tumor is suspected
B. Full thickness excision of the tumor with 1 rib margin is necessary; do not compromise resection to avoid large chest wall defect
C. Large tumors may warrant incisional biopsy
D. Needle biopsy is best for suspicious mets or myeloma
E. Sternal tumors should be treated by sternectomy

5. Principles of reconstruction

A. A defect less than 5 cm does not require reconstruction
B. Posterior defects do not require reconstruction due to scapula
C. Defects larger than 5 cm will require reconstruction
D. Skeletal stabilization can be accomplished with a mesh patch or methyl methacrylate
E. Soft tissue reconstruction can be done in a variety of ways, including myocutaneous flaps (latissimus dorsi, pectoralis major, rectus abdominus) and omental transposition

6. Results

A. Low operative mortality and good postoperative pulmonary function
B. Overall long term survival is about 50-70%, with best rates for chondrosarcoma and rhabdomyosarcoma, and worst rates for malignant fibrous histiocytoma
C. Survival is better with wide excision
D. Adjunctive therapy may improve survival