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Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome is a rare autosomal recessive congenital disorder that includes childhood-onset camptodactyly, synovial hyperplasia-related arthropathy, progressive coxa vara deformity and noninflammatory pericarditis. A seven-year-old male patient had a diagnosis of CACP. He had pericardial effusion and underwent surgical tube drainage. CACP syndrome is seen very rarely, and differential diagnosis is very important. CACP is usually treated medically, but surgery may sometimes be preferred, as in our patient.

The aetiologies, clinical features and follow-up data of 62 children with pericarditis admitted to a university hospital during a 6-year period were retrospectively assessed. Uraemic pericarditis was the most frequent and infections the second most frequent cause. In this series, the proportion of children with purulent pericarditis is less than in previous reports from developing countries. Familial Mediterranean fever, neoplasias, acute rheumatic fever and post-pericardiotomy syndrome were other important causes of pericarditis.

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome is a rare autosomal recessive congenital disorder that includes childhood-onset camptodactyly, synovial hyperplasia-related arthropathy, progressive coxa vara deformity and noninflammatory pericarditis. A seven-year-old male patient had a diagnosis of CACP. He had pericardial effusion and underwent surgical tube drainage. CACP syndrome is seen very rarely, and differential diagnosis is very important. CACP is usually treated medically, but surgery may sometimes be preferred, as in our patient. [ABSTRACT FROM AUTHOR]