SIR 2024
Embolization
David Lim, MBBS
Consultant
Changi General Hospital, Singapore
Financial relationships: Full list of relationships is listed on the CME information page.
Saebeom Hur, MD, PhD (he/him/his)
Professor
Seoul National University Hospital, Republic of Korea
Financial relationships: Full list of relationships is listed on the CME information page.
Do Hoon Kim, MD
Fellow
Seoul National University Hospital, Republic of Korea
Disclosure information not submitted.
Hee Eun Moon, MD
Resident
Seoul National University Hospital, Republic of Korea
Disclosure information not submitted.
Seunghyun Lee, MD
Assistant Professor
Seoul National University Hospital, Republic of Korea
Disclosure information not submitted.
Gorham-Stout disease (GSD) is an extremely rare complex lymphatic anomaly, characterised by intraosseous lymphatic malformation with progressive osteolysis {1}. Chylothorax can occur when the lymphatic malformation involves the thoracic cage and vertebrae. If conservative management is unsuccessful, thoracic duct ligation or embolization can be performed {1}. A recent study proposed an algorithmic approach towards treatment of non-traumatic chylothorax, suggesting that in some scenarios, either thoracic duct embolization (TDE) alone is insufficient, or should not be performed {2}. The aim of this study is to evaluate the safety and efficacy of percutaneous lymphatic embolization performed for GSD patients, in regards to TDE and embolization of pleural or lymphatic collaterals.
Materials and methods:
Retrospective single institution study was performed. Five consecutive GSD patients (all male) with chylothorax (n=3) and haemo-lymphothorax (n=2) underwent lymphatic intervention between January 2013 and December 2022. Dynamic contrast-enhanced MR lymphangiography was performed in four patients prior to intervention. TDE was performed via transabdominal access of the cisterna chyli, thoracic duct or retroperitoneal lymphatic vessel. Embolization of parietal pleural lymphatics or collaterals were performed either from the transabdominal thoracic duct access, or direct percutaneous puncture of the dilated lymphatic structures. The lymphatic structures embolized were the thoracic duct (n=4), parietal pleural lymphatics (n=4) and other lymphatic collaterals (n=3). Embolic agents used were n-butyl-cyanoacrylate (NBCA) glue and coils (n=3) and NBCA glue only (n=2).
Results:
Key imaging findings of giant thoracic duct (n=3) and dilated parietal pleural lymphatics (n=5) were identified. Thoracic duct embolization (TDE) alone achieved clinical success in only 25% of cases (1 out of 4). With additional embolization of parietal pleural lymphatics and other collaterals, clinical success was achieved in 80% of cases (4 out of 5). One patient developed chylous ascites after TDE. The mean follow-up duration was 31 months (range, 4-82 months). One patient passed away 20 months after treatment. The other patients were all in stable condition during follow-up review.
Conclusion: Percutaneous lymphatic embolization of the thoracic duct and/or pleural lymphatic collaterals is a possible treatment option for GSD related chylothorax, while TDE alone may not be effective.