Splenic artery pathology presentation, operative interventions, and outcomes in 88 patients with vascular Ehlers-Danlos syndrome

Shalhub, Sherene; Nkansah, Reginald; El-Ghazali, Asmaa; Hillenbrand, C.J.; Vaidya, Sandeep; Schwarze, Ulrike; Byers, Peter H.

doi:10.1016/j.jvs.2023.04.007

Cited by 4 publications

(1 citation statement)

References 35 publications

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“…Given the lack of large natural history studies for this particular population, precautions similar to those undertaken in the care of patients with VEDS undergoing operative aortic or arterial repairs can be practiced, with a low threshold to obtain imaging studies if the postoperative course is unexpected. 14 , 15 , 16 , 17 , 18 …”

Section: Discussionmentioning

confidence: 99%

Iliac artery dissection and rupture in a patient with classic Ehlers-Danlos syndrome due to COL5A1 null variant

Pujari,

Shalhub

2024

Journal of Vascular Surgery Cases, Innovations and Techniques

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Section: Discussionmentioning

confidence: 99%

Iliac artery dissection and rupture in a patient with classic Ehlers-Danlos syndrome due to COL5A1 null variant

Pujari,

Shalhub

2024

Journal of Vascular Surgery Cases, Innovations and Techniques

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Successful management of splenic artery dissection after sigmoid colon perforation in vascular Ehlers–Danlos syndrome

Yoshizaki,

Matsuo,

Yasuda

et al. 2024

surg case rep

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Background Ehlers–Danlos syndrome (EDS) is a genetic disorder that causes fragility of the systemic connective tissues. Of the 13 subtypes, vascular EDS (vEDS) is associated with abnormalities in collagen production, resulting in arterial rupture and intestinal perforation. Herein, we report the case of a man with confirmed vEDS who survived a ruptured dissected splenic artery aneurysm triggered by perforation of the sigmoid colon. Case presentation A 48-year-old man presented to our hospital with sudden severe lower abdominal pain. The patient was genetically diagnosed with vEDS at the age of 43 years. Abdominal computed tomography (CT) showed fluid and free air surrounding the sigmoid colon. These findings suggested sigmoid colon perforation, and emergency surgery was needed. Hartmann’s procedure was performed. The resected specimen showed a 2-cm-sized depression around the perforation. Histopathological findings showed an abscess and exudate in the serosa of the perforation and thinning of the intrinsic muscular layer in the depressed area. The patient was doing well postoperatively; however, on the ninth postoperative day, sudden upper abdominal pain developed. CT revealed an intra-abdominal hemorrhage due to rupture of a dissecting splenic artery aneurysm. The aneurysm was not observed on preoperative CT and was distant from the surgical site. Urgent transcatheter arterial embolization was performed. Although embolization of the splenic artery was attempted during the procedure, the arterial dissection spread to the common hepatic artery. Moreover, the proper hepatic and gastroduodenal arteries were poorly visualized, probably due to vasospasm. Although complications associated with extensive embolization were a concern, embolization of the splenic and common hepatic arteries was necessary to save the patient’s life. After embolization, angiography showed that the left hepatic blood flow was maintained from the inferior phrenic artery, and the right hepatic inflow was maintained from the superior mesenteric artery via the peribiliary vascular plexus in the hilar area. The patient recovered well and was discharged on the 19th postoperative day. Conclusions vEDS can cause arterial rupture after intestinal surgery. Therefore, careful post-operative management is necessary. Moreover, cooperation with interventional radiologists is important for prompt treatment of vascular complications.

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Endovascular and Open Surgical Treatment of Ruptured Splenic Artery Aneurysms: A Case Report and a Systematic Literature Review

Rinaldi,

Brioschi,

Marone

2023

JCM

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Background: Ruptured splenic artery aneurysms (r-SAA), although rare, are burdened by high morbidity and mortality, even despite emergent surgical repair. It is suggested that endovascular treatment can achieve reduction in peri-operative death and complication rates, as in other vascular diseases, but evidence of such benefits is still lacking in this particular setting. We report a case of an r-SAA treated by trans-arterial embolization and then converted to open surgery for persistent bleeding, and we provide a systematic review of current results of open and endovascular repair of r-SAAs. Materials and Methods: A 50-year-old male presenting in shock for a giant r-SAA underwent emergent coil embolization and recovered hemodynamic stability. On the following day, he underwent laparotomy for evacuation of the huge intraperitoneal hematoma, but residual bleeding was noted from the splenic artery, which was ligated after coil removal, and a splenectomy was performed. A systematic literature review of the reported mortality and complications of r-SAA undergoing open (OSR) or endovascular (EVT) treatment was performed using the main search databases. All primary examples of research published since 1990 were included regardless of sample size. The main outcome measures were mortality and reinterventions. Secondary outcomes were post-operative complications. Results: We selected 129 studies reporting on 350 patients—185 treated with OSR and 165 with EVT. Hemodynamically unstable patients and ruptures during pregnancy were more frequently treated with open repair. Overall, there were 37 deaths (mortality: 10.6%)—24 in the OSR group and 13 in the EVTr group (mortality: 12.9% and 7.8% respectively, p-value: 0.84). There were 37 reinterventions after failed or complicated endovascular repair —6 treated with endovascular re-embolization and 31 with laparotomy and splenectomy (22.4%); there were 3 (1.6%) reinterventions after open repair. Overall complication rates were 7.3% in the EVT group (n: 12) and 4.2% in the OSR group (n: 7), and did not require reintervention. No significant differences in overall complications or in any specific complication rate were observed between the two groups. Conclusions: Current results of r-SAA treatment show equipoise terms of morbidity and mortality between open and endovascular repair; however, in case of hemodynamic instability and rupture during pregnancy, open surgery might still be safer. Moreover, endovascular repair is still burdened by a significantly higher rate of reinterventions, mostly with conversions to open surgery.

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Splenic artery pathology presentation, operative interventions, and outcomes in 88 patients with vascular Ehlers-Danlos syndrome

Cited by 4 publications

References 35 publications

Iliac artery dissection and rupture in a patient with classic Ehlers-Danlos syndrome due to COL5A1 null variant

Iliac artery dissection and rupture in a patient with classic Ehlers-Danlos syndrome due to COL5A1 null variant

Successful management of splenic artery dissection after sigmoid colon perforation in vascular Ehlers–Danlos syndrome

Endovascular and Open Surgical Treatment of Ruptured Splenic Artery Aneurysms: A Case Report and a Systematic Literature Review

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