Severe grade
Less 1 % factor
Spontaneous bleeding
<0.01 Ui/ml
Joints and muscles
Moderate grade
1–5 % factor
Occasional spontaneous bleeding.
0.01–0.05 Ui/ml
Bleeding with surgery or trauma
Mild grade
5–40 % factor
Bleeding only with surgery or trauma
0.05–0.4 UI/ml
Most clinical manifestations are related to articular bleeding (70–80 %) or muscle hematomas (10–20 %); fortunately severe bleedings that compromise the patient’s life (in the nervous system) are rare (less than 5 %). The most commonly affected joints are the ankles, knees, and elbows [2].
One of the most frequent limitations in life’s quality of hemophilic patients is problems associated with repetitive articular bleeding. Intra-articular bleeding disrupts the normal functioning of the articular cartilage, resulting in an early arthropathy with great limitation for the patient due to pain and secondary functional impotence; this situation leads these patients to be subsidiary of surgeries with high risk of perioperative bleeding, such as total knee replacement, as well as others with lower risk of bleeding, such as arthroscopic synovectomies.
The success of these interventions lies in the multidisciplinary approach between the orthopedic surgeon, anesthesiologist, and hematologist in a reference center with experience in the treatment of hemophilia [3].
14.2 Preoperative Approach
14.2.1 History
In the anamnesis we must ask the patient about bleeding frequency, trying to find the location and especially the triggering effect of it, which will guide us (along with lab tests) about the degree of factor deficiency.
We will ask about transfusion-related infections, such as hepatitis C virus or HIV. We must also corroborate the presence of other associated diseases such as:
Hypertension is more prevalent in hemophilic patients than in the general population; an optimal control previous to the surgical intervention is very important [4].
Dyslipidemia is less frequent in hemophilic patient than in general population [5].
On the other hand, a decreased risk of death from ischemic heart disease in the hemophilic population compared to the general population has been described, although it is increasing in the last years. There is an association between the use of factor and myocardial infarction. The presence of myocardial infarction in a hemophilic patient includes individualized attention due to the fact that antiplatelet therapy is not indicated [6].
14.2.2 Laboratory Tests
Platelet count, hemoglobin level, complete biochemical analysis, and clotting tests with factors VIII or IX levels
Presence of inhibitors
14.2.3 Preoperative Pain Treatment
Most surgical interventions in hemophilic patients are orthopedic surgeries, due to an advanced hemophilic arthropathy. Before coming to surgery, these patients have been treated with analgesics, with varying doses of morphine, and when pain is not controlled despite them, the orthopedic intervention is indicated [7]. It is important to corroborate in the preoperative evaluation, the usual dose of morphine consumed by the patient in order to administer it postoperatively. Table 14.2 summarizes the common analgesics used in hemophiliacs.
Pain management is done, as the intensity increases. In the second step COX-2 are indicated, which must be prescribed with caution in patients with hypertension or renal dysfunction. Other anti-inflammatory are not used in hemophilic patients due to the bleeding risk [10].
14.2.4 Physical Examination
Once the anamnesis is concluded and the regular medication consumed registered, we will focus on the physical examination, with special attention to the airway exploration, as these patients are usually candidates for general anesthesia. The management of the airway should be exquisite, with a well-organized intubation plan. It includes different options and the use different devices, due to the fact that in these patients multiple intubation attempts can trigger mucosal bleeding and endanger the patient’s life. Airway exploration test on their own has limited sensitivity and specificity, but when used in combination, their predictive value increases [11].
14.2.5 Final Considerations in the Preoperative Evaluation
As final considerations in relation to the preoperative evaluation, the following conditions shall apply:
Advice the complete surgical team (anesthesiologist, hematologist, and orthopedic surgeon) when scheduling the surgery so the whole team is aware about the patients pathology.
Confirm with the hospitals pharmacy enough factor availability for a proper perioperative management.
Confirm with the blood bank that blood products needed for surgery are reserved.
14.3 Perioperative Management
There are two important situations, hemostatic considerations and different anesthetic options.
14.3.1 Hemostatic Considerations
We must pay attention to the presence of inhibitors and to the level of the deficient factor.
Patients Without Inhibitors
The objective is to obtain an adequate level of deficient factor so the surgery can be performed. The hematologist is the main responsible of obtaining an appropriate level of preoperative factor and does also keep it during all the surgery and the postoperative period. Table 14.3 shows the percentage of factor needed in each phase and the duration of the treatment with factor depending on the type of surgery.
% factor target | Maintenance days | |
|---|---|---|
Major surgery | ||
Preoperative | 60–80 % | |
Postoperative 1 | 30–40 % | 1–3 days |
Postoperative 2 | 20–30 % | 4–6 days |
Postoperative 2 | 10–20 % | 7–14 days |
Minor surgery | ||
Preoperative | 40–50 % | |
Postoperative | 20–50 % | 1–5 days |
The reference value of 60–80 % factor just prior to anesthetic induction is the value the anesthesiologist should check in order to carry out the surgery.
Intravenous administration of concentrated either recombinant or plasma-derived factor is the correct way to get a proper percentage of factor for an adequate surgery hemostasis [13].
The half-life of factor ranges between 8 and 12 h. In the case of factor VIII, each unit per kg of patient weight given intravenously increases the plasma level about 2 IU/dl [14].
In case of unavailability of concentrated factor, cryoprecipitate or fresh frozen plasma can be administered. The initial dose of fresh frozen plasma administered is 15–20 ml/kg [15].
Patients with Inhibitors
Inhibitors are IgG antibodies that neutralize coagulation factors. They are more common in hemophilia A, with an incidence of 20–30 %, whereas in hemophilia B, they range from 5 to 10 %. They are more common in severe grades of hemophilia than in cases of mild to moderate hemophilia [16].
It is particularly important in the perioperative period, because the exposition to high doses of deficient factor, as it occurs in this period, can precipitate the antibody appearance. It must be suspected when the response to the factors infusion decreases; accurate diagnosis is determined with a laboratory test [17].
If inhibitors appears, depending on the range of them, higher doses of factor (in case of bit inhibitor) or recombinant activated factor VII will be necessary, requiring even activated prothrombin complex in cases of high amount of inhibitor [18, 19].
Other therapeutic options to improve hemostasis, like desmopressin and antifibrinolytic therapy have low utility in major surgery. Desmopressin is useful to prevent bleeding in mild or moderate hemophilia A [20]. Antifibrinolytics, in particular tranexamic acid, are used to reduce bleeding in case of dental procedures [21].
Related posts:
Hematological Concepts and Hematological Perioperative Treatment
Musculoskeletal Manifestations of Hemophilia
Shoulder Surgery in Hemophilia
Pharmacoeconomics of Orthopedic Surgery in Hemophilia
Advanced Hemophilic Arthropathy of the Ankle: Total Ankle Replacement or Ankle Fusion?
Imaging of the Hemophilic Joints
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