Hybrid Repair of a Thoraco-abdominal Aortic Aneurysm Associated with Loeys-Dietz Syndrome
Introduction: Loeys-Dietz syndrome (LDS) is a genetic syndrome caused by mutations in transforming growth factor beta receptors (TGFBR) 1 and 2. It can manifest with craniofacial, musculoskeletal, cognitive abnormalities, and vascular pathologies including early onset aortic root aneurysms, extensiv...
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| Format: | Article |
| Language: | English |
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Elsevier
2021-01-01
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| Series: | EJVES Vascular Forum |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2666688X21000174 |
| _version_ | 1819099923095748608 |
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| author | Ahmed A. Naiem Robert J. Doonan Oren K. Steinmetz |
| author_facet | Ahmed A. Naiem Robert J. Doonan Oren K. Steinmetz |
| author_sort | Ahmed A. Naiem |
| collection | DOAJ |
| description | Introduction: Loeys-Dietz syndrome (LDS) is a genetic syndrome caused by mutations in transforming growth factor beta receptors (TGFBR) 1 and 2. It can manifest with craniofacial, musculoskeletal, cognitive abnormalities, and vascular pathologies including early onset aortic root aneurysms, extensive aortic dissections, and TAAA. Open repair is considered the gold standard treatment but carries morbidity risks, especially in patients with multiple previous aortic procedures. Endovascular treatment is associated with treatment failure when used in the native aorta, because of inherent wall weakness precluding seal. This case report adds to the available literature on hybrid treatment of LDS associated aortic pathologies. Report: This is the report of staged hybrid TAAA treatment in a 24 year old male patient with multiple previous aortic procedures via sternotomy and thoracotomy. Retrograde infrarenal aortic visceral debranching was performed using 14 mm by 7 mm bifurcated Dacron grafts. These emerged from the limbs of an 18 mm by 9 mm bifurcated Dacron graft in an aortobi-iliac reconstruction. This was followed by staged thoracic endovascular aortic repair (TEVAR) seven days later using three endografts (26 mm–22 mm × 150 mm distal, 30 mm × 200 mm bridging, then 32 mm × 100 mm proximal). The endograft landed in an old thoracic aortic graft proximally and the new infrarenal aortic graft distally. Follow up at 11 months showed patency and no sac expansion. Conclusion: Hybrid TAAA repair was a valid treatment option in this patient with LDS and multiple previous aortic procedures. It minimised the morbidity of revision surgery and mitigated potential treatment failure by achieving an endovascular seal in surgical grafts. Short term surveillance showed no complications. Limitations to making recommendations include lack of long term follow up. |
| first_indexed | 2024-12-22T00:54:35Z |
| format | Article |
| id | doaj.art-9e9d3e63aaf74c608c9d4720cffc2888 |
| institution | Directory Open Access Journal |
| issn | 2666-688X |
| language | English |
| last_indexed | 2024-12-22T00:54:35Z |
| publishDate | 2021-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | EJVES Vascular Forum |
| spelling | doaj.art-9e9d3e63aaf74c608c9d4720cffc28882022-12-21T18:44:21ZengElsevierEJVES Vascular Forum2666-688X2021-01-01512326Hybrid Repair of a Thoraco-abdominal Aortic Aneurysm Associated with Loeys-Dietz SyndromeAhmed A. Naiem0Robert J. Doonan1Oren K. Steinmetz2Division of Vascular Surgery, McGill University, Montreal, Quebec, CanadaDivision of Vascular Surgery, McGill University, Montreal, Quebec, CanadaCorresponding author. McGill University Health Centre, Royal Victoria Hospital, 1001 Decarie Boulevard, Montreal, QC, H4A 3J1, Canada.; Division of Vascular Surgery, McGill University, Montreal, Quebec, CanadaIntroduction: Loeys-Dietz syndrome (LDS) is a genetic syndrome caused by mutations in transforming growth factor beta receptors (TGFBR) 1 and 2. It can manifest with craniofacial, musculoskeletal, cognitive abnormalities, and vascular pathologies including early onset aortic root aneurysms, extensive aortic dissections, and TAAA. Open repair is considered the gold standard treatment but carries morbidity risks, especially in patients with multiple previous aortic procedures. Endovascular treatment is associated with treatment failure when used in the native aorta, because of inherent wall weakness precluding seal. This case report adds to the available literature on hybrid treatment of LDS associated aortic pathologies. Report: This is the report of staged hybrid TAAA treatment in a 24 year old male patient with multiple previous aortic procedures via sternotomy and thoracotomy. Retrograde infrarenal aortic visceral debranching was performed using 14 mm by 7 mm bifurcated Dacron grafts. These emerged from the limbs of an 18 mm by 9 mm bifurcated Dacron graft in an aortobi-iliac reconstruction. This was followed by staged thoracic endovascular aortic repair (TEVAR) seven days later using three endografts (26 mm–22 mm × 150 mm distal, 30 mm × 200 mm bridging, then 32 mm × 100 mm proximal). The endograft landed in an old thoracic aortic graft proximally and the new infrarenal aortic graft distally. Follow up at 11 months showed patency and no sac expansion. Conclusion: Hybrid TAAA repair was a valid treatment option in this patient with LDS and multiple previous aortic procedures. It minimised the morbidity of revision surgery and mitigated potential treatment failure by achieving an endovascular seal in surgical grafts. Short term surveillance showed no complications. Limitations to making recommendations include lack of long term follow up.http://www.sciencedirect.com/science/article/pii/S2666688X21000174Hybrid repairLoeys-Dietz syndromeTEVARThoracoabdominal aortic aneurysmVisceral debranching |
| spellingShingle | Ahmed A. Naiem Robert J. Doonan Oren K. Steinmetz Hybrid Repair of a Thoraco-abdominal Aortic Aneurysm Associated with Loeys-Dietz Syndrome EJVES Vascular Forum Hybrid repair Loeys-Dietz syndrome TEVAR Thoracoabdominal aortic aneurysm Visceral debranching |
| title | Hybrid Repair of a Thoraco-abdominal Aortic Aneurysm Associated with Loeys-Dietz Syndrome |
| title_full | Hybrid Repair of a Thoraco-abdominal Aortic Aneurysm Associated with Loeys-Dietz Syndrome |
| title_fullStr | Hybrid Repair of a Thoraco-abdominal Aortic Aneurysm Associated with Loeys-Dietz Syndrome |
| title_full_unstemmed | Hybrid Repair of a Thoraco-abdominal Aortic Aneurysm Associated with Loeys-Dietz Syndrome |
| title_short | Hybrid Repair of a Thoraco-abdominal Aortic Aneurysm Associated with Loeys-Dietz Syndrome |
| title_sort | hybrid repair of a thoraco abdominal aortic aneurysm associated with loeys dietz syndrome |
| topic | Hybrid repair Loeys-Dietz syndrome TEVAR Thoracoabdominal aortic aneurysm Visceral debranching |
| url | http://www.sciencedirect.com/science/article/pii/S2666688X21000174 |
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