Gorham-Stout Disease Management during Pregnancy

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.

CT and MRI Findings of Focal Splenic Lesions and Ascites in Generalized Lymphatic Anomaly, Kaposiform Lymphangiomatosis, and Gorham-Stout Disease

“…To the best of our knowledge, this is the first study to evaluate the number and size of focal splenic lesions associated with LAs. Similar to the findings of the present study, patients with GLA [ 8 ] presented with relatively larger focal splenic lesions, whereas patients with KLA [ 7 , 9 ] or GSD [ 10 ] presented with relatively smaller focal splenic lesions on CT or MRI images, as reported in several case reports.…”

“…[ 6 ] Several case reports have reported multiple splenic cysts or nodules in patients with GLA, [ 8 ] KLA, [ 7 , 9 ] or GSD. [ 10 ] However, the number and size of the focal splenic lesions were mainly not revealed in these articles. In addition, no study has been reported the histopathological findings of the focal splenic lesions.…”

“…In the present study, a majority of the focal splenic lesions exhibited marked hyperintensity similar to cerebrospinal fluid on T2-weighted images, and contrast enhancement was not observed in large focal splenic lesions. Focal splenic lesions associated with LAs have been previously reported as “splenic cysts.” [ 3 , 5 , 8 , 10 ] Similarly, although we wanted to label the lesions as “splenic cysts,” we used the term “focal splenic lesions” because contrast-enhanced imaging was not performed in all the patients in the present study. In addition, contrast enhancement of small focal splenic lesions, especially those below the section thickness, could not be accurately evaluated because of the partial volume effect.…”

“…To date, several original articles [ 1 , 3 , 5 , 6 ] and case reports [ 7 - 10 ] have reported focal splenic lesions associated with LAs. Meanwhile, ascites associated with LAs has been reported in a relatively fewer original articles [ 1 , 2 ] and case reports.…”

“…Meanwhile, ascites associated with LAs has been reported in a relatively fewer original articles [ 1 , 2 ] and case reports. [ 7 , 10 ] However, to the best of our knowledge, no study has presented a detailed investigation comparing the frequency of focal splenic lesions and ascites among GLA, KLA, and GSD or evaluated the number and size of focal splenic lesions among them. Although the diagnosis of GLA, KLA, or GSD is difficult because of overlapping disease entities, the investigation of abdominal imaging findings in this study may be useful for an accurate diagnosis or a prediction of disease state.…”