# Orthognathic Surgery in Patients With Von Willebrand’s Disease: A Report of Four Cases and Literature Review

**Authors:** Marika Sato, Hayato Hamada, On Hasegawa, Yoko Kawase-Koga, Daichi Chikazu

PMC · DOI: 10.7759/cureus.57305 · Cureus · 2024-03-31

## TL;DR

This paper reports on four patients with Von Willebrand’s disease who safely underwent orthognathic surgery with proper preoperative management.

## Contribution

The study highlights the importance of preoperative screening and multidisciplinary care for orthognathic surgery in patients with VWD.

## Key findings

- Three out of four patients had no perioperative complications with Factor VIII/VWF concentrate use.
- One patient with type 3 VWD experienced laryngeal edema and epistaxis.
- Two patients were diagnosed with VWD during preoperative screening.

## Abstract

Von Willebrand’s disease (VWD), characterized by quantitatively or qualitatively abnormal von Willebrand factor (VWF), is the most common inherited bleeding disorder. There is limited evidence of treatment using orthognathic surgery in patients with VWD. This report focuses on four patients with VWD who underwent orthognathic surgery and received Factor VIII/VWF concentrates (Confact F) preoperatively. One patient with type 3 (severe) VWD underwent delayed extubation owing to laryngeal edema and exhibited epistaxis thereafter. No perioperative complications were observed in any of the other patients. Two of the four patients were diagnosed with VWD during preoperative screening. Most young adults do not experience general anesthesia and, therefore, may not have undergone blood tests at a hospital. Thus, preoperative screening and adoption of a multidisciplinary approach to orthognathic surgery is important in patients with bleeding disorders such as VWD. Close communication between anesthetists, surgeons, and hematologists is essential to ensure effective management during the perioperative period.

## Linked entities

- **Proteins:** VWF (von Willebrand factor)
- **Diseases:** Von Willebrand’s disease (MONDO:0024574), VWD (MONDO:0024574)

## Full-text entities

- **Genes:** VWF (von Willebrand factor) [NCBI Gene 7450] {aka F8VWF, VWD}
- **Diseases:** laryngeal edema (MESH:D007819), inherited bleeding disorder (MESH:D025861), VWD (MESH:D014842), epistaxis (MESH:D004844), bleeding disorders (MESH:D006470), type 3 (severe) VWD (MESH:D056729)
- **Chemicals:** Confact F (-)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

4 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11059190/full.md

## References

20 references — full list in the complete paper: https://tomesphere.com/paper/PMC11059190/full.md

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Source: https://tomesphere.com/paper/PMC11059190