Primary pulmonary lymphangiectasia in Noonan syndrome: apropos of an extremely rare manifestation and a brief literature review

Submitted: August 19, 2020
Accepted: September 23, 2020
Published: December 10, 2020
Abstract Views: 1042
PDF: 619
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Noonan syndrome (NS) is a genetic multisystem disorder characterised by distinctive facial features, developmental delay, learning difficulties, short stature, congenital heart disease, renal anomalies, bleeding difficulties and lymphatic malformations. Although lymphatic dysplasias are present in 20% of patients with NS, however pulmonary lymphangiectasia has rarely been described. In this present paper, we report a 24-year-old male who was diagnosed with Noonan syndrome and primary pulmonary lymphangiectasia by using chest imaging modalities. A brief overview of the current literature is also provided laying emphasis on the clinical, pathogenetic and diagnostic aspects of this uncommon Noonan syndrome complication.

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Dendrinou, Georgia-Emmanuela, Panagiotis Zagarelos, Angelos Sofronas, and Stamatis Katsenos. 2020. “Primary Pulmonary Lymphangiectasia in Noonan Syndrome: Apropos of an Extremely Rare Manifestation and a Brief Literature Review”. Monaldi Archives for Chest Disease 90 (4). https://doi.org/10.4081/monaldi.2020.1576.