Samira Sadigh Batha, Nazanin Nasr, Nasser Kaviani
With meticulous multidisciplinary planning, appropriate perioperative factor replacement, and careful intra- and postoperative management, safe and effective dental rehabilitation can be achieved in pediatric patients with Type 3 VWD.
BACKGROUND: Type 3 von Willebrand disease (VWD) is the most severe and rare form of this inherited bleeding disorder, characterized by near-complete absence of von Willebrand factor (VWF) and markedly reduced Factor VIII activity. These patients are at high risk of excessive bleeding during invasive procedures, including dental treatment.
CASE PRESENTATION: A 4-year-old boy with a confirmed diagnosis of Type 3 VWD presented with intermittent pain in the maxillary left lateral incisor, without clinical or radiographic signs of an abscess. Comprehensive dental rehabilitation was performed under general anesthesia. Treatment consisted of pulpotomy and placement of stainless-steel crowns for primary molars, as well as pulpectomy and resin composite restorations for primary incisors. Hemostatic management included preoperative and postoperative pdVWF/F VIII concentrate administration in consultation with a hematologist.
CONCLUSION: With meticulous multidisciplinary planning, appropriate perioperative factor replacement, and careful intra- and postoperative management, safe and effective dental rehabilitation can be achieved in pediatric patients with Type 3 VWD.