Case Report: Hybrid Approach As a Rescue Treatment in a Patient with Vascular Ehlers-Danlos Syndrome

Overview

Journal Front Surg

Specialty General Surgery

Date 2023 Nov 8

PMID 37936948

Authors

Suehyun Park

Deokbi Hwang

Woo-Sung Yun

Hyung-Kee Kim

Seung Huh

Affiliations

Soon will be listed here.

Abstract

Vascular Ehlers-Danlos Syndrome (vEDS) is a rare connective tissue disorder associated with gene mutation encoding type III collagen. Given the possible fatal prognosis if not treated timely, it is important to suspect and diagnose as soon as possible. Despite advances in endovascular technique, access point complications remain a serious challenge in patients with vEDS. Here, we describe a 30-year-old male patient who was diagnosed with vEDS after consecutive events of bilateral iliac vessels at an interval of 3 months: (1) spontaneous dissecting aneurysm of right iliac artery and (2) arteriovenous fistula between left internal iliac artery (IIA) and left common iliac vein. This patient was treated with iliac stent-grafts and overlapping femoral interposition graft (Dacron) in the 1st operation and access artery repair with surgical dissection after coil embolization of IIA and stent-graft insertion into left common to external iliac arteries in the 2nd operation. The patient has been treated with beta-blockers and anticoagulants for the management of vEDS and postoperative deep vein thrombosis, respectively. The stent-grafts in both iliac arteries and the access sites have been well-tolerated without any adverse effects for 14 months following the 2nd operation. In conclusion, given the vascular fragility and the potential for future events, additional vascular manipulation should be avoided unless it is in a life-threatening condition. In particular, meticulous hybrid interventions can be effective treatments.

References

Baderkhan H, Wanhainen A, Stenborg A, Stattin E, Bjorck M . Celiprolol Treatment in Patients with Vascular Ehlers-Danlos Syndrome. Eur J Vasc Endovasc Surg. 2020; 61(2):326-331. DOI: 10.1016/j.ejvs.2020.10.020. View

Mortani Barbosa Jr E, Pyeritz R, Litt H, Desjardins B . Vascular Ehlers-Danlos syndrome presenting as rapidly progressive multiple arterial aneurysms and dissections. Am J Med Genet A. 2011; 155A(12):3090-4. DOI: 10.1002/ajmg.a.34332. View

Brooke B, Arnaoutakis G, McDonnell N, Black 3rd J . Contemporary management of vascular complications associated with Ehlers-Danlos syndrome. J Vasc Surg. 2009; 51(1):131-8. PMC: 6042287. DOI: 10.1016/j.jvs.2009.08.019. View

Shalhub S, Black 3rd J, Cecchi A, Xu Z, Griswold B, Safi H . Molecular diagnosis in vascular Ehlers-Danlos syndrome predicts pattern of arterial involvement and outcomes. J Vasc Surg. 2014; 60(1):160-9. PMC: 4396069. DOI: 10.1016/j.jvs.2014.01.070. View

Hagspiel K, Bonatti H, Sabri S, Arslan B, Harthun N . Metachronous bilateral posterior tibial artery aneurysms in Ehlers-Danlos syndrome type IV. Cardiovasc Intervent Radiol. 2010; 34(2):413-8. DOI: 10.1007/s00270-010-9929-4. View

Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J . The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017; 175(1):8-26. DOI: 10.1002/ajmg.c.31552. View

Byers P, Belmont J, Black J, De Backer J, Frank M, Jeunemaitre X . Diagnosis, natural history, and management in vascular Ehlers-Danlos syndrome. Am J Med Genet C Semin Med Genet. 2017; 175(1):40-47. DOI: 10.1002/ajmg.c.31553. View

Ong K, Perdu J, De Backer J, Bozec E, Collignon P, Emmerich J . Effect of celiprolol on prevention of cardiovascular events in vascular Ehlers-Danlos syndrome: a prospective randomised, open, blinded-endpoints trial. Lancet. 2010; 376(9751):1476-84. DOI: 10.1016/S0140-6736(10)60960-9. View

Brooke B, Habashi J, Judge D, Patel N, Loeys B, Dietz 3rd H . Angiotensin II blockade and aortic-root dilation in Marfan's syndrome. N Engl J Med. 2008; 358(26):2787-95. PMC: 2692965. DOI: 10.1056/NEJMoa0706585. View

10.

Yasuda S, Imoto K, Uchida K, Machida D, Yanagi H, Sugiura T . Successful endovascular treatment of a ruptured superior mesenteric artery in a patient with Ehlers‒Danlos syndrome. Ann Vasc Surg. 2013; 27(7):975.e1-5. DOI: 10.1016/j.avsg.2013.01.004. View

11.

Benrashid E, Ohman J . Current management of the vascular subtype of Ehlers-Danlos syndrome. Curr Opin Cardiol. 2020; 35(6):603-609. DOI: 10.1097/HCO.0000000000000797. View

12.

Lim S, Duddy M . Endovascular treatment of a carotid dissecting pseudoaneurysm in a patient with Ehlers-Danlos syndrome type IV with fatal outcome. Cardiovasc Intervent Radiol. 2007; 31(1):201-4. DOI: 10.1007/s00270-007-9135-1. View

13.

Bergqvist D, Bjorck M, Wanhainen A . Treatment of vascular Ehlers-Danlos syndrome: a systematic review. Ann Surg. 2013; 258(2):257-61. DOI: 10.1097/SLA.0b013e31829c7a59. View

14.

Da Rocha De Sousa F, Colucci N, Dupuis A, Toso C, Buchs N, Abbassi Z . Surgical management of vascular Ehlers-Danlos syndrome and its challenges: a case report. Swiss Med Wkly. 2020; 150:w20379. DOI: 10.4414/smw.2020.20379. View

15.

Tonnessen B, Sternbergh 3rd W, Mannava K, Money S . Endovascular repair of an iliac artery aneurysm in a patient with Ehlers-Danlos syndrome type IV. J Vasc Surg. 2007; 45(1):177-9. DOI: 10.1016/j.jvs.2006.08.071. View

16.

Alqahtani M, Claudinot A, Gaudry M, Bartoli A, Barral P, Vidal V . Endovascular Management of Vascular Complications in Ehlers-Danlos Syndrome Type IV. J Clin Med. 2022; 11(21). PMC: 9658028. DOI: 10.3390/jcm11216344. View

17.

Eagleton M . Arterial complications of vascular Ehlers-Danlos syndrome. J Vasc Surg. 2016; 64(6):1869-1880. DOI: 10.1016/j.jvs.2016.06.120. View

18.

Kurata A, Oka H, Ohmomo T, Ozawa H, Suzuki S, Fujii K . Successful stent placement for cervical artery dissection associated with the Ehlers-Danlos syndrome. Case report and review of the literature. J Neurosurg. 2004; 99(6):1077-81. DOI: 10.3171/jns.2003.99.6.1077. View

19.

Pepin M, Schwarze U, Superti-Furga A, Byers P . Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med. 2000; 342(10):673-80. DOI: 10.1056/NEJM200003093421001. View