Anesth Pain Med.  2021 Jan;16(1):75-80. 10.17085/apm.20098.

Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report -

• Park JW
• Seo J
• Kim SH
• Jung KT

• KMID: 2512285
• DOI: http://doi.org/10.17085/apm.20098

Abstract

Background
Hereditary angioedema (HAE) is a rare disease caused by the deficiency of C1 esterase inhibitor. HAE has a risk of life-threatening complications such as capillary leak syndrome (CLS) and disseminated intravascular coagulation (DIC). Case: A 42-year-old man with HAE presented for deceased-donor kidney transplantation. Prophylactic fresh frozen plasma (FFP) was given before surgery because of the risk of edema development. With careful management during anesthesia, there were no problems during surgery. However, generalized edema, hypotension, hypoalbuminemia, massive drainage of serosanguineous fluids from the intraabdominal space, and DIC occurred on the day after surgery. CLS was suspected and sustained hypotension with generalized edema became worse despite treatment with albumin, danazol, FFP, and vasoactive drugs. The patient’s condition worsened despite intensive care and he died due to shock.
Conclusions
The anesthesiologist should prepare for the critical complications of HAE and prepare the appropriate treatment options.

Keyword

Capillary leak syndrome; Complement C1 inhibitor protein; Complement C1s; Disseminated intravascular coagulation; Hereditary angioedema; Kidney transplantation

Figure

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  Fig. 1. Course and treatment of the patient after surgery. A patient with hereditary angioedema who was treated with danazol presented for deceased-donor kidney transplantation. Generalized edema with hypoalbuminemia developed the day after surgery and severe hypotension occurred. Despite intensive care, the patient died due to shock. DDKT: deceased-donor kidney transplantation, FFP: fresh frozen plasma, CRRT: continuous renal replacement therapy, DNAR: Do-Not-Attempt-Resuscitation, C1-INH: C1 esterase inhibitor, Hb: hemoglobin, WBC: white blood cells: BUN: blood urine nitrogen, PT: prothrombin time, INR: international normalized ratio, aPTT: activated partial thromboplastin time, FDP: fibrinogen degradation products, RBC: red blood cell, POD: postoperative day.

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