Splenic Rupture Secondary to Vascular Ehlers–Danlos Syndrome Managed by Coil Embolization of the Splenic Artery
15 May 2019
22 September 2019
22 November 2019 (online)
Aim Atraumatic splenic rupture is uncommon and life-threatening. It may be related to underlying pathology and be the initial manifestation of the condition. Vascular Ehlers–Danlos syndrome (V-EDS) is a rare autosomal dominant collagen vascular disorder, associated with vessel fragility and rupture. We describe a child presenting with splenic rupture managed by embolization of the splenic artery. She was subsequently diagnosed with V-EDS.
Case Description A 11-year-old girl with thalassemia trait presented with sudden onset of abdominal pain and hypovolemic shock. There was no history of trauma. Following resuscitation, abdominal computed tomography showed hemoperitoneum and active splenic arterial extravasation. Angiography demonstrated four bleeding points, from irregular vessels supplying the upper two-thirds of the spleen. These were not amenable to supraselective embolization. Therefore, coil embolization of the main splenic artery was performed, with no splenic supply seen on the postembolization angiogram. Her postoperative recovery was complicated by pancreatitis secondary to partial ischemia of the pancreatic tail. Subsequent extensive investigations excluded hematological, myeloproliferative, and infective causes for her splenic rupture. A safeguarding investigation was completed, with no pertinent factors identified. Findings of thin skin, abnormal bruising, and hypermobile joints raised a clinical suspicion of a connective tissue disorder. Genetic testing revealed a de novo mutation of the COL3A1 gene.
Conclusions There are only four reports of V-EDS causing splenic rupture in the literature to date. These patients were all adults and only one had not previously been diagnosed with V-EDS. All underwent splenectomy. While V-EDS presenting with abdominal visceral rupture in children has been reported, this is the first report of a child with V-EDS presenting with splenic rupture. It is the only case of splenic rupture secondary to V-EDS that has been managed minimally invasively by embolization.
- 1 Byers PH, Belmont J, Black J. , et al. Diagnosis, natural history, and management in vascular Ehlers-Danlos syndrome. Am J Med Genet C Semin Med Genet 2017; 175 (01) 40-47
- 2 Gedik E, Girgin S, Aldemir M, Keles C, Tuncer MC, Aktas A. Non-traumatic splenic rupture: report of seven cases and review of the literature. World J Gastroenterol 2008; 14 (43) 6711-6716
- 3 Carter J, Fenves AZ. Understanding vascular-type Ehlers-Danlos syndrome and avoiding vascular complications. Proc Bayl Univ Med Cent 2017; 30 (01) 52-53
- 4 Cortini F, Marinelli B, Seia M. , et al. Next-generation sequencing and a novel COL3A1 mutation associated with vascular Ehlers-Danlos syndrome with severe intestinal involvement: a case report. J Med Case Reports 2016; 10 (01) 303
- 5 Ong KT, Perdu J, De Backer J. , et al. Effect of celiprolol on prevention of cardiovascular events in vascular Ehlers-Danlos syndrome: a prospective randomised, open, blinded-endpoints trial. Lancet 2010; 376 (9751): 1476-1484
- 6 Ahuja C, Farsad K, Chadha M. An overview of splenic embolization. AJR Am J Roentgenol 2015; 205 (04) 720-725
- 7 Schnüriger B, Inaba K, Konstantinidis A, Lustenberger T, Chan LS, Demetriades D. Outcomes of proximal versus distal splenic artery embolization after trauma: a systematic review and meta-analysis. J Trauma 2011; 70 (01) 252-260
- 8 Guillon R, Garcier JM, Abergel A. , et al. Management of splenic artery aneurysms and false aneurysms with endovascular treatment in 12 patients. Cardiovasc Intervent Radiol 2003; 26 (03) 256-260
- 9 Tokuda T, Tanigawa N, Kariya S. , et al. Pancreatitis after transcatheter embolization of a splenic aneurysm. Jpn J Radiol 2010; 28 (03) 239-242
- 10 Hamers RL, Van Den Berg FG, Groeneveld AB. Acute necrotizing pancreatitis following inadvertent extensive splenic artery embolisation for trauma. Br J Radiol 2009; 82 (973) e11-e14
- 11 Khurana A, Abdel Khalek M, Brown J, Barry B, Jaffe BM, Kandil E. Acute necrotizing pancreatitis following splenic artery embolization. Trop Gastroenterol 2011; 32 (03) 226-229
- 12 Paul DB, Opalek JM. Proximal splenic arterial embolization may also result in pancreatic necrosis. J Trauma 2011; 71 (01) 268-269