Chest Wall Deformities

Which is true for Jeune's disease?
1. Hypoplastic scapula
2. AKA asphyxiating thoracic dystrophy
3. Absent pectoralis major
4. Rib overgrowth is involved
True or false: spinal defects, such as hemivertebrae, are often associated with costal abnormalities?
1. True
2. False
What is SprengleÂ’s deformity?
1. Hypoplastic scapula
2. AKA asphyxiating thoracic dystrophy
3. Absent pectoralis major
4. Rib overgrowth is involved
What is Poland's syndrome?
1. Hypoplastic scapula
2. AKA asphyxiating thoracic dystrophy
3. Absent pectoralis major
4. Rib overgrowth is involved
In what syndrome are anomalies of the rib and sternum and particularly pectus defects seen (congenital heart disease may also be present)?
1. Sprengel's deformity
2. Poland's syndrome
3. Marfan's syndrome
4. Holt-Oram syndrome
What is the most common location of supernumerary ribs?
1. Cervical
2. Thoracic
3. Lumbar
T or F: In sternal cleft (also known as bifid sternum or congenital sternal fissure), the heart is always outside the chest?
1. True
2. False
Which are sternal clefts NOT associated with?
1. Cleft lip or palate
2. Hydrocephalus
3. CNS abnormalities
4. Down's syndrome
Which is NOT part of Pentology of Cantrell?
1. Sternal Cleft
2. Ectopia cordis
3. Bochdalek diaphragm hernia
4. Pericardial defect
T or F: Most sternal clefts involve the manubrial end, or the lower end of the sternum?
1. True
2. False
Inferior sternal clefts are usually associated with what?
1. Sprengel's deformity
2. Poland's syndrome
3. Marfan's syndrome
4. Pentolgy of Cantrell
T or F: In partial ectopia cordis, the heart is partially outside the chest?
1. True
2. False
T or F: a rib graft is usually needed when sternal cleft is repaired during the first two years of life?
1. True
2. False
True or false: in pectus excavatum, the resected cartilage specimen often shows disorderly arrangement of cartilage cells, perichonrdritis, and areas of aseptic necrosis?
1. True
2. False
What is the incidence of pectus excavatum?
1. 1 in 400 births
2. 1 in 4,000 births
3. 1 in 10,000 births
4. 1 in 50,000 births
At what age is pectus excavatum seen?
1. Adolescence
2. First 1 - 2 months of life
3. First 1 - 2 years of life
4. 4 - 5 years of age
What EKG abnormalities are seen in association with pectus defects?
1. Left axis deviation
2. Right axis deviation
3. Weinkebach type I block
4. ST elevation
True or false: cardiac catheterization in upright exercising patients with pectus excavatum may demonstrate diminished cardiac output?
1. True
2. False
True or false: stress testing demonstrates abnormal cardiovascular or respiratory parameters in about 1/5 of patients with pectus excavatum?
1. True
2. False
Exercise performance studies have demonstrated improvement in ___ after pectus repair?
1. Vital capacity
2. Functional reserve volume
3. Maximal voluntary ventilation
4. Tidal volume
What is the incidence of major recurrence after pectus repair?
1. Under 1 Percent
2. 2 - 3 Percent
3. 5 - 10 Percent
4. 10 - 20 percent
Pectus carinatum occurs approximately _____ as often as pectus excavatum?
1. One-half
2. One-third
3. One-fourth
4. One-tenth
Associated disorders such as Marfan's, scoliosis, kyphosis, and musculoskeletal defects are more or less common in carinatum vs. excavatum defects?
1. Excavatum
2. Carinatum
3. Equivalent
Which is probably a major contributor to pectus carinatum?
1. Hypertrophy of sternum
2. Hypertrophy of costal cartilages
3. Neither
4. Both
True or false: the incidence of asthma is significantly increased in carinatum versus excavatum defects. Also, loss of lung compliance, progressive emphysema, and pulmonary infection are increased in carinatum?
1. True
2. False

Chest Wall Deformities

Quiz