Protein-Z-deficiency as a rare case of unexpected perioperative bleeding in a patient with spinal cord injury.

Abstract

Case report describing the management of repeated perioperative bleeding probably due to Protein-Z-deficiency in a post-traumatic paraplegic patient. To describe the difficulty in diagnosing this rare form of hypocoagulability and the monitoring and substitution concept during three elective surgical interventions. Spinal Cord Injury Center, Bergmannstrost, Halle, Germany. A 19-year-old male suffering from a post-traumatic paraplegia sub Th8 (ASIA-A) since childhood had experienced two life-threatening intraoperative bleeding incidents before finally Protein-Z-deficiency as the underlying coagulation disorder was diagnosed. After substitution of 2000 IE PPSB (Beriplex P/N) a repeatedly postponed implantation of a sphincter-externus (Brindley-) stimulator could be performed without bleeding complications, and this was also true for two additional urological interventions 1 year later. Protein-Z levels were monitored before, during and after the operations. The preoperative application of between 1000 and 2000 IE PPSB was safe and sufficient to raise the patients' plasma Protein-Z level to almost normal and so prevent excessive intraoperative blood loss. In case of repeated bleeding tendency of unknown origin it is mandatory to look for rare causes of hypocoagulability such as Protein-Z-deficiency. We developed a substitution concept using a plasma concentrate with guaranteed Protein-Z amount (PPSB) allowing the safe performance of elective surgical interventions.