Disappearing multiple visceral aneurysms in Vascular Ehlers-Danlos syndrome

Abstract

Objective

To assess the complex management of arterial anomalies in Vascular Ehlers-Danlos Syndrome (vEDS).

Methods

We report the case of a 34-year-old male, diagnosed with vEDS, who presented with acute intraperitoneal hemorrhage caused by the rupture of a splenic artery aneurysm, treated in emergency with coil embolization and splenectomy. Computed Tomography (CT) scan showed the concomitant presence of right renal artery (RRA) and common hepatic artery (CHA) aneurysms.

Results

Both aneurysms were conservatively managed and the patient went through serial CT imaging. After 3 months, rapid regression of the vascular abnormalities led to complete disappearing of RRA and CHA aneurysms, confirmed at 24-month imaging follow-up. In the same time span, two pseudoaneurysms developed in other sites used for transarterial access, requiring two secondary interventions. The present case emphasizes the unpredictability of disease’s evolution and arterial complications in vEDS. Conservative management of complex lesions such as visceral artery aneurysms, which in this case resulted to be the best strategy, avoided the risks associated with surgical intervention in such fragile tissues. The reported complications underline that operative indications should be carefully weighed in these patients.

Keywords

visceral artery aneurysm connective tissue disorders ehlers-danlos syndrome percutaneous embolization stenting

Get full access to this article

View all access options for this article.

References

Chan

. Serial CT imaging of rapid progression and spontaneous regression of multiple arterial abnormalities in a patient with vascular ehlers-danlos syndrome vascular disease. Management 2014; 11(11): E274–E228.

Nishikawa

Sakamoto

Katsuyama

, et al. Multiple appearing and vanishing aneurysms: primary angiitis of the central nervous system. Case report. J Neurosurg 1998; 88(1): 133–137. DOI: 10.3171/jns.1998.88.1.0133.

Yadav

Saxena

Dutt

, et al. Vanishing aneurysms in Behcet’s disease-A case series. Monaldi Arch Chest Dis. 2021;91(1). DOI: 10.4081/monaldi.2021.1451. Published 2021 Feb 2.[In.

da Rocha de Sousa

Colucci

Dupuis

, et al. Surgical management of vascular Ehlers-Danlos syndrome and its challenges: a case report. Swiss Med Wkly 2020; 150: w20379. Published 2020 Dec 5. doi:10.4414/smw.2020.20379.

Cikrit

Miles

Silver

. Spontaneous arterial perforation: the Ehlers-Danlos specter. J Vasc Surg 1987; 5(2): 248–255.

Germain

. Ehlers-Danlos syndrome type IV. Orphanet J Rare Dis 2007; 2: 32. Published 2007 Jul 19. DOI: 10.1186/1750-1172-2-32.

Pepin

Schwarze

Rice

, et al. Survival is affected by mutation type and molecular mechanism in vascular Ehlers-Danlos syndrome (EDS type IV). Genet Med 2014; 16(12): 881–888. DOI: 10.1038/gim.2014.72.

Oderich

Panneton

Bower

, et al. The spectrum, management and clinical outcome of Ehlers-Danlos syndrome type IV: a 30-year experience. J Vasc Surg 2005; 42(1): 98–106. DOI: 10.1016/j.jvs.2005.03.053.

Bergqvist

Björck

Wanhainen

. Treatment of vascular Ehlers-Danlos syndrome: a systematic review. Ann Surg 2013; 258(2): 257–261. DOI: 10.1097/SLA.0b013e31829c7a59.

10.

Malfait

De Paepe

. The Ehlers-Danlos syndrome. Adv Exp Med Biol 2014; 802: 129–143, DOI: 10.1007/978-94-007-7893-1_9.

11.

Byers

. Vascular Ehlers-Danlos Syndrome. In: Adam

Everman

Mirzaa

, et al. (eds) GeneReviews® [Internet]. Seattle, WA: University of Washington, Seattle, 1999, pp. 1993–2022. https://www.ncbi.nlm.nih.gov/books/NBK1494/. Sep 2 [Updated 2019 Feb 21].

12.

Bade

Queral

Mukherjee

, et al. Endovascular abdominal aortic aneurysm repair in a patient with Ehlers-Danlos syndrome. J Vasc Surg 2007; 46(2): 360–362. DOI: 10.1016/j.jvs.2007.03.045.

13.

Solomon

Abrams

Lichtenstein

. GI manifestations of Ehlers-Danlos syndrome. Am J Gastroenterol 1996; 91(11): 2282–2288.

14.

Byard

Keeley

Smith

. Type IV Ehlers-Danlos syndrome presenting as sudden infant death. Am J Clin Pathol 1990; 93(4): 579–582. DOI: 10.1093/ajcp/93.4.579.

15.

Freeman

Swegle

Sise

. The surgical complications of Ehlers-Danlos syndrome. Am Surg 1996; 62(10): 869–873.

16.

Berney

La Scala

Vettorel

, et al. Surgical pitfalls in a patient with type IV Ehlers-Danlos syndrome and spontaneous colonic rupture. Report of a case. Dis Colon Rectum 1994; 37(10): 1038–1042. DOI: 10.1007/BF02049321.

17.

Brooke

Arnaoutakis

McDonnell

, et al. Contemporary management of vascular complications associated with Ehlers-Danlos syndrome. J Vasc Surg 2010; 51(1): 131–138. DOI: 10.1016/j.jvs.2009.08.019.

18.

Ong

Perdu

De Backer

, et al. Effect of celiprolol on prevention of cardiovascular events in vascular Ehlers-Danlos syndrome: a prospective randomised, open, blinded-endpoints trial. 2016; 388(10044): 564. Dosage error in published abstract; MEDLINE/PubMed abstract corrected; Dosage error in article text]. Lancet. 2010;376(9751):1476–1484. DOI: 10.1016/S0140-6736(10)60960-9.

19.

Frank

Adham

Seigle

, et al. Vascular ehlers-danlos syndrome: long-term observational study. J Am Coll Cardiol. 2019;73(15):1948–1957. doi:10.1016/j.jacc.2019.01.058.

20.

Atanabe

Wada

Tei

, et al. A novel gene therapy strategy for Vascular Ehlers-Danlos syndrome by the combination with RNAi mediated inhibition of a mutant allele and transcriptional activation of a normal allele. Mol Ther 2005; 11: S240, DOI: 10.1016/j.ymthe.2005.07.158.

21.

Zilocchi

Macedo

Oderich

, et al. Vascular Ehlers-Danlos syndrome: imaging findings. AJR Am J Roentgenol 2007; 189(3): 712–719. DOI: 10.2214/AJR.07.2370.

22.

Chu

Johnson

Dietz

, et al. Vascular complications of Ehlers-Danlos syndrome: CT findings. AJR Am J Roentgenol 2012; 198(2): 482–487. DOI: 10.2214/AJR.11.6603.

23.

van de Laar

IMBH

Baas

De Backer

, et al. Surveillance and monitoring in vascular Ehlers-Danlos syndrome in European Reference Network For Rare Vascular Diseases (VASCERN). Eur J Med Genet 2022; 65(9): 104557. DOI: 10.1016/j.ejmg.2022.104557.