CC-BY-NC-ND 4.0 · AJP Rep 2017; 07(04): e226-e229
DOI: 10.1055/s-0037-1615259
Case Report
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Gorham-Stout Disease Management during Pregnancy

Elena Bargagli1, Caterina Piccioli1, Edoardo Cavigli2, Marianna Scola1, Elisabetta Rosi1, Federico Lavorini1, Luca Novelli3, Dario Ugolini4, Tommaso Notaristefano4, Pieralli Filippo5, Vittorio Miele2, Camilla E. Comin3, Massimo Pistolesi1, Luca Voltolini4
  • 1Department of Clinical and Experimental Biomedical Sciences, Careggi University Hospital, Florence, Italy
  • 2Department of Radiodiagnostic and Emergency, Careggi University Hospital, Florence, Italy
  • 3Department of Pathology, Careggi University Hospital, Florence, Italy
  • 4Thoracic Surgery Unit, Careggi University Hospital, Florence, Italy
  • 5Subintentive Medicine Section, Careggi University Hospital, Florence, Italy
Further Information

Publication History

05 March 2017

12 September 2017

Publication Date:
19 December 2017 (online)

Abstract

Gorham-Stout Disease (GSD) is a rare lymphatic disorder affecting children or young adults with no predilection of sex. It is generally associated with vanishing bone osteolytic lesions, thoracic and abdominal involvement, and diffuse pulmonary lymphangiomatosis. Chylous effusions and chylothorax, consequent to the abnormal proliferation of lymphatic vessels, may induce respiratory failure with a high mortality risk. Extrapulmonary alterations may include chylous ascites, lymphopenia, and destructing bone disease for overgrowth of lymphatic vessels. Here, we report the case of a young woman who developed a severe and recalcitrant GSD with persistent unilateral chylothorax during pregnancy. The complex management of this patient during and after pregnancy was discussed and compared with literature data to contribute to the definition of a correct diagnostic and therapeutic approach to this rare lymphatic disease.