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. Am.

May-Hegglin and other Platelet Dysfunctions as Complications to Compartment Syndrome: A case report

“…Though it is rare for a MHA patient to develop severe bleeding intraand post operatively, the skilled foot and ankle surgeon should be aware of the risk of bleeding requiring transfusions. 5 Desmopressin acetate (DDAVP), is a synthetic vasopressin analogue that has been used perioperatively in patients with MHA. It is an altered form of vasopressin in which deamination of hemicysteine at position 1 and substitution of Darginine for L-arginine at position 8 has occurred.…”

“…5 Sehbai, et al, reported a case where 34-year old woman with known MHA underwent a craniotomy secondary to an intractable seizure disorder since childhood. 5 After an extensive family history, past medical history of the patient, and extensive workup which included; magnetic resonance imaging (MRI) of the brain, positron emission tomography (PET) scan, and 24 hour video EEG, the woman underwent craniotomy and resection of the temporal lobe foci of seizure activity. She was admitted one day prior to surgery and transfused with 6 units of platelets, and one hour before surgery was given DDAVP.…”

“…5 Chabane, et al, reported a 24 year old female that was diagnosed with severe thrombocytopenia after giving birth. She was later diagnosed with MHA.…”

Epstein Syndrome Presenting as Renal Failure in Young Patients

“…[5,10] Thrombocytopenia in these patients usually does not respond to treatment with corticosteroid or splenectomy. [11,12] Reduction of GP1b/IX/V in large platelets may contribute to bleeding diathesis, but the risk of bleeding is not high. [13] Renal involvement typically presents with variable degrees of proteinuria or microscopic hematuria in young individuals, although patients can also present with severe renal impairment and histological findings of global glomerulosclerosis, interstitial fibrosis, and marked tubular atrophy.…”

May Hegglin Anomaly with R1339X Masquerading as Idiopathic Thrombocytopenic Purpura And Dengue Fever (A Presentation of Family)

“…A genetic International Journal of Hematology Research neutrophil and monocytes [2] . Clinical manifestations of MHA vary from mild to severe bleeding, but the latter tends to be unusual [3] . A rare disorder, it is often misdiagnosed and can lead to inappropriate administration of drugs.…”