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Pectus deformities

Antonio Messineo

ANTONIO MESSINEO AND MARCO GHIONZOLI

Pectus excavatum and pectus carinatum, which represent the two main anterior chest wall deformities, are often associated with systemic weakness of the connective tissues and poor muscular development of the human trunk, including chest, abdomen, and spine. Both forms have, therefore, a markedly increased association with scoliosis and connective tissue disorders such as Marfan and Ehlers-Danlos syndromes.

Pectus excavatum, as its name suggests, presents with an excavated, sunken, or funnel chest and accounts for around 84% of all deformities (see Figure 2.1). Classifications allow us to differentiate asymmetric/symmetric, localized/diffuse, and long/short defect.

Pectus carinatum, a chest wall protuberance, which constitutes approximately 13% of chest wall deformities, presents in two forms: (1) the more frequent chondrogladiolar defect (which can be symmetric or asymmetric) (see Figure 2.2), and (2) the rarer, upper defect, the chondromanubrial one (see Figure 2.3).

PECTUS EXCAVATUM REPAIR

In the 1920s, Ferdinand Sauerbrucha, pioneer in thoracic surgery, performed the first pectus repair using the bilateral costal cartilage resection and sternal osteotomy technique. He advocated the use of external traction for 5-6 weeks to hold the sternum in its corrected position and prevent recurrence. This technique was soon used by other surgeons in Europe and rapidly gained popularity in the United States as well.

Two decades later, Ravitch published his experience with eight patients in which he had used a radically extended modification of Sauerbruch’s technique. Since the sternum was cut loose from all its attachments, he hypothesized that the sternum would no longer sink back into the chest and considered the use of external traction unnecessary. Such a modified procedure, however, was accompanied by an increased recurrence rate. To overcome this problem, in the 1950s, Wallgren and Sulamaa proposed the use of a slightly curved stainless-steel bar as an internal support. In the same period, J. Alex Haller drew attention to the risk of acquired asphyxiating chondrodystrophy in very young patients who had undergone the Ravitch procedure. This report prompted many surgeons to refrain from performing open pectus repair in young children, preferring to wait until the pubertal period. Moreover, many surgeons reverted to a procedure that entailed a decreased amount of cartilage resection and a more limited skin incision, the so-called modified Ravitch procedure.

In 1998, Nuss published his revolutionary experience with a minimally invasive technique that did not require any cartilage resection or sternal osteotomy: this procedure relied on internal bracing, with a curved stainless-steel bar inserted, under thoracoscopic view, through two lateral chest incisions.

PRINCIPLES AND JUSTIFICATION

The majority of children with pectus excavatum are asymptomatic; they are referred because they experience psychological distress and have a negative body image. A small subset complains of nonspecific chest pain and shortness of breath. These patients present with a very characteristic pattern: rounded shoulders, sloped ribs, potbelly and sunken chest. The excavatum defect is often associated with scoliosis and heart displacement toward the left hemithorax.

PREOPERATIVE ASSESSMENT AND PREPARATION

Chest computed tomography scanning or thoracic magnetic resonance imaging provides an accurate assessment of anatomical situation. The ratio of the distance between the sternum and vertebral bodies and the transverse diameter of the chest through the deepest portion of the defect may be used to calculate (Haller index). In normal children, this index is less than 2.5, whereas the index may range from 3 to 7 in those with severe deformities. The asymmetry index should also be calculated to determine the severity of defect.

Simultaneous pulmonary and cardiac evaluation has shown that a severe deformity can cause compression of the right side of the heart, resulting in right-ventricular outflow distortion. The vast majority of children show normal pulmonary function at rest, while a few individuals with severe deformities may have mild restrictive patterns.

In patients with pectus excavatum, the appropriate selection of children who will benefit from defect correction still remains the main issue. Surgical indications include chest pain and/or dyspnea on exertion, cosmetic concerns, and psychological disturbance. We believe that repair should be performed in early adolescence, after pubertal growth spurt, when patients are mostly aware of their body image and can exhibit strong motivation to undergo the operation.

ANESTHESIA

Chest wall reconstruction is performed under general anesthesia with or without thoracic epidural analgesia.

RAVITCH PROCEDURE

The modified Ravitch operation involves the resection of all abnormal costal cartilages, a concept that is applicable equally to excavatum and carinatum deformities. The sternum is displaced by longitudinal cartilage overgrowth, and, therefore, once it has been freed from such deformed cartilage, a fracture of its anterior table is required to restore a normal position.

Ravitch procedure technique

INCISION

1. A transverse, rather than vertical, incision through the deepest or prominent portion of the defect is used.

SKIN AND MUSCLE FLAPS

1. Following adequate subcutaneous dissection, which starts in the midline and moves laterally, pectoralis muscle flaps are created, exposing the costochondral junction. The defect may involve many ribs, but usually only cartilages from the 5th to the 8th bilaterally are altered. A minimum of four cartilages for each side should be excised (see Figure 2.4).
   

   
   

   

SUBPERICHONDRIAL RESECTION OF THE DEFORMED CARTILAGES

1. At each involved cartilage, perichondrium is longitudinally incised, exposing the deformed cartilage (see Figure 2.5a). Caution is taken to avoid entering the pleural space. Each altered cartilage is resected from the ossified part to the sternal attachment. The perichondrium should be preserved as a template for the new cartilage growth (see Figure 2.5b).

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