Pectus Arcuatum (Currarino-Silverman Syndrome) is a rare congenital thoracic deformity where carinatum deformity is seen on one side, while excavatum deformity may accompany on the other side. This condition is characterized by severe manubriosternal angulation, a short sternum, where the upper portion (manubrium) protrudes forward, resembling pectus carinatum, while the lower portion angles inward toward the heart, similar to pectus excavatum. Due to the forward angulation of the upper ribs (especially ribs 1-3), this deformity is sometimes referred to as the “horns of the steer.”
Pectus arcuatum is believed to result from the premature obliteration of the sternal sutures within the breastbone. These sutures are areas where separate sections of the sternum naturally fuse together during fetal development. Normally, this process allows the breastbone to transition from four separate pieces into a single, solid structure. However, in pectus arcuatum, this fusion occurs too early, leading to the characteristic abnormal curvature of the sternum.
Most patients with pectus arcuatum appear asymptomatic during childhood but may begin to experience progressively worsening shortness of breath with exertion and/or chest pain during adolescence. Initially, pectus arcuatum was thought to be strongly associated with congenital heart defects, such as ventricular septal defects (VSD) and atrial septal defects (ASD). However, this is not typically the case. The majority of patients with pectus arcuatum actually have structurally normal hearts despite the chest wall deformity.
Most patients with pectus arcuatum eventually require a modified Ravitch-type “open” procedure, often incorporating a double osteotomy (two cuts in the breastbone) to correct the deformity. Recently developed titanium plates and screws provide improved stabilization of the breastbone during surgery, leading to better long-term results. In some cases, a hybrid approach may be used, which involves removing the protruding upper ribs, partially cutting the breastbone, and placing a Nuss bar to push the lower portion of the breastbone forward.
Because pectus arcuatum is rare, the treatment plan is individualized. Your surgeon will discuss the various surgical options and recommend the most appropriate approach based on your specific anatomy and needs.