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Mark V. Mazziotti, MD Texas Children's Hospital

Chest Wall Deformities

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Outline 1

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Pectus Excavatum

Pectus Carinatum

Poland Syndrome

Sternal Defects

Thoracic Deformities in Diffuse Skeletal Deformities

Asphyxiating Thoracic Dystrophy: Jeune Syndrome

Spondylothoracic Dysplasia: Jarcho-Levin Syndrome

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Pectus Excavatum • Most common anterior chest wall deformity •  Involves posterior depression of the sternum and

lower costal cartilages • Boys: Girls 3:1 •  38/10,000 births (Caucasians) • Noted in infancy or around puberty • Tall and lanky with “poor posture” • Cause: Unknown

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Pectus Excavatum •  Despite 6 decades of research there is no consensus on

what degree of cardiopulmonary impairment this deformity produces

•  Pulmonary Function Evaluation –  Decreased muscular ability and patient effort –  Increased mean oxygen uptake with maximal effort

•  Cardiovascular Function –  Anterior indentation of the right ventricle –  Difficulty increasing stroke volume in exercise –  Mitral Valve Prolapse

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Pectus Excavatum • Body Image –  Validated psychomotor assessments administered

to patients < 21 year of age before and after operation show marked improvement in psychosocial functioning

• Evaluation –  Pectus CT scan –  Pulmonary Function Testing –  Echocardiography

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Pectus Excavatum

1) Progressive or symptomatic pectus excavatum 2)  Restrictive disease, decreased work production, or

decreased oxygen uptake on pulmonary function testing

3)  CT scan with Haller index of >3.25 4)  Cardiac abnormalities including mitral valve

prolapse or bundle branch block 5)  Recurrent pectus excavatum after failed repair

Indications for Surgery  

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Pectus Excavatum

Surgical Treatment • Ravitch Repair –  Open Repair

• Nuss Procedure –  Minimally-invasive Repair

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Pectus Excavatum

Postoperative Management • Pain management: Epidural vs. PCA • Mean 6 day stay in the hospital with 4 weeks

recovery at home • May participate in all activities with bar in place Timing of Bar Removal

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Pectus Carinatum

•  Protrusion of the body of the sternum and lower costal cartilages

•  Less frequent than pectus excavatum by 1:5, unless you live in Argentina

•  Cause is unknown

•  Family history in 26%

•  Scoliosis in 15%

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Pectus Carinatum: Treatment

• Bracing

• Surgical technique – Ravitch procedure – Minimally-invasive

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Chest Wall Deformities •  Mostly pectus excavatum and pectus carinatum

•  Pectus excavatum patients may suffer from exercised-induced shortness of breath and chest pain

•  Pectus excavatum and pectus carinatum patients both may have decreased psychosocial function related to their body image

•  Correction of pectus excavatum is primarily operative whereas correction of pectus carinatum is primarily nonoperative