Pectus Arcuatum
Pectus arcuatum (also known as Currarino-Silverman syndrome) is a rare variant of pectus excavatum, originally described in 1958. Patients typically have a very short sternum (breastbone) which points forward at the top portion (the manubrium) and then angulates backwards (toward the heart) at its bottom portion. One can think of pectus arcuatum as a type of pectus carinatum (at the top) and pectus excavatum (at the bottom). Because the upper ribs (especially ribs 1-3) angulate forward, some have termed this deformity the "horns of the steer."
Causes of Pectus Arcuatum/Currarino-Silverman Syndrome
It is believed that pectus arcuatum results from premature obliteration of the sternal sutures within the breastbone. These are the areas of bone within the breastbone that fuse together during fetal life so that the breastbone is no longer four separate pieces of bone but, rather, a single solid piece.
Symptoms of Pectus Arcuatum/Currarino-Silverman Syndrome
Most patients with pectus acuatum appear to be relatively asymptomatic until adolescence, when they develop progressively-worsening shortness of breath with exertion and/or chest pain.
When originally described, pectus arcuatum was almost always associated with underlying congenital heart disease, including various holes in the heart, such as ventricular septal defects and atrial septal defects. However, this is generally not the case. Most patients with pectus acuatum actually have structurally normal hearts.
Treatment Options for Pectus Arcuatum/Currarino-Silverman Syndrome
Unfortunately, because the sternum (breastbone) is sharply angulated back toward the heart, external compression bracing is not recommended because it generally worsens the deformity. Since it is rare, we have seen numerous patients with pectus arcuatum come to us after being incorrectly diagnosed and incorrectly treated.
Most patients with pectus arcuatum eventually require a modification of the Ravitch-type "open" procedure. We prefer a "double osteotomy" (double cutting of the breastbone) as described by Shamberger and Welch at the Boston Children's Hospital and then extensively modified by Professor Jean-Marie Wihlm in France. Recently-developed titanium plates and screws allow for much better stabilization of the breastbone during surgery with better results. These include:
- Less postoperative pain
- Much faster recovery
- Earlier discharge from the hospital
Some patients with pectus arcuatum can be treated with a "hybrid" procedure, as developed by Dr. Sherif Emil in Montreal, Canada, with removal of the protruding top ribs, partial cutting of the breastbone, and placement of a Nuss bar to push the lower portion of the breastbone forward.
Since pectus arcuatum is rare and the approach to each patient somewhat different, your surgeon will meet with you to discuss various treatment options and suggest the best approach.