190 Left ventricle cardiac fibroma in patient with gorlin – goltz syndrome

Abstract

of the palms develops with conjunctival hyperemia and cracking of the lips. Perivascular hyperechogenicity in the coronary arteries snd mild mitral regurgitation was registered on the echo. K.A.B., a 4-year-old girl, her disease started with fever and enlarged cervial lymph nodes on the right measuring 5x3 cm, so she was hospitalized and ceftriaxone therapy was introduced. Diagnosis of KD was made after the development of polymorphic rash, conjunctival hyperemia, edema and erythema of the palms and soles, and dry, cracking lips. Echocardiography revealed mild mitral regurgitation and uniformly broad LCA with hyperechogenicity. I.Š., a 7-year-old girl had fever and right-sided cervical lymphadenopathy four days before admission and was treated as an outpatient with peroral cephalosporin. During hospitalization antibicrobial therapy was changed initially to ceftriaxone and azithromycin, then due to the absence of clinical and laboratory response to cefazolin and clindamycin. On the tenth day of the disease swelling of the hands and feet with conjunctival hyperemia and raspberry tongue were noted. Echocardiographically ectatic proximal part of LCA (3.9 mm) was registered and IVIG with ASA introduced. During the longterm follow-up no residual changes in the coronary arteries were recorded. Conclusion The aim of this paper is showing that in an acute febrile disease one must also think about Kawasaki disease, especially when there is absence of adequate response to antibiotic therapy. Immunoglobulin therapy has been shown to prevent the development of serious cardiovascular complications and remains the first choice in the treatment of children with KD. In case of persistent fever and resistant cases, corticosteroids and other immunomodulatory and biological therapy may be considered.