Pectus excavatum from a pediatric surgeon's perspective

Abstract

Historically, pectus excavatum (PE) was reported to be congenital, but in our experience only 22% are noticed in the first decade of life. Thus far, genetic studies support an autosomal recessive heritability, which coincides with only 40% of our patients having some positive family history, but is also contradictory given a constant sex ratio of 4:1 in favor of males. This inconsistency may be explained by the effect of more than one pectus disease-associated allele. Once the deformity is noticed, it tends to progress slowly until puberty, when rapid progression is often seen. We recommend surgical repair at around 12-14 years of age since the chest wall is still typically flexible and because this allows us to keep the bar in place as the patient progresses through puberty which may help decrease growth-related recurrences. Patients with mild to moderate PE are treated with therapeutic deep breathing, posturing, and aerobic exercises, and in appropriately selected patients, the vacuum bell may also be offered. Patients that have severe symptomatic PE are offered Minimally Invasive Repair of Pectus Excavatum (MIRPE). The surgical technique in children is similar to that of adults, except for the higher forces involved that often necessitate sternal elevation and more involved stabilization strategies. Postoperative management includes pain control, deep breathing, and early ambulation. Exercise restriction is mandatory for the first six weeks with slow resumption of normal activity after 12 weeks.

Keywords: Pediatric pectus excavatum; conservative management; genetics; minimally invasive repair; post-operative management.

Figure 1

Eighteen month old patient with severe congenital pectus excavatum. Twenty two percent of our pectus excavatum patients are less than 10 years old.

Figure 2

Pectus excavatum is a progressive deformity. This patient progressed from mild to severe & asymmetric before puberty. At the time of puberty, the chest becomes more rigid and patients start to exhibit cardiopulmonary symptoms.

Figure 3

A congenital pectus excavatum patient who had both cardiac and pulmonary compression and fulfilled the criteria for pectus repair. He was therefore repaired at the age of 5 years.

Figure 4

This is the same patient, now 15 years old and 10 years post pectus repair and 7 years post bar removal. Our philosophy is that the best age for repair is between 11 and 15 years, but we will repair whenever the patient fulfills the criteria for surgery.

Figure 5

Graph showing the effects of different durations of bar placement.

Figure 6

Shows the effect of age on long term outcome.