Orthognathic surgery in a patient with von Willebrand disease: case report

Aim: This study aims to report the perioperative management of a patient with von Willebrand disease (vWd) who underwent orthognathic surgery. The report follows the guidelines of the Case Report Guidelines (CARE) and focuses on the steps taken to prevent bleeding during the surgical procedure. Methods: A 39-year-old female patient with skeletal Class III was treated with maxillary advancement and mandibular setback. Despite normal test results for ristocetin cofactor activity, measures were taken to prevent bleeding, including atraumatic surgical techniques, use of antifibrinolytic medication, induced hypotension during anesthesia, and preparation of blood products for transfusion during trans and postoperative periods if needed. In the end, these measures were not required. Results: The patient did not experience any bleeding during the surgical procedure or postoperative period, demonstrating the effectiveness of the measures taken to manage their blood dyscrasia. Two years after the surgery, the patient had satisfactory aesthetic and functional results and no evidence of relapse. Conclusion: Thus, this case report demonstrates that vWd does not prevent largescale oral and maxillofacial surgeries such as orthognathic surgery as long as proper precautions are taken pre-, intraand postoperatively

Oral Hygiene and Dentition Status in Patients with Congenital Hemorrhagic Disorders: A Comparative Study

Abstract Objective: To compare the status of oral hygiene and dentition in patients with congenital hemorrhagic disorders with their age-matched healthy counterparts. Also, the prevalence of fear of dentists/ dental treatment among these patients was assessed. Material and Methods: This study was performed on children and adults with von Willebrand disease (vWD), hemophilia (A or B), and healthy subjects. Oral health and dentition status was assessed using the simplified oral hygiene index (OHI-S), plaque index, and the decayed, missing, filled teeth (dmft/DMFT) index. One-way ANOVA test was employed to compare the oral hygiene and dentition status of subjects in the three groups. A p-value of <0.05 was considered statistically significant. Results: The DMFT score did not vary significantly between the groups (p>0.05). Higher OHI-S scores and a poor oral hygiene status was observed more in the hemophilia group than the vWD group and healthy controls. A total of 27.3% of the subjects in the vWD group, 18.2% of subjects in the hemophilia group, and no subjects in the healthy group had a fear of dentists or dental treatment. Conclusion: Subjects with vWD had a higher number of carious teeth when compared to the other groups. Poor oral hygiene status was observed in subjects with hemophilia.

Atendimento odontológico em pacientes com hemofilia e doença de von Willebrand / Dental care in patients with hemophilia and von Willebrand disease

Os pacientes com transtornos de coagulação sanguínea constituem um grupo que requer atenção e cuidados especiais na prática odontológica. A hemofilia e a doença de von Willebrand são as mais comuns das coagulopatias hereditárias e devem ser detectadas antes que qualquer tratamento odontológico seja realizado. Uma boa anamnese, associada a um bom exame físico e bucal podem ajudar na detecção dessas patologias. O cirurgião-dentista deve estar preparado para oferecer o tratamento adequado a esses pacientes, o qual depende da severidade da doença e do tipo de procedimento a ser realizado. Procedimentos menos invasivos geralmente não necessitam de cuidados especiais e podem ser realizados rotineiramente, desde que alguns cuidados básicos sejam tomados. Cirurgias e técnicas anestésicas de bloqueio do nervo alveolar inferior devem ser realizadas com maior precaução, a fim de minimizar os riscos de sangramento e outras complicações. O hematologista deve ser consultado sempre que qualquer tratamento invasivo for planejado.

Conduta terapêutica em portador da doença de von Willebrand: relato de caso clínico / Therapeutic management in carriers of von Willebrand disease: a case report

A doença de von Willebrand é a mais comum das doenças hereditárias de coagulação, caracterizada por uma anormalidade quantitativa ou qualitativa do fator von Willebrand, glicoproteína presente no plasma, plaquetas e células endoteliais. Resulta em duplo defeito hemorrágico, caracterizado por tempo de sangramento prolongado e níveis plasmáticos baixos do fator VIII de coagulação. É importante que o cirurgião-dentista conheça essa entidade e os riscos originários da mesma. O trabalho conjunto desse profissional e do hematologista é essencial para a segurança nas abordagens odontológicas em portadores dessa doença. Este artigo relata um caso clínico envolvendo a extração de molares permanentes em portador de von Willebrand do tipo 1, com ênfase na utilização de acetato de desmopressin e ácido tranexâmico para a prevenção de quadro hemorrágico.

von Willebrand's disease is the most commom of the coagulation hereditary disorders and it is characterized by a quantitative and a qualitative abnormality of the von Willebrand's factor, which is a glycoprotein found in plasma, platelets and endothelial cells. It has a double bleeding defect characterized by a prolonged bleeding time and lower plasmatic levels of the clotting VIII factor. It is important that the clinician knows this disease and the risks that it involves. It is essential that dentists and hematologists work together in order to give a secure treatment to von Willebrand carriers. This clinical case describes a dental treatment, which involved permanent molars extraction in a thirteen years old patient, with type 1 von Willebrand disease, emphasizing the use of desmopressin acetate and tranexamic acid in prevention of the hemorragic aspect.

Hemorragia pós operatória em portadores da doença de von Willebrand / Pos operative hemorrhage in patients with von Willebrand's disease

Os autores descrevem dois casos clínicos de pacientes portadores de doença de von Willebrand näo diagnosticada e enfocam os cuidados pré, trans e pós operatórios a serem tomados. Após uma breve revisäo da literatura, concluíram que a anamnese precisa ser valorizada, assim como a inter-relaçäo cirurgiäo-dentista e hematologista no acompanhamento desses pacientes

Hemofilia: aspectos de interesse odontológico / Hemophilia: aspects of dental interest

Os autores abordaram os aspectos gerais da hemofilia. salientando a importância dessa grave doença hereditária. Enfocaram a necessidade do seu conhecimento básico pelo cirurgiäo-dentista, bem como a importância do trabalho multidisciplinar integrado para a manutençäo do equilíbrio dos portadores, por ocasiäo dos tratamentos odontológicos, de rotina ou especializados