ISSN: 2320-5407 Int. J. Adv. Res.

12(03), 1016-1018

Journal Homepage: - www.journalijar.com

Article DOI: 10.21474/IJAR01/18486

DOI URL: http://dx.doi.org/10.21474/IJAR01/18486

RESEARCH ARTICLE
PERIOPERATIVE MANAGEMENT OF A PATIENT WITH INHERITED FACTOR VII DEFICIENCY
AND CROHN DISEASE : A CASE REPORT AND REVIEW OF THE LITERATURE

Salim Chajai1, Asmae Chaker1, Al Mahdi Awab1, Rachid El Moussaoui1, Ahmed El Hijri1, Abderrahim
Azzouzi1, Hamza Ouzaouit2 and Abdelmalek Hrora2
1. Surgical Anesthesia Department, Ibn Sina University Hospital, Rabat, Morocco.
2. Gastrointestinal Surgery Department, Ibn Sina University Hospital, Rabat, Morocco.
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Manuscript Info Abstract
……………………. ………………………………………………………………
Manuscript History Inherited factor VII deficiency is a rare coagulation disorder. There is
Received: 28 January 2024 no direct correlation between factor VII activity and the bleeding risk.
Final Accepted: 29 February 2024 We report the case of a 24 years old patient with Crohn’s disease and
Published: March 2024 inherited factor VII deficiency who underwent ileocecal resection. The
patient was considered as a high bleeding risk and was thus substituted
with factor VII perioperatively. In the presence of thromboembolic risk
factors, pharmacological venous thromboembolism prophylaxis was
also introduced.

Copy Right, IJAR, 2024,. All rights reserved.

……………………………………………………………………………………………………....
Introduction:-
Inherited factor VII deficiency is a rare coagulation disorder. There is no direct correlation between factor VII
activity and the bleeding risk. Perioperative management is based on expert opinion rather than recognized
guidelines. We report the case of a 24 years old patient with Crohn’s disease and inherited factor VII deficiency who
underwent ileocecal resection.

Case report
A 24 years old patient admitted for the perioperative management of a Crohn’s disease related intestinal stricture.
The patient has a known inherited factor VII deficiency, and presented a bleeding requiring blood transfusion
following a dental extraction when he was 8 years old. At 16 years old, the patient had a perianal fistulotomy under
general anesthesia with perioperative factor VII substitution, and pathology revealed a Crohn’s disease. The patient
is since on Adalimumab and Mesalazine.

The current history was consistent with an intestinal occlusion secondary to an ileocecal valve stricture, presenting
as a König syndrome that lasted for two months. Laparoscopic surgery was performed after the patient received
2milligrams (33mcg/kg) of activated recombinant factor VII (rFVIIa) intravenously, as preoperative coagulation
studies showed a prothrombine time of 22,5 seconds (normal : 10 to13 seconds) with a normal activated partial
thromboplastin time and a Factor VII activity of 27% . The surgery, an ileocecal resection with ileocolic
anastomosis went uneventful with minimal bleeding. The patient subsequently received 2mg rFVIIa every 6 hours
for five days until he was discharged, for a total dose of 666mcg/kg. Venous thromboembolic prophylaxis was
administered for two weeks using subcutaneous enoxaparin 40mg.

Corresponding Author:- Salim Chajai

Address:- Surgical Anesthesia Department, Ibn Sina University Hospital, Rabat, Morocco.
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Discussion:-
Factor VII (FVII) is a serine protease zymogen synthetized by the liver exclusively. It is a vitamin K dependent
coagulation factor. Its plasmatic concentration ranges from 0,35 to 0,6mg/l, which corresponds to the 70 to 140%
rates commonly used by laboratories. Its half life is very short approximating 4 to 6 hours, and most of factor VII
circulates as a single strand inactive form. As a unique feature, a minimal proportion of weakly active double strand
Factor VII (1 to 3%) can circulate freely even in the absence of coagulation activation. The active enzymatic form is
linked to Tissue factor (TF) which plays the role of a cofactor, increasing the catalytic activity of FVII. FVII
becomes active through proteolytic cleavage by proteases such as activated factor X, activated factor IX and
activated factor VII. The TF-FVII complex is considered as the initiator of coagulation in vivo, the process of
coagulation being triggered by the binding of little amounts of free circulating activated FVII (FVIIa) to TF or
resulting from the autoactivation of FVII inside the TF-FVII complex. The complex TF-FVIIa can then exert its
enzymatic activity on factor IX and factor X, with the final product being the formation of a fibrin clot. This step is
regulated negatively by the tissue factor pathway inhibitor (TFPI) [1]. The involvement of multiple factors explains
the absence of correlation between the plasmatic values and the hemorrhagic risk in deficient patients.

