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Introduction Implementing an intrathecal drug delivery system (IDDS) in a patient diagnosed with von Willebrand disease (vWD) necessitates meticulous planning due to the high risk of bleeding complications.

Methods Case report: This case involved a 55-year-old woman previously diagnosed with complex regional pain syndrome. Initially, she underwent spinal cord stimulation (SCS) without incident. However, after 28 months, the device was removed due to malfunctioning electrodes, and she opted for an IDDS. Following SCS removal, epidural patient-controlled analgesia was administered as a bridging therapy until the IDDS could be implanted. Compared with the SCS procedures, significant bleeding occurred during this phase. Although the patient had cirrhosis, her liver function and coagulation profiles were normal at the time. Nonetheless, an elevated PFA-100 test prompted referral to a hematologist, who suspected vWD. The hematologist recommended pre-procedural intravenous administration of a mixture of 0.3 ug/kg desmopressin and 50 mL normal saline over 30 to 40 minutes. Additionally, the use of cryoprecipitate or fresh frozen plasma (FFP) was advised if there was any history of bleeding. Given her recent frequent bruising, two pints of FFP were administered. After the preoperative administration of desmopressin in the waiting room and the initiation of FFP, she was transferred to the operating room where the procedure was performed under aseptic conditions without any complications.

Conclusions Conclusion: With careful preoperative preparation and vigilant perioperative management, IDDS implantation in patients with vWD can be conducted safely and effectively, mitigating the inherent risks associated with this condition.