title: Perioperative management of a patient with May-Hegglin anomaly requiring craniotomy.
abstract: May-Hegglin anomaly (MHA) is a rare type of autosomal dominant platelet disorder associated with mutations in the gene encoding nonmuscle myosin heavy chain 9 (MYH9). It is characterized by the presence of large platelets, leukocyte inclusions, and thrombocytopenia. The bleeding tendency is usually mild, but severe hemorrhages have been reported. This is the first reported case of a patient with MHA who underwent craniotomy for intractable seizure disorder of temporal lobe origin. Patients who have thrombocytopenia have a higher likelihood of developing intraoperative or postoperative intracranial hematoma and bleeding complications. The patient was administered desmopressin (DDAVP) prior to the neurosurgical procedure and had no complications. With this approach, the use of platelet concentrates could be avoided. We discuss the role of DDAVP in MHA and related platelet disorders and review the current literature.
authors.affiliation: Section of Hematology/Oncology, West Virginia University School of Medicine, Morgantown, West Virginia 26505, USA. asehbai@hsc.wvu.edu
authors.forename: Aasim S
authors.initials: AS
authors.lastname: Sehbai
authors.forename: Jame
authors.initials: J
authors.lastname: Abraham
authors.forename: Vidya K
authors.initials: VK
authors.lastname: Brown
mesh_terms: D000328:Adult; D016063:Blood Loss, Surgical / Q000517:prevention & control*; D003399:Craniotomy; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage / Q000627:therapeutic use; D004827:Epilepsy / Q000097:blood / Q000150:complications / Q000601:surgery*; D005260:Female; D006490:Hemostatics / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D010375:Pedigree; D019990:Perioperative Care / Q000379:methods*; D010976:Platelet Count; D017713:Platelet Transfusion; D013921:Thrombocytopenia / Q000097:blood / Q000150:complications / Q000235:genetics / Q000601:surgery*; D016896:Treatment Outcome
chemical_list: D006490:Hemostatics; D003894:Deamino Arginine Vasopressin