A 4-Week-Old Female Infant with Type 3 von Willebrand Disease Presenting with Nosebleeds and Uncontrolled Bleeding Following Surgical Frenectomy: A Clinical Case.

Abstract

BACKGROUND Congenital von Willebrand disease can be autosomal recessive or dominant and is classified into types 1, 2, and 3, based on laboratory findings. Severe von Willebrand factor deficiency (type 3 von Willebrand disease) is a rare, congenital deficiency of plasma coagulation factor, the symptoms of which can manifest as early as during the first weeks of a child's life. This report describes the case of a 4-week-old female infant with congenital von Willebrand disease presenting with nosebleeds and uncontrolled bleeding following surgical frenectomy. CASE REPORT A 4-week-old female newborn experienced prolonged bleeding episodes, repeated nosebleeds, and gastrointestinal bleeding following the procedure of inferior labial frenulum cutting (frenectomy). During hematology diagnostics at the neonatal ward, the cause of the described manifestations was identified. In the tested parameters of the coagulation system, the activity of von Willebrand factor in the patient was <1% (below detectable levels). In addition, a secondary decrease in the child's serum FVIII activity (2.1%) was observed. We confirmed type 3 von Willebrand disease. CONCLUSIONS Although type 3 of the disease is an extremely rare disorder within the coagulation cascade, it is important to be aware of the symptoms that can signal a bleeding diathesis, also in children in the first weeks of life. In every patient, regardless of age, in the presence of bleeding and abnormalities in the results of hemostasis system tests, plasma coagulation disorders should be investigated.