ABSTRACT

Noonan syndrome is an autosomal dominant inherited disease. The most common respiratory malformations are pectum carinatum, pectum excavatum, pulmonary lymphangiectasia, and chylothorax. It has also been associated with cardiovascular malformations, such as pulmonary valve stenosis, aortic coarctation, and pulmonary branch stenosis. We present the case of a 6-year-old male, with a diagnosis of aberrant right subclavian artery, pulmonary sequestration and Noonan syndrome, sent for evaluation with a diagnosis of asthma. A review of the literature was carried out in various medical journals in search of some association or previous case. We conclude that the diagnosis is complex, since there is no association in the literature between these pathologies. Therefore, it is important to carry out an adequate medical history and physical examination, as well as adequate imaging studies in children with this syndrome.

KEYWORDS

Noonan syndrome, pulmonary sequestration, aberrant right subclavian artery, bronchopulmonary malformation.

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