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

https://doi.org/10.4081/monaldi.2020.1576

Authors

  • Georgia-Emmanuela Dendrinou Department of Pneumonology, 401 General Military Hospital of Athens, Greece. https://orcid.org/0000-0001-8188-4829
  • Panagiotis Zagarelos Department of Endocrinology, 401 General Military Hospital of Athens, Greece.
  • Angelos Sofronas Department of Radiology, 401 General Military Hospital of Athens, Greece.
  • Stamatis Katsenos | skatsenos@yahoo.gr Department of Pneumonology, 401 General Military Hospital of Athens, Greece.

Abstract

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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Published
2020-12-10
Info
Issue
Section
Pneumology - Case Reports
Keywords:
Noonan syndrome, pulmonary lymphangiectasia
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How to Cite
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.