Congenital Chest Wall Disorders
Marcus E. Eby
Chest wall deformities can be divided into 2 main categories:
Congenital versus acquired
Pediatric congenital chest wall deformities present anytime between birth and adolescence and can subdivided into 2 main groups:
Depression or protrusion of the sternum
(eg, pectus excavatum [PE] or pectus carinatum [PC])
Aplasia or dysplasia of the chest wall
(eg, thoracic ectopia cordis, sternal clefts, Poland syndrome)
The most common chest wall deformities are congenital PE (88%) and PC (5%).1
Pectus Excavatum
BACKGROUND
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).
PE is the most common congenital chest wall deformity.
Epidemiology: It occurs in 1 in 400 births (0.25%); ˜95% of cases are Caucasian patients, boys > girls (5:1 ratio).2
ETIOLOGY
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).
Commonly proposed mechanism is an abnormal posterior tethering of the sternum to the diaphragm during development, causing the “caved-in” appearance.
Clinical note: The theory is supported by ˜1 out of 3 patients acquiring PE after repair of a posterolateral congenital diaphragmatic (Bochdalek) hernia.3
 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
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).
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.
MANAGEMENT
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)
The open surgical repair of PE involves various modifications of the original procedure described by Brown and modified by Ravitch.
A transverse thoracic incision is made in the inframammary crease.
Electrocautery is used to create cutaneous flaps, and the pectoralis muscle is elevated to expose the depressed sternum and costal cartilages.
The perichondrium is scored longitudinally, and the deformed cartilages are resected either partially or completely with preservation of the perichondrial sheaths.
The xiphoid may require division from the sternum if it is expected to protrude after correction.5
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.
The posterior aspect of the sternum is dissected free, elevated, and fractured by upward traction. The osteotomy is closed with nonabsorbable sutures.
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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