Titre original :
Splenic artery pathology presentation, operative interventions, and outcomes in 88 patients with Vascular Ehlers Danlos Syndrome
Titre en français :
Présentation, prise en charge et devenir de 88 patients avec pathologie de l'artère splénique liée à une maladie d'Ehlers Danlos
Auteurs :
Sherene Shalhub, Reginald Nkansah, Asmaa El-Ghazali, C.J. Hillenbrand, Sandeep S. Vaidya, Ulrike Schwarze, Peter H. Byers,
Revue :
Journal of Vascular Surgery,2023, https://doi.org/10.1016/j.jvs.2023.04.007.
Background
Vascular Ehlers-Danlos Syndrome (VEDS) is rare and associated with arteriopathies. The aim of this study is to investigate the presentation, operative interventions, and outcomes of splenic arterial pathology in a population of more than 1,500 individuals with genetically confirmed VEDS due to pathogenic COL3A1 variants.
Methods
Cross-sectional analysis of 1,547 individuals was performed. The data were assembled by harmonizing data from three overlapping cohorts with genetically confirmed VEDS: the VEDS Collaborative Natural History Study (N=242), a single center cohort (N=75), and the UW Collagen Diagnostic Lab cohort (N=1,231). Duplicates were identified and removed. Patients were selected for analysis if they had splenic artery aneurysm (SAA), pseudoaneurysm, dissection, thrombosis, or rupture. Demographics, COL3A1 variants, interventions, and outcomes were analyzed. Comparisons by splenic artery rupture were made.
Results
A total of 88 patients presented between 1992 and 2021 with splenic artery pathology (5.7% of the cohort, Mean age at diagnosis 37+11.1 years, 50% male). One third were diagnosed with VEDS prior to the splenic artery pathology diagnosis and 17% were diagnosed post-mortem. Most had a positive family history (61%). Most had COL3A1 variants associated with minimal normal collagen production (71.589.7%). Median follow up was 8.5 (IQR 0.9-14.7) years. Initial presentation was rupture in 47% of the cases. Splenic artery rupture overall was 51% (N=45) including 4 cases of splenic rupture. There were no major differences in VEDS related manifestations or COL3A1 variant type by rupture status. SAA was noted in 39% of the cases. Only 12 patients had splenic artery diameter documented in 12 cases with a median diameter of 12 (IQR 10.3-19.3) mm. A total of 34 (38.6%) patients underwent 40 splenic arterial interventions: 21 open surgical, 18 embolization, and 1 unknown procedure. More than one splenic artery intervention was performed in 5 (14.7%) cases. Open repair complications included arteriovenous fistula (n=1), intestinal or pancreatic injury (1 each), and four intraoperative deaths. There were no deaths or access site complications related to splenic artery embolization. Four (23.5%) developed a new SAA in the remaining splenic artery post embolization. All-cause mortality was 35% (n=31) including 22 related to a ruptured splenic artery.
Conclusions
Splenic arteriopathy in VEDS is associated with variants that affect the structure and secretion of type III collagen and frequently present with rupture. Rupture and open repair are associated with high morbidity and mortality while embolization is associated with favorable outcomes. Suggest repair considerations at SAA diameter of 15 mm. Long term follow up is indicated as secondary splenic arteriopathy can occur.