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Year : 2003  |  Volume : 51  |  Issue : 1  |  Page : 114-115

Spontaneous subdural hematoma in a young adult with hemophilia

Department of Neurosurgery, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi-110029

Correspondence Address:
Department of Neurosurgery, Neurosciences Centre, A.I.I.M.S., New Delhi-110029

 » Abstract 

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.

How to cite this article:
Agrawal D, Mahapatra A K. Spontaneous subdural hematoma in a young adult with hemophilia . Neurol India 2003;51:114-5

How to cite this URL:
Agrawal D, Mahapatra A K. Spontaneous subdural hematoma in a young adult with hemophilia . