Congenital Chest Wall Disorders
Marcus E. Eby
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Chest wall deformities can be divided into 2 main categories:
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Congenital versus acquired
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Pediatric congenital chest wall deformities present anytime between birth and adolescence and can subdivided into 2 main groups:
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Depression or protrusion of the sternum
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(eg, pectus excavatum [PE] or pectus carinatum [PC])
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Aplasia or dysplasia of the chest wall
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(eg, thoracic ectopia cordis, sternal clefts, Poland syndrome)
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The most common chest wall deformities are congenital PE (88%) and PC (5%).1
Pectus Excavatum
BACKGROUND
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PE is a congenital deformity of the anterior thoracic wall in which the sternum and rib cage grow abnormally to produce a “caved-in” appearance of the chest (also known as funnel chest) (Figure 34.1).
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PE is the most common congenital chest wall deformity.
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Epidemiology: It occurs in 1 in 400 births (0.25%); ˜95% of cases are Caucasian patients, boys > girls (5:1 ratio).2
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ETIOLOGY
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The exact cause of PE development has yet to be established although studies have confirmed a strong genetic predisposition to have PE (˜40% of PE cases have a family member with an associated chest wall deformity).
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Commonly proposed mechanism is an abnormal posterior tethering of the sternum to the diaphragm during development, causing the “caved-in” appearance.
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Clinical note: The theory is supported by ˜1 out of 3 patients acquiring PE after repair of a posterolateral congenital diaphragmatic (Bochdalek) hernia.3
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Figure 34.1 Pectus excavatum. Photograph of a 4.5-year-old girl with a symmetric pectus excavatum deformity. Note that the depression extends to the sternal notch. (Reprinted with permission from Shamberger RC. Chest wall deformities. In: Shields TW, ed. General Thoracic Surgery. 4th ed. Baltimore, MD: Williams & Wilkins; 1994:529-557.) |
SYMPTOMS
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Most common complaints include exercise intolerance, lack of endurance, and shortness of breath on exertion (usually asymptomatic during childhood; may become symptomatic during more active teenage years).
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Suspected pathophysiology of easy fatigability: Decreased chest wall motion during respirations at the area of the pectus defect results in less efficient mechanism of breathing during strenuous exercise.
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MANAGEMENT
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Surgical correction of PE is offered to symptomatic patients who are interested in attempting to improve exercise tolerance. This can be performed either as a minimally invasive or open approach depending on surgeon preference.1
OPEN REPAIR OF PECTUS EXCAVATUM: (RAVITCH PROCEDURE)
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The open surgical repair of PE involves various modifications of the original procedure described by Brown and modified by Ravitch.
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A transverse thoracic incision is made in the inframammary crease.
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Electrocautery is used to create cutaneous flaps, and the pectoralis muscle is elevated to expose the depressed sternum and costal cartilages.
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The perichondrium is scored longitudinally, and the deformed cartilages are resected either partially or completely with preservation of the perichondrial sheaths.
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The xiphoid may require division from the sternum if it is expected to protrude after correction.5
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An anterior table, wedge-shaped, sternal osteotomy is performed at the cephalad transition from the normal to the depressed sternum near the level of the insertion of the second or third costal cartilages.
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The posterior aspect of the sternum is dissected free, elevated, and fractured by upward traction. The osteotomy is closed with nonabsorbable sutures.
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A drain is positioned below the muscle flaps. The muscle flaps are sutured back into position, and the incisions are closed.5,6
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