Spontaneous splenic rupture, mesenteric ischemia and spinal infarction after aortic repair for acute type A dissection in a patient with sickle cell trait

Makoto Toyoda,Kagami Miyaji,Yoshikiyo Matsunaga,Masaki Nie,Kouki Nakashima,Tadashi Kitamura

doi:10.1007/s11748-020-01520-1

Makoto Toyoda, Kagami Miyaji + Show 4 more

Open Access

https://doi.org/10.1007/s11748-020-01520-1

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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.

Highlights

CaseSickle cell trait (SCT) is a benign condition resulting from a heterozygous mutation of beta-globin gene allele that encodes hemoglobins (Hbs) A and S
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
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

Summary

Introduction

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. 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. CT showed a reduced contrast effect in the peripheral branch of the superior mesenteric artery, ischemic small bowel, and liver and spleen infarct (Fig. 1c). Sickle cells adhered to the endothelium and aggregated with each other (Fig. 2a, b) The day, he was diagnosed with sepsis with peritonitis, kidney, and liver failure. The patient was transferred to another hospital 31 days after the first operation

Discussion

Conclusion

Findings

Compliance with ethical standards