Currarino-Silverman syndrome, also known as pectus carinatum type 2 deformity, is a rare disorder.
Patients present with a high carinate chest deformity due to a premature fusion of the manubriosternal joint and sternal ossification centers. Congenital heart diseases have been described in more than 50% of the patients 2,3,5.
The majority of cases are symmetric, but asymmetric and even mixed deformities (e.g. ipsilateral carinatum and contralateral excavatum) may occur. Other associated musculoskeletal abnormalities may be present, such as scoliosis and, in rare cases, Poland syndrome.
Treatment and prognosis
Surgical correction can be done by bilateral resection of the third through seventh costal cartilages and osteotomy, usually with a satisfactory result.
History and etymology
Described by the American pediatric radiologists Guido Currarino and Frederic N Silverman in 1958 2.
- 1. Desmarais TJ, Keller MS. Pectus carinatum. (2013) Current opinion in pediatrics. 25 (3): 375-81. doi:10.1097/MOP.0b013e3283604088 - Pubmed
- 2. Currarino G, Silverman FN. Premature obliteration of the sternal sutures and pigeon-breast deformity. (1958) Radiology. 70 (4): 532-40. doi:10.1148/70.4.532 - Pubmed
- 3. Chidambaram B, Mehta AV. Currarino-Silverman syndrome (pectus carinatum type 2 deformity) and mitral valve disease. (1992) Chest. 102 (3): 780-2. Pubmed
- 4. Shamberger RC, Welch KJ. Surgical correction of pectus carinatum. (1987) Journal of pediatric surgery. 22 (1): 48-53. Pubmed
- 5. Mehta AV, Chidambaram B, Suchedina AA, Garrett AR. Radiologic abnormalities of the sternum in Turner's syndrome. (1993) Chest. 104 (6): 1795-9. Pubmed