Lymphatic abnormalities in Noonan's syndrome

Abstract

Five boys who had Noonan's syndrome and lymphatic abnormalities are reported. The youngest boy had clinical lymphoedema and the other four showed dermal backflow after interdigital injection of Patent Blue indicating impairment of flow along the superficial lymphatics. One boy had severe bilateral chylothorax. The lymphographic findings in four of these boys are reported. Patients with the Noonan syndrome frequently have oedema of the hands and feet at birth, which decreases during the first years of life [10]. It has been demonstrated by lymphography that similar peripheral oedema in patients with the Turner's syndrome is due to lymphatic hypoplasia [1, 3]. We report certain lymphatic abnormalities diagnosed by lymphography in four out of five patients with Noonan's syndrome.