Spontaneous splenic rupture, mesenteric ischemia and spinal infarction after aortic repair for acute type A dissection i
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CASE REPORT
Spontaneous splenic rupture, mesenteric ischemia and spinal infarction after aortic repair for acute type A dissection in a patient with sickle cell trait Makoto Toyoda1 · Tadashi Kitamura2 · Kouki Nakashima1 · Yoshikiyo Matsunaga1 · Masaki Nie1 · Kagami Miyaji2 Received: 8 June 2020 / Accepted: 11 October 2020 © The Author(s) 2020
Abstract Sickle cell trait (SCT), a benign hematological condition affecting approximately 300 million individuals globally, is associated with an increased risk of vaso-occlusive disease. However, the risks related to surgery employing cardiopulmonary bypass in patients with SCT are not well established. Herein, we report the case of a 27-year-old African American man with SCT who underwent an emergency aortic repair for acute Stanford type A aortic dissection using hypothermic circulatory arrest. The patient developed a sickle cell crisis, which was followed by spontaneous splenic infarction and rupture, nonocclusive mesenteric ischemia, and spinal infarction. Keywords Aortic dissection · Mesenteric ischemia · Sickle cell trait · Splenic rupture · Spinal infarction
Introduction
Case
Sickle cell trait (SCT) is a benign condition resulting from a heterozygous mutation of beta-globin gene allele that encodes hemoglobins (Hbs) A and S. Typically, individuals with SCT have no symptoms; however, they are at a higher risk of venous thromboembolism, chronic kidney disease and exertional rhabdomyolysis [1]. Approximately 8% of African Americans and approximately 300 million individuals globally have SCT [2]. However, few studies to date have reported on patients with SCT who have undergone open heart surgery using cardiopulmonary bypass (CPB), during which hypothermia, hypoxemia, or acidosis can result in vaso-occlusive disease. Here, we report the case of a patient with SCT who presented with acute Stanford type A aortic dissection and developed splenic rupture, nonocclusive mesenteric ischemia, and spinal infarction following an emergency aortic repair.
A 27-year-old African American man with untreated hypertension presented with precordial pain. He had previously been diagnosed with SCT based on a sickle hemoglobin level of 40.4% during a screening test while newly employed. He had never experienced complications correlated with SCT. His blood pressure was 142/74 mm Hg, and his heart and respiratory rates were 54/min and 20/min, respectively. Blood oxygen saturation and body temperature were normal. Laboratory data were normal except for an increase in D-dimer (6.0 μg/mL) and C-reactive protein (2.7 mg/dL). Contrast-enhanced computed tomography (CT) showed Stanford type A aortic dissection and splenomegaly measuring 13 cm in the maximum length without malperfusion. (Fig. 1a, b). The patient was transferred to the operating theater for emergency aortic repair. Following median sternotomy, CPB was initiated with direct aortic true lumen cannulation and right atrial cannulation. Circulatory arrest with selective cerebral perfusion was initiated at a bladder temperature of 3
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