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Perioperative management of a patient with purpura fulminans syndrome due to protein C deficiency

[La démarche anesthésique périopératoire adoptée chez une patiente atteinte du syndrome de purpura fulminans causé par un déficit en protéine C]

Kojiro Kumagai, MD^*, Kimitoshi Nishiwaki, MD PhD, Kosei Sato, MD, Hanae Kitamura, MD, Kayo Yano, MD, Toru Komatsu, MD PhD^* and Yasuhiro Shimada, MD PhD

^* From the Department of Anesthesiology, Aichi Medical University School of Medicine, Aichi;
the Department Of Anesthesiology, Nagoya University School of Medicine and
the Department Of Anesthesiology, Japanese Red Cross Nagoya First Hospital, Nagoya, Japan.

Dr. Kojiro Kumagai, Department of Anesthesiology, Aichi Medical University School of Medicine, Nagakute-cho Aichi, 480-1195, Japan. Phone: +81-561-62-3311; Fax: +81-561-63-6621; E-mail: kkumagai{at}aichi-med-u.ac.jp

Purpose: Protein C is a vitamin K-dependent anticoagulant and homozygous protein C deficiency is a rare fatal thrombotic disease. This report describes the perioperative management of homozygous protein C deficiency in a patient who underwent a total of three surgical procedures under general anesthesia and the successful use of activated protein C concentrate.

Clinical features: A female baby, who developed disseminated intravascular coagulation and purpura fulminans shortly after birth, was diagnosed as purpura fulminans syndrome due to homozygous protein C deficiency. At one month of age, she suffered bilateral retinal detachment and glaucoma due to retinal hemorrhage. After marked improvement of her condition after administration of activated protein C concentrate, she underwent a left iridectomy and implantation of a Broviak catheter under general anesthesia. Her intraoperative course was uncomplicated but, on postoperative day four, she presented another episode of massive cutaneous necrosis and gangrene. Activated protein C concentrate was administered again, with good results. She underwent replacement of a Broviak catheter at four months of age, and right iridectomy for glaucoma at eight months. Both were uneventful.

Conclusion: The perioperative management of homozygous protein C deficiency and purpura fulminans requires appropriate measures for thromboembolic prophylaxis. Sufficient iv fluid administration is necessary. Attention should be paid to decrease the risk of tissue compression such as that associated with positioning, blood pressure cuff, and endotracheal intubation, which may cause necrosis over pressure points. Replacement therapy with activated protein C concentrate appears safe and effective. The anesthetic management is reviewed and discussed.