Perioperative Management of a Patient with Congenital Afibrinogenemia who Underwent Ileocecalectomy; A Case Report

Tomoyuki Tagi, Shojiro Kikuchi, Tokunari Okayama, Toshiya Ochiai, Teruhisa Sonoyama and Hisakazu Yamagishi

Department of Digestive Surgery, Kyoto Prefectural University of Medicine

We report a case of congenital afibrinogenemia and perioperative management after ileocecal resection with fibrinogen supplementary therapy. A 36-year-old man followed up for congenital afibrinogenemia and reporting right lower abdominal pain was found in preoperative diagnosis to have localized peritonitis due to acute appendicitis. Preoperative serum fibrinogen was 50 mg/dl (normal range: 200-400 mg/dl) because he had received supplementary fibrinogen therapy as an outpatient. Serum fibrinogen increased to 198 mg/dl after intravenous fibrinogen administration interoperatively. The operative diagnosis was local peritonitis due to diverticulitis, necessitating ileocecal resection with no bleeding tendency. Fibrinogen was also administered postoperatively and serum fibrinogen maintained at 48-199 mg/dl. No complications occurred postoperatively. Supplementary fibrinogen therapy effectively prevented abnormal bleeding during operation, wound healing failure, and suture rupture. Perioperative management of afibrinogenemia is unclear and no consensus exists on how to maintain minimum plasma fibrinogen perioperatively. Our findings and discussions in the literature suggest that the serum fibrinogen concentration should be maintained above 100 mg/dl perioperatively.

Key words
afibrinogenemia, fibrinogen, perioperative management

Reprint requests
Tomoyuki Tagi Department of Digestive Surgery, Kyoto Prefectural University of Medicine
465 Kajii-cho, Kawaramachi-Hirokoji, Kamigyou-ku, Kyoto, 602-8566 JAPAN

Accepted
March 30, 2005

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