Perioperative anesthesia management of high-molecular-weight kininogen deficiency: a case report and literature review.

A prolonged activated partial thromboplastin time (APTT) is a common abnormal finding in preoperative coagulation screening. Failure to accurately identify its underlying etiology can lead to a misjudgment of bleeding risk, potentially resulting in surgical delays, unnecessary transfusion of blood products, or even invasive further investigations. These consequences, in turn, increase both healthcare costs and patient risk. The prolongation of APTT is attributable to a variety of pathophysiological states, including coagulation factor deficiencies and the presence of pathological inhibitors. Among these, High Molecular Weight Kininogen (HMWK) deficiency is an extremely rare autosomal recessive disorder, characteristically marked by a significantly prolonged APTT in vitro, yet without a corresponding clinical bleeding tendency. This study report provides a detailed account of the anesthesia management during the perioperative period in a patient with HMWK deficiency. The patient is a 27-year-old female who, due to a markedly prolonged APTT, underwent genetic testing that revealed a defect in the kininogen-1 gene (KNG1) and was diagnosed with HMWK deficiency. She was scheduled to undergo laparoscopic salpingostomy, ovarian lesion resection, and hysteroscopic endometrial lesion resection due to abnormal uterine bleeding, bilateral hydrosalpinx, and a left ovarian cyst. The patient did not receive prophylactic transfusion of blood products, such as fresh frozen plasma or coagulation factors, nor any pharmacological intervention during the perioperative period. No abnormal bleeding or thrombotic events were observed. This case report, incorporating a systematic literature review and based on the pathophysiological principle of intact hemostatic function in patients with HMWK deficiency, culminates in the formulation of a set of perioperative anesthetic management strategies for this patient population.