ABSTRACT
Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome are genetic aortopathies that result from abnormal collagen matrix formation associated with vascular complications and early death. Identification of simultaneous COL3A1 and SMAD3 mutations as well as subsequent open and endovascular repair have not been reported. We present a case of a staged complete aortic replacement in a patient with a 7-cm aneurysm of his aortic arch and confirmed genetic mutations for Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome. This case highlights that, despite increased operative risk, successful staged repair of the entire aorta can be achieved in a patient with multiple severe genetic aortopathies.
ABSTRACT
Management of vascular trauma remains a challenge and traumatic injuries result in significant morbidity and mortality. Vascular trauma can be broadly classified according to mechanism of injury (iatrogenic, blunt, penetrating, and combination injuries). In addition, this can be further classified by anatomical area (neck, thoracic, abdominal, pelvic, and extremities) or contextual circumstances (civilian and military).
Subject(s)
Abdominal Injuries , Thoracic Injuries , Vascular System Injuries , Wounds, Nonpenetrating , Wounds, Penetrating , Humans , Vascular System Injuries/diagnosis , Vascular System Injuries/surgery , Wounds, Penetrating/diagnosis , Wounds, Penetrating/surgery , Extremities , Abdominal Injuries/diagnosis , Abdominal Injuries/surgeryABSTRACT
Primary aortoenteric fistula (AEF) is an uncommon but life-threatening condition. We present a case of primary AEF in an octogenarian with previous endovascular aortic repair, type II endoleak and end stage liver and renal disease. He was successfully treated with accessory renal artery ligation, duodenojejunostomy, aneurysm sac debridement and irrigation and closure of the aneurysm sac over a drain. The patient made an excellent recovery and was discharged on POD #7, with no complications noted after over a year of follow up. This approach may represent a valuable option to manage primary AEF versus open endograft explant, particularly in severely ill patients.
Subject(s)
Duodenal Diseases/therapy , Duodenostomy , Embolization, Therapeutic , End Stage Liver Disease/complications , Endoleak/therapy , Intestinal Fistula/therapy , Jejunostomy , Kidney Failure, Chronic/complications , Renal Artery/surgery , Vascular Fistula/therapy , Aged, 80 and over , Aortic Aneurysm, Abdominal/surgery , Blood Vessel Prosthesis Implantation/adverse effects , Debridement , Duodenal Diseases/diagnostic imaging , Duodenal Diseases/etiology , End Stage Liver Disease/diagnosis , Endoleak/diagnostic imaging , Endoleak/etiology , Endovascular Procedures/adverse effects , Humans , Intestinal Fistula/diagnostic imaging , Intestinal Fistula/etiology , Kidney Failure, Chronic/diagnosis , Ligation , Male , Renal Artery/diagnostic imaging , Treatment Outcome , Vascular Fistula/diagnostic imaging , Vascular Fistula/etiologyABSTRACT
BACKGROUND: Thoracic endovascular aortic repair (TEVAR) is the treatment of choice for thoracic blunt aortic injury (TBAI). A 20 mm proximal seal zone is recommended based on aneurysmal disease literature which often results in coverage of the left subclavian artery (LSA). The aim of this study was to analyze our experience with TEVAR for TBAI and evaluate whether 20 mm is required to achieve successful remodeling. METHODS: This is a single-center, retrospective study of all consecutive patients who received a TEVAR for treatment of moderate and severe TBAI between April 2014 and November 2018. Three-dimensional software reconstruction was used for computed tomography (CT) scan centerline measurements. Outcomes included technical success, need for reinterventions, and immediate and long-term aortic-related complications. RESULTS: Sixty-one patients underwent TEVAR for TBAI during the study period. Twenty-eight (46%) patients underwent LSA coverage with an average distance from the LSA to the injury of 6.4 mm (0-15.1 mm). Of the 33 (54%) patients who did not undergo coverage of the LSA, 22 patients (66%) had less than 20 mm of proximal seal zone. The mean distance from the LSA to injury in this group was 16.6 mm (7.9-29.5 mm). None of the patients with LSA coverage developed ischemic symptoms, and an average decrease in left arm systolic blood pressure of 24.8 mm Hg (0-62 mm Hg) was noted versus the right arm. There was no aortic-related mortality in either group. Follow-up CT scans revealed excellent remodeling. CONCLUSIONS: Immediate outcomes of TEVAR for TBAI with LSA coverage are well tolerated; however, the long-term sequela of LSA coverage is unknown. Exclusion of the injury and excellent remodeling appear to occur with less than 20 mm of proximal seal, and perhaps more attention should be made to preservation of the LSA.
