The Turkish Journal of Pediatrics
2014 , Vol 56 , Num 6
Camptodactyly-Arthropathy-Coxa Vara-Pericarditis (CACP) Syndrome
1Department of Cardiovascular Surgery, Divisions of 2Pediatric Cardiology, and 3Pediatric Intensive Care, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey. E-mail: zeyileten@gmail.com
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.
Keywords :
pericarditis, familial arthropathy, camptodactyly