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.

Gorham-Stout Disease Management during Pregnancy / Voltolini, Luca; Pistolesi, Massimo; Comin, Camilla; Miele, Vittorio; Filippo, Pieralli; Notaristefano, Tommaso; Ugolini, Dario; Novelli, Luca; Lavorini, Federico; Rosi, Elisabetta; Scola, Marianna; Cavigli, Edoardo; Piccioli, Caterina; Bargagli, Elena. - In: AMERICAN JOURNAL OF PERINATOLOGY REPORTS. - ISSN 2157-6998. - ELETTRONICO. - 07:(2017), pp. e226-e229. [10.1055/s-0037-1615259]

Gorham-Stout Disease Management during Pregnancy

Voltolini, Luca;Comin, Camilla;Miele, Vittorio;Lavorini, Federico;
2017

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.
2017
07
e226
e229
Goal 3: Good health and well-being for people
Voltolini, Luca; Pistolesi, Massimo; Comin, Camilla; Miele, Vittorio; Filippo, Pieralli; Notaristefano, Tommaso; Ugolini, Dario; Novelli, Luca; Lavori...espandi
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1107950
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