Elevated prothrombin time on routine preoperative laboratory results in a healthy infant undergoing craniosynostosis repair: Diagnosis and perioperative management of congenital factor VII deficiency

• Published in: International journal of surgery case reports Vol. 24; pp. 77 - 79
• Main Authors: Jones, Kareen L., Greenberg, Robert S., Ahn, Edward S., Kudchadkar, Sapna R.
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ltd 01.01.2016; Elsevier
• Subjects: Bleeding disorders; Case Report; Craniosynostosis; Factor VII deficiency; Pediatric patients; Perioperative management; PTT; Case report; Factor VII deficiency; PT; CBC; Bleeding disorders; Craniosynostosis; CMP; Perioperative management; INR; Pediatric patients
• ISSN: 2210-2612; 2210-2612
• DOI: 10.1016/j.ijscr.2016.05.004

•Factor VII deficiency is a rare autosomal disorder with genotypic and phenotypic variability.•Preoperative lab evaluation should at a minimum consist of a hematocrit, platelet count, type and screen, and coagulation studies for high-risk surgeries.•Treatment of acute hemorrhage in factor VII deficient patients primarily consists of factor VII (FVII) replacement therapy. Congenital factor VII deficiency is a rare bleeding disorder with high phenotypic variability. It is critical that children with congenital Factor VII deficiency be identified early when high-risk surgery is planned. Cranial vault surgery is common for children with craniosynostosis, and these surgeries are associated with significant morbidity mostly secondary to the risk of massive blood loss. A two-month old infant who presented for elective craniosynostosis repair was noted to have an elevated prothrombin time (PT) with a normal activated partial thromboplastin time (aPTT) on preoperative labs. The infant had no clinical history or reported family history of bleeding disorders, therefore a multidisciplinary decision was made to repeat the labs under general anesthesia and await the results prior to incision. The results confirmed the abnormal PT and the case was canceled. Hematologic workup during admission revealed factor VII deficiency. The patient underwent an uneventful endoscopic strip craniectomy with perioperative administration of recombinant Factor VIIa. Important considerations for perioperative laboratory evaluation and management in children with factor VII deficiency are discussed. Anesthetic and surgical management of the child with factor VII deficiency necessitates meticulous planning to prevent life threatening bleeding during the perioperative period. A thorough history and physical examination with a high clinical suspicion are vital in preventing hemorrhage during surgeries in children with coagulopathies. Abnormal preoperative lab values should always be confirmed and addressed before proceeding with high-risk surgery. A multidisciplinary discussion is essential to optimize the risk-benefit ratio during the perioperative period.