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CASE REPORT
Year : 2022 | Volume
: 30
| Issue : 3 | Page : 82-84
Rhinoplasty on patients with von Willebrand's disease: Tranexamic acid may be a better choice than desmopressin in selected cases
Galip Gencay Ustun1, Zuhre Kaya2
1 Department of Plastic Reconstructive and Aesthetic Surgery, Faculty of Medicine, Hacettepe University, Ankara, Turkey
2 Department of Pediatric Hematology, Faculty of Medicine, Gazi University, Ankara, Turkey
Correspondence Address:
Dr. Galip Gencay Ustun
Department of Plastic Reconstructive and Aesthetic Surgery, Hacettepe University Faculty of Medicine, Altindag, 06100, Ankara
Turkey
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/tjps.tjps_7_22
Von Willebrand's disease (VWD) is the most common hereditary bleeding disorder. Due to its frequency, occasionally, patients with this disease apply for rhinoplasty. Classically, desmopressin is used in emergent cases, and it is highly effective. However, the lack of long-term treatment and possible side effects limit the desmopressin treatment. Because the fibrinolytic activity in the nasal mucosa is high, antifibrinolytics such as tranexamic acid are beneficial in all types of VWD. An 18-year-old patient with Type 1 VWD who was operated without facing any complications is presented. Treatment involved 4 days of treatment starting the day before the surgery and healing process was uneventful. Patients with a diagnosis of VWD can be operated successfully with preoperative tranexamic acid treatment and using vasoconstrictor agents and avascular surgery planes. In Type 1 VWD or low von Willebrand's factor cases, tranexamic acid is a safe and successful treatment alternative due to its long-term use, local and systemic applicability, and regional affinity.
Keywords: Bleeding, desmopressin, rhinoplasty, tranexamic acid, von Willebrand's disease
Postoperative epistaxis is a possible postoperative complication for patients undergoing rhinoplasty. Majority of the patients do not need any treatment as it is self-limited.[1] However, in patients with a bleeding disorder, postoperative epistaxis may cause prolonged hospitalization, need for transfusion, poor esthetic outcome, and revision surgery.
Von Willebrand's disease (VWD) is the most common hereditary bleeding disorder with an incidence of 1% in the general population. This disease occurs as a result of a defect in the structure or function of the von Willebrand's factor (VWF) affecting platelet adhesion and FVIII stabilization.[2] High bleeding scores and positive family history in association with VWF levels <30 U/dL are strong diagnostic criteria in all types of VWD; however, no consensus exists on the definitive diagnostic criteria for patients with Type 1 VWD who have VWF levels between 30 and 50 U/dL. The terminology “low VWF” is also used for these patients.[2] Because VWD is relatively common, patients with this disease may arise from time to time among patients who apply for rhinoplasty. However, there is still uncertainty among physicians regarding the perioperative management of patients with Type 1 VWD or low VWF. Treatment regimens using VWF concentrate, antifibrinolytic, and desmopressin have not been optimized in patients, especially with low VWF whose bleeding manifestations are variable.[3]
Case ReportWe presented an 18-year-old girl who had been diagnosed with Type 1 VWD or low VWF at 12 years old [Figure 1],[Figure 2],[Figure 3]. She had experienced several ecchymosis, heavy menstrual bleeding, and intermittent epistaxis that was controlled with tranexamic acid alone. Her FVIII level, VWF: Ag level, VWF: RCo level, and VWF: RCo/Ag ratio were 48%, 36%, 40%, and more than 1, respectively. Her pediatric bleeding score was 4 points. She was also three different times tested for Type 1 VWD. Prophylaxis plan included starting tranexamic acid at a dose of 15 mg/kg every 6–8 h as an oral route, the day before the surgery. Moreover, it was decided to continue this treatment at least for 3 days postoperatively. Nasal packings were removed at the postoperative 3rd day. After treatment for 3 days, due to the absence of bleeding, bruising, or excessive edema, tranexamic acid was discontinued. No complications were encountered afterward.
Figure 2: Postoperative day 2. Note the absence of bruising and excessive edema
Figure 3: Postoperative quarter view of the patient 12 months after the operation Discussion There have been few publications on the rhinoplasty experience in patients with VWD.[4],[5] Patients are generally diagnosed before the surgery. However, some patients are also diagnosed with postoperative epistaxis. Once diagnosed, classically desmopressin is used, and especially in emergent cases, desmopressin is highly effective. However, we thought that desmopressin may not be the standard of care for patients who have already been diagnosed with VWD. Desmopressin can be used in patients with very low VWF levels and can be given in a maximum of two doses (24 h apart). Bleeding may persist up to a few days, and this treatment period may be insufficient when long-term treatment is required. In addition, desmopressin is not preferred to be used in major surgeries because of the risk of tachyphylaxis and hyponatremia.[3]
Since the fibrinolytic activity in the nasal mucosa is high, antifibrinolytics such as tranexamic acid are beneficial in all types of VWD.[2],[3] Although it has previously been shown to be beneficial in reducing bleeding, bruises, and edema in rhinoplasty patients,[6],[7] there is no example of using tranexamic acid in patients with VWD. In type 1 VWD or low VWF cases, tranexamic acid is a safe and successful treatment alternative due to its long-term use, local and systemic applicability, and regional affinity.
Of course, some surgical principles are also of importance to reduce postoperative epistaxis and bruising.[8] The first author prefers open rhinoplasty, as it gives direct visualization of the surgical field, and coagulation of bleeding foci is easier. Dissecting through avascular planes (subperichondrial/subperiosteal) will reduce the risk of bleeding. At certain stages of the surgery (e.g., skin incision and membranous septum dissection), the surgeon has to cut through vascular planes and the injection of vasoconstrictor agents into these areas at the beginning of the operation and waiting for 15 min before the first incision will also reduce bleeding. Nasal subunits without a structural abnormality should not be intervened. The author thought that the maxillary crest is a highly vascularized bony structure and bleeding is common if it is osteotomized. This bleeding is self-limited and may not be threatening in the majority of the rhinoplasty patients but can be problematic in a patient with a bleeding disorder. The surgeon has to keep in mind that it would be technically challenging to cauterize these bleedings as they emerge directly from the bone, and visualization is difficult. Therefore, if there is no deviation that seriously narrows the airway, it will be appropriate not to interfere with the maxillary crest. In the author's experience, as frightening as it seems, bleeding from the lateral osteotomy lines is minimal with proper subperiosteal dissection and the use of narrow-tipped lateral osteotomies. If bleeding is seen from lateral osteotomy incisions, it should be noted that bleeding often originates in the immediate vicinity of the incision, and not deeper. The author also uses 16G IV lines as drains in all patients to reduce postoperative bruising and edema, for the first 24 h.
Rhinoplasty can be planned in patients with VWD, thanks to improved surgical technique and pharmacological treatment options. Standardizing these surgical techniques in all surgeries will also reduce the development of bleeding and bruises. The authors thought that tranexamic acid is an alternative to desmopressin in patients with VWD for prophylaxis. Moreover, with further studies, it may be proven to be a better choice than desmopressin. Success will be achieved if antifibrinolytic treatment is applied in Type 1 VWD or low VWF cases diagnosed with a presurgical period.Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published, and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
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