AI Article Synopsis
- Gorham-Stout Disease (GSD) is a rare lymphatic disorder affecting mainly children and young adults, characterized by bone lesions and abnormal lymphatic vessel growth.
- The condition can lead to serious complications like respiratory failure due to fluid accumulation in the chest (chylothorax) and poses a high mortality risk.
- The case report discusses a young woman's severe GSD during pregnancy and the complex management strategies used, aiming to enhance understanding and treatment of this rare disease.
Article 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.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5736391 | PMC |
| http://dx.doi.org/10.1055/s-0037-1615259 | DOI Listing |
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