Neurology India, Vol. 51, No. 1, Jan-Mar, 2003, pp. 114-115
Spontaneous subdural hematoma in a young adult with hemophilia
D. Agrawal, A. K. Mahapatra
Department of Neurosurgery, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi-110029, India.
Accepted on 14.07.2001.
Code Number: ni03035
We report a case of spontaneous acute subdural hematoma in a 30-year-old man, who was diagnosed with hemophilia during his hospital stay. He developed an extradural hematoma following evacuation of the acute SDH, which was also evacuated. He had a good outcome. Management of such a patient is discussed.
Key Words: Spontaneous subdural hematoma, Hemophilia.
Spontaneous development of an acute subdural hematoma is rare, and the etiology is varied. Hemophilia is the commonest hereditary bleeding disorder but its presentation as acute subdural hematoma is uncommon.
A 30-year-old non-hypertensive and non-diabetic man presented with history of headache and vomiting for 3 days and altered sensorium for 1 day. There was no history of any trauma, fever, drug or alcohol intake. No history suggestive of bleeding diathesis could be elicited. The patient had undergone a colostomy at 11 years of age for blunt trauma, from which he had made an uneventful recovery.
He was unconscious with stable vital signs. His Glasgow coma scale was 8/15 (E1 V2 M5). He had a left hemiparesis (grade 3/5). There was no evidence of any external injury.
A contrast-enhanced computerized tomography (CT) head revealed a large acute subdural hematoma in the right parieto-occipital region with gross midline shift and effacement of cisterns. His biochemical investigation revealed no abnormality. Bleeding and clotting time and prothrombin time were normal.
A right parieto-occipital craniotomy was done and the subdural hematoma was evacuated. The underlying brain appeared normal and there was no evidence of any AVM or any other vascular lesion at the operative site. There was diffuse oozing of blood in the operative field and hemostasis was achieved with difficulty. He regained consciousness on the first postoperative day. Cerebral angiography revealed no evidence of any vascular lesion. On the fourth postoperative day he became drowsy. A CT scan revealed a large extradural hematoma. Blood tests for coagulopathy showed no abnormality. Patient was reoperated and the extradural hematoma was evacuated. Diffuse oozing was again noticed at the operative site and hemostasis was acheived with difficulty.
He improved again after the second surgery. Coagulation profile showed Plasma Thromboplastin time (PTT) to be severely deranged. Factor VIII levels were found to be 2.7% of normal and a diagnosis of Hemophilia was made. He was given cryoprecipitate and fresh frozen plasma, and he made a good recovery.
Spontaneous acute subdural hematoma is a rare condition and multiple, etiological factors have been identified.1-6 Few of the common conditions that can lead to acute SDH are hypertension,5 arterial bleeding from an aneurysm,2,4,5 bleeding as a result of anticoagulant therapy, and bleeding and coagulation disorders.7 Hemophilia can cause spontaneous acute SDH. Rarely, acute subdural hematoma can be a result of cocaine abuse3 or due to cocaine-induced vasculitis.
Hemophilia is the most common hereditary bleeding disorder, with an incidence of 0.7-0.8/1 0,000.8 Classic hemophilia A is characterized by an inherited defect in Factor VIII while Factor IX deficiency leads to hemophilia B (Christmas disease). Intracranial hemorrhage is a rare complication of hemophilia, with frequency of about 2.2-7.8%,9 and a mortality of 34%.10
Diagnosis of hemophilia A is made on the basis of Factor VIII assay and depending on the activity, may be classified as "mild", "moderate" or "severe." Severe cases have less than 1% of the Factor and bleed spontaneously into joints and muscles. Moderately deficient patients have levels of 2% to 5%, while mildly affected patients have 7% to 15%. The moderate hemophiliacs have no spontaneous bleeding, but usually bleed after minor or major trauma. In mild hemophiliacs greater trauma is necessary for bleeding.
Once diagnosis is made, indications for surgery should be no different for non-hemophiliacs suffering from intracranial hematoma.11 However, operation must follow adequate restoration of clotting deficiency by Factor VIII replacement. Factor VIII, in varying concentrations, is found in FFP, cryoprecipitate and Factor VIII concentrate. One ml of FFP contains 1 IU of clotting factor activity, while cryoprecipitate contains between 80 and 100 IU in a volume of 25 ml. Factor VIII concentrate contains between 250 and 2000 IU of activity in a reconstituted volume of 25 ml.
The desired level of replacement of clotting factor for intracranial surgery is controversial. A level of 30% to 50% maintained for 10-14 days is considered to be adequate.10,12 However, others recommend 100% replacement during surgery, and the level is then maintained at 50% to 75% in the postoperative period, up to 2 weeks.11
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