CC-BY-NC-ND 4.0 · AJP Rep 2017; 07(04): e226-e229
DOI: 10.1055/s-0037-1615259
Case Report
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Gorham-Stout Disease Management during Pregnancy

Elena Bargagli
Department of Clinical and Experimental Biomedical Sciences, Careggi University Hospital, Florence, Italy
,
Caterina Piccioli
Department of Clinical and Experimental Biomedical Sciences, Careggi University Hospital, Florence, Italy
,
Edoardo Cavigli
Department of Radiodiagnostic and Emergency, Careggi University Hospital, Florence, Italy
,
Marianna Scola
Department of Clinical and Experimental Biomedical Sciences, Careggi University Hospital, Florence, Italy
,
Elisabetta Rosi
Department of Clinical and Experimental Biomedical Sciences, Careggi University Hospital, Florence, Italy
,
Federico Lavorini
Department of Clinical and Experimental Biomedical Sciences, Careggi University Hospital, Florence, Italy
,
Luca Novelli
Department of Pathology, Careggi University Hospital, Florence, Italy
,
Dario Ugolini
Thoracic Surgery Unit, Careggi University Hospital, Florence, Italy
,
Tommaso Notaristefano
Thoracic Surgery Unit, Careggi University Hospital, Florence, Italy
,
Pieralli Filippo
Subintentive Medicine Section, Careggi University Hospital, Florence, Italy
,
Vittorio Miele
Department of Radiodiagnostic and Emergency, Careggi University Hospital, Florence, Italy
,
Camilla E. Comin
Department of Pathology, Careggi University Hospital, Florence, Italy
,
Massimo Pistolesi
Department of Clinical and Experimental Biomedical Sciences, Careggi University Hospital, Florence, Italy
,
Luca Voltolini
Thoracic Surgery Unit, Careggi University Hospital, Florence, Italy
› Author Affiliations
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.