Hereditary factor VII deficiency is rare with a prevalence of 1/500 000, and defining its clinical picture and
treatment relies on data from international multicentric studies like the International Registry on Congenital Factor
VII Deficiency (IRF7) [2] and the Seven Treatment Evaluation Registry (STER) [3]. This autonomic recessive
disorder can be caused by various mutations and is thus heterogenous. The deficit can be quantitative (type I) or
qualitative (type II). It can be completely asymptomatic in 30% of patients, even during surgeries, or it can present
with severe to lethal bleeding ranging from epistaxis and ecchymosis to intracranial hemorrhage.

The diagnosis can be made during a family screening or in the context of bleeding. It is established in the presence
of a prolonged prothrombin time and an isolated low FVII activity. The lower limit of the reference range of the
FVII activity, defined as 70%, is based on the statistical analysis of a reference sample reflecting the normal
population. However, data from the STER and IRF7 show that the deficit can be considered significant when values
are lower than 26% [4]. Bleeding is uncommon for values exceeding 10%, and spontaneous hemorrhage is unusual
when rates exceed 20% [2,5,6]. Data from the STER and IRF7 also showed that bleeding at presentation predicted
subsequent bleeding risk [6]. It has thus been proposed to classify the bleeding risk using personal or family
bleeding history and FVII activity. Patients with a high risk are those having a FVII activity lower than 2% and a
bleeding history, while patients with a low risk have a FVII activity higher than 20% with no bleeding history [5].
When planning an invasive procedure, especially major, the bleeding risk cannot be underestimated, even in
asymptomatic patients. These patients can bleed later in life [4]. Also, the bleeding anamnesis gives little
information in younger children. Our patient, who was asymptomatic until the age of 8, bled after a dental extraction
and needed a blood transfusion. As a consequence, we considered him as a high risk patient.

The ideal substitution for factor VII deficiency is recombinant FVIIa concentrate (rFVIIa). When unavailable,
plasma derived concentrates, fresh frozen plasmas or prothrombin complex concentrate can be used. Practice is
variable when it comes to the dosing and duration of rFVIIa substitution. For major surgery, a minimal dose of
13mcg/kg administered at least three times at 6 hours intervals was efficient in preventing bleeding [7]. For invasive
procedures and minor surgery, a total cumulated dose of 20mcg/kg in one day was efficient in preventing bleeding
[8]. It is suggested to continue the substitution until healing and hemostasis are guaranteed. If it is decided to
proceed to surgery without substituting the patient, rFVIIa must be kept available.

FVII substitution can come with a risk of venous or arterial thrombosis, especially when other risk factors exist [9],
like the presence of an inflammatory bowel disease. The use of antithrombotic prophylaxis in patients with factor
VII deficiency is reported in the litterature. It has been suggested to give pharmacologic prophylaxis to
asymptomatic patients with a FVII activity greater than 30%, or with a FVII between 10 and 30% with a history of
thrombosis, and to avoid it in the presence of a severe deficiency [10].

Conclusion:-
As a conclusion, inherited factor VII deficiency is a rare coagulation disorder, and a bleeding risk exists in the
presence of a hemorrhagic history or with a factor VII activity lower than 20%. The proposed substitution is a
minimum dose of 13mcg/kg administered at least three times at 6 hours intervals for major surgery and a total

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cumulated dose of 20mcg/kg in one day for minor surgery and invasive procedures. In the presence of
thromboembolic risk factors, pharmacologic thromboembolic prophylaxis should be considered.

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