Hemostatic Management in Pediatric Patients With Type I von Willebrand Disease Undergoing Oral Surgery: Case Report and Literature Review

Von Willebrand disease (vWD) (Online Mendelian Inheritance in Man 193400) is a hematologic condition characterized by reduced platelet adhesiveness linked to factor VIII (FVIII) and von Willebrand factor (vWF) deficiencies.(1,2) vWF, otherwise known as factor VIII-related protein is a large glycoprotein synthesized by endothelial cells and megakaryocytes, and represents the major component of the FVIII-vWF multimeric complex. 3 vWF has bridging molecular functions between vessel walls and platelets. vWF level can be measured by immunoassay as plasmatic levels of vWF antigen (vWF: Ag).(4,5) A clinical classification of the different vWD subtypes has been developed according to the levels and patterns of the vWF antigen multimers.(6) Type I vWD represents 50% to 75% of cases and is characterized by quantitative reduction of all multimers, divided into autosomal dominant and recessive forms, with the protein complex FVIII/vWF appearing qualitatively normal.(7) An important clinical variability in the vWD type is observed, with the vWF plasmatic half-time modulating the vWD phenotype, and mild, moderate or severe type I vWD has been described.(8) The most severe reduction is observed in homozygous patients. Type II vWD is divided into IIA, IIB, IIC, IIM, and IIN forms and is characterized by qualitative defects of FVIII/vWF complex.(1) Many allelic variants of subtypes IIA, IIB, and III have been described, showing the genetic heterogeneity of the condition.(1) Type III vWD represents the most severe and rare form of the disease with very low or undetectable levels of vWF.(9) The genetic mutations involved in type 3 vWD exhibit high variability and consist mainly of large partial vWF gene deletions, with insertions resulting in frame shift mutations and splice site mutations.(10) vWD corresponds to the hemorrhagic risk during oral surgery and requires hematologic conditioning in the context of a multidisciplinary team that must include the pediatrician, pediatric dentist, radiologist, hematologist, anesthesiologist, and oral maxillofacial surgeon.(11) However, few cases of pediatric oral surgery in a vWD context have been reported.(12) In the present report, we describe the case of a young girl with vWD type I and 2 impacted anistrophic cone-shaped mesiodens in the premaxilla. Completed by a review of the published data, the aims of the present clinical report are to highlight the importance of the multidisciplinary management consisting of careful hematologic conditioning, atraumatic surgical treatment, and clinical follow-up.