Case report | Volume 36, Article 46, 29 May 2020 | 10.11604/pamj.2020.36.46.23561

Congenital Factor V deficiency: perioperative management (case report)

Mohamed Anass Fehdi, Mohamed Lazraq, Sabah Benhamza, Abdelhak Bensaid, Youssef Miloudi, Najib ElHarrar

Corresponding author: Mohamed Anass Fehdi, Anesthesia and Critical Care Department, 20th August Hospital, University Hospital Ibn Rochd, Casablanca, Morocco

Received: 15 May 2020 - Accepted: 16 May 2020 - Published: 29 May 2020

Domain: Intensive care medicine,Internal medicine,Otolaryngology (ENT)

Keywords: Anesthesiology, perioperative management, congenital factor V deficiency, hematology

©Mohamed Anass Fehdi et al. Pan African Medical Journal (ISSN: 1937-8688). This is an Open Access article distributed under the terms of the Creative Commons Attribution International 4.0 License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Cite this article: Mohamed Anass Fehdi et al. Congenital Factor V deficiency: perioperative management (case report). Pan African Medical Journal. 2020;36:46. [doi: 10.11604/pamj.2020.36.46.23561]

Available online at: https://www.panafrican-med-journal.com/content/article/36/46/full

Home | Volume 36 | Article number 46

Case report

Congenital Factor V deficiency: perioperative management (case report)

Congenital Factor V deficiency: perioperative management (case report)

Mohamed Anass Fehdi1,&, Mohamed Lazraq1, Sabah Benhamza1, Abdelhak Bensaid1, Youssef Miloudi1, Najib El Harrar1

 

1Anesthesia and Critical Care Department, 20th August Hospital, University Hospital Ibn Rochd, Casablanca, Morocco

 

 

&Corresponding author
Mohamed Anass Fehdi, Anesthesia and Critical Care Department, 20th August Hospital, University Hospital Ibn Rochd, Casablanca, Morocco

 

 

Abstract

Factor V congenital deficiency is a rare hereditary disease, it exposes patients to hemorrhagic risk, with high morbi-mortality. Its management is a real challenge for practitioners. Perioperative management of patients with Factor V congenital deficiency needs anesthetists, hematologists and surgeons to work in close collaboration.

 

 

Introduction    Down

Factor V congenital deficiency is a rare hereditary factor deficiency, due to lack in plasmatic concentration of factor V. It is responsible for hemorrhages, going from mild to severe hemorrhagic symptoms. The perioperative management of patients with factor V deficiency is a real challenge for anesthetists, as the hemorrhagic risk is higher in these patients. We report the case of a patient with factor V deficiency, and our experience in the perioperative management of her cochlear implant surgery.

 

 

Patient and observation Up    Down

A 45 years old female patient, with history of bilateral deafness, secondary to bilateral chronic otitis media. The pose of cochlear implant under general anesthesia was indicated. Preanesthetic evaluation indicated a safe anesthesia with no risk of complications. Biological assessment has showed an abnormal hemostasis, with a very low prothrombin time (PT) at 15%, and a long activated Partial Thromboplastin Time (aPTT) at 50 seconds (for a normal aPTT at 28 seconds). In the first time we referred our patient for a hematological assessment. The screening of plasmatic coagulation factors, has objectified a very low Factor V level at 1% (normal range from 70% to 120%). The screening of circulating antibodies was negative. Members of family were also tested, and the patient´s brother was found to be carrying the same factor deficiency, which indicated its hereditary nature. Further interrogation of the patient has clarified the presence of menorrhagia episodes in the past. For perioperative management, and with the help of hematologists, fresh frozen plasma (FFP) transfusion was performed, 24 hours before surgery, in order to bring factor V level between 15% and 20%. Another transfusion one hour before surgery was necessary. The surgical procedure was performed with no complications, particularly hemorrhagic accidents. The surgeons judged the hemostasis as satisfying during surgery. The transfusion of fresh frozen plasma was maintained at D-1 after surgery, and after that a daily screening of PT and Factor V level helped us modulating the FFP transfusion. The patient´s postoperative recovery was without any complications. She was transferred to hematology ward for further follow-up and surveillance.

 

 

Discussion Up    Down

Factor V congenital deficiency is rare hereditary disease. Its transmission is autosomal recessive, secondary to mutation in F5 gene (1q23) [1]. The biological diagnosis is based on the low level of Factor V, long aPPT and low PT [1-3]. It is responsible for a hemorrhagic syndrome, of variable severity, with no correlation between the plasmatic concentration of factor V and the severity of hemorrhagic symptoms, in fact it is more related to the level of factor V in platelets [4]. Our patients had a very low factor V level, with mild symptoms. Due to the lack of concentrated factor V, the perioperative management of patients with congenital factor V deficiency, requires a transfusion protocol with fresh frozen plasma, aiming a factor V level between 15% and 20%, the hemostasis then is sufficient [1-3]. Platelets transfusion might be necessary in severe cases [2,3]. Although the hemorrhagic risk, the final outcome is often favorable, as long as the diagnosis is done early, and the perioperative management suitably [1,2,4].

 

 

Conclusion Up    Down

The perioperative management of patients with congenital factor V deficiency requires an effective collaboration between anesthetists, surgeons and hematologists. The preanethetic evaluation is a crucial moment that should be done thoroughly. A transfusion protocol should be carried out suitably to guarantee a good outcome in these patients.

 

 

Competing interests Up    Down

The authors declare no competing interests.

 

 

Authors' contributions Up    Down

The case report was written by Mohamed Anass Fehdi, the references were assured by Pr Lazraq Mohamed as well as the first review. Pr Sabah Benhamza helped with the 2nd review. Our work wes closely supervised by Pr Abdelhak Bensaid, Pr Youssef Miloudi and Pr Najib Alharrar. All the authors have approuved the final version of the manuscript.

 

 

References Up    Down

  1. Peyvandi F, Menegatti M. Treatment of rare factor deficiencies in 2016. Hematology Am Soc Hematol Educ Program. 2016 Dec 2;2016(1):663-669. PubMed | Google Scholar

  2. Meidert AS, Kinzinger J, Möhnle P, Pekrul I, Spiekermann K, Thorsteinsdottir J et al. Perioperative Management of a Patient with Severe Factor V Deficiency Presenting with Chronic Subdural Hematoma: A Clinical Report. World Neurosurg. 2019; 127:409-413. PubMed | Google Scholar

  3. Bennett K, Daley ML, Pike C. Factor V deficiency and menstruation: a gynecologic challenge. The American College of Obstetricians and Gynecologists. 1997 May;89(5 Pt 2):839-40. PubMed | Google Scholar

  4. Song JW, Um MR, Ahn HS, Hong CY. A case of congenital factor V deficiency. J Korean Med Sci. 1987;2(3):179-82. PubMed | Google Scholar

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Case report

Congenital Factor V deficiency: perioperative management (case report)

Case report

Congenital Factor V deficiency: perioperative management (case report)

Case report

Congenital Factor V deficiency: perioperative management (case report)