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Pectus Arcuatum and Currarino-Silverman Syndrome


Pectus Arcuatum (PA) is a particular and rare type of protruding deformity that is becoming more and more identified today. Although it is sometimes considered as a subgroup of Pectus Carinatum (PC), specific anatomic characteristics make it classify as a separate entity, both for its different pathophysiology and for its specific geometrical surgical treatment.

Several synonyms refer to it: Pouter pigeon breast, type II chondro-manubrial PC, horns of steer depression, horseshoe deformity or Currarino-Silverman syndrome (1, 2, 3).



The initial description as “Curarino-Silverman syndrome” in 1958 (2), established a different cause from the excess of cartilages length generally admitted for PE and PC.

It is a premature complete fusion of the sternal pieces (sternebrae), including the manubrio-sternal junction, during the early growing phase, as a consequence of inadequate segmentation of the embryonic sternum.

The result is a very short and broad sternum in one single piece often without xiphoid process. This pathognomonic specificity is well seen on imaging and asks for a special surgical action on this flat multi-angular bone. Also noticeable are a protrusion of the parasternal cartilages, from the 2nd to the 4th bilaterally even more prominent than the sternum itself and a significant elongation of the 5th to 8th inferior cartilages.

Another consequence is the frequent association with spinal deformities: kyphosis, scoliosis.


The historical Currarino-Silverman syndrome is a PA associated with congenital heart defects in 3/10 of the initially described cases, such as patent ductus arteriosus, atrial septal defect or ventricular septal defect (2, 3). Thus, cardiovascular evaluation should be performed in all patients with PA.


Clinical example

The depression usually observed under the sternal protrusion has sometimes been considered as an additional PE also requiring correction but profile imaging generally shows that it is actually an optical illusion.

However there are some seldom cases of mixed deformity associating a superior PA and an underlying true PE: Figure 1 A.                                  

              Figure 1 A: Pectus Arcuatum with an inferior component of Pectus Excavatum

        1 B: Anatomic and cosmetic result of open surgery correction


Principles of PA correction

There is no possibility or indication for bracing or for a minimally invasive technique and the only option for correction is a Sterno-Costo-Chondroplasty (SCC) in open surgery (1, 3):
Fig 1 B.


As a PA is mainly a protruding deformity, it must be corrected like a PC along two perpendicular axes, one vertical and the other transversal, to achieve three-dimensional flattening. But important differences require special techniques (1):

  • The “one piece” sternum correction necessitates three osteotomies, including one segmental at the top of the protrusion.

  • The parasternal cartilages are resected and aligned by limited chondrectomies.

  • The angles of the lateral costal deformities are adapted by double chondrotomies or cortical costotomies.

  • An associated inferior depression should always be corrected like a true PE.

  • Systematic anterior contention bridging during 1 year is recommended:

    • Pre-sternal bar: 1 or 2

    • ± Retro-sternal bar if PE component


Jean-Marie Wihlm, M.D.


Brichon et al. Ann Thorac Surg. 2010

Currarino et al. Radiology. 1958

Gritsiuta et al. J Thorac Dis. 2021

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