Orthognatic surgery in hemophilia A

Hemophilia is a recessive genetic disorder caused by a mutation on the X chromosome, with variable expression. Females are carriers and males express the disease by mother inheritance. This entity consists of a blood coagulation deficiency due to a decrease level of the anti-hemophilic globulin protein (coagulation factor). In hemophilia A the deficiency affects factor VIII in different stages from mild to severe according to the plasma level of the activity of the coagulation factor. Surgical procedures may develop mayor bleeding complications if inadequate preoperative measures take place. In order to succeed with extensive surgical procedures such as orthognatic surgery, a protocol must be established. We present a case report of a 24-year-old white male with hemophilia A referred to the Maxillofacial Surgery Department at Santiago’s Military Hospital by his actual orthodontist for a surgical evaluation. He presented a dento-maxillofacial dismorfosis with mandibular progenie, left lateral chin deviation and an anteroposterior maxillary deficiency. A combined orthodontic-surgical treatment was planned. Recombinant coagulation factor VIII (Fandhi) and other haemostatic agents, and local haemostatic measures were administered. LeFort I osteotomy and bilateral split sagital ramus osteotomy were carried out without intraoperative or postoperative hemorrhagic complications. A complete surgical and hematological evaluation is shown and a protocol proposal is described.

Conflict of interest: None declared.

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Feb 5, 2018 | Posted by in Oral and Maxillofacial Surgery | Comments Off on Orthognatic surgery in hemophilia A
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