Coeliac Axis Thrombosis After Surgical Correction of Spinal Deformity in Type VI Ehlers-Danlos Syndrome

Abstract

An unusual case of postoperative thrombosis of celiac artery in a patient of Type VI Ehlers-Danlos syndrome (EDS) with severe kyphotic deformity is reported. To describe an unusual complication of celiac artery thrombosis following surgical correction of kyphotic deformity in Type VI EDS. Neurologic deficit following surgical correction for kyphoscoliotic deformities in patients with Type VI has been reported in 4 cases previously. There has been no previous report of combined celiac artery thrombosis leading to infarction of major abdominal organs along with quadriparesis below C7-C8. Case report and literature review. Subsequent to a 2-stage surgical correction with posterior instrumentation and fusion of spine, this patient with Type VI EDS developed celiac artery thrombosis leading to infarction of major abdominal organs. At laparotomy, he required hemihepatectomy, splenectomy, cholecystectomy, and a repair of gastric perforation. Following his abdominal catastrophe, he developed quadriparesis possibly due to anterior spinal artery ischemia. Spine surgeons treating Type VI EDS with progressive kyphoscoliosis should be aware of such an unusual complication of celiac artery occlusion in late postoperative period. Preoperative antithrombotic medication should be monitored carefully to avoid such catastrophic complication. The prognosis remains poor following anterior spinal artery ischemia due to infarction or thrombosis.