Spontaneous rupture of the spleen in type IV Ehlers-Danlos syndrome: Report of a case

Abstract

The vascular type of Ehlers-Danlos syndrome, type IV, is associated with severe complications, including arterial rupture and visceral perforation. However, to our knowledge, there has been only one previous report of splenic rupture caused by a spontaneous hemorrhage in type IV Ehlers-Danlos syndrome. We report another case of this uncommon complication, occurring in a 35-year-old woman who presented after the sudden onset of acute abdominal pain. Patients should be stabilized quickly in the intensive care unit and the most timesaving surgical techniques used. Moreover, tissues must be handled with great care intraoperatively in view of their extreme fragility. Despite prompt and appropriate treatment, the prognosis is often dismal.

Spontaneously ruptured splenic aneurysm in a young patient with Ehlers-Danlos syndrome

“…Spontaneous fatal parenchymal haemorrhage of the spleen has also been described in two patients with EDS 7 8. Our patient’s family history highlights the potentially fatal nature of this collagen disorder with both vascular and intestinal complications seen in the same family.…”

Sigmoid colon perforation induced by the vascular type of Ehlers-Danlos syndrome: Report of a case

“…[1][2][3][4] However, patients who are affected by EDS, particularly the vascular EDS type (vEDS), develop complications associated with tissue weakness, and surgical or interventional therapy is often required. [1][2][3][4][9][10][11] Until genetic and biochemical testing was suffi ciently developed, a considerable number of patients who died unexpectedly could not be diagnosed as having vEDS. In the present case, the reason for the colonic perforation was unclear after a histopathological examination, and 6 months passed until the diagnosis of vEDS could be made by genetic and biomolecular assays.…”

Gastrointestinal Surgery and Related Complications in Patients with Ehlers-Danlos Syndrome: A Systematic Review

“…However, there are few documented case reports of spontaneous liver rupture [26], splenic rupture [27] and gallbladder rupture [16], probably due to a reduced amount of structurally intact supportive tissue in these organs. Pancreatitis in patients with EDS has been described [28] and the cause is thought to be microvascular damage to the capillaries in the pancreas.…”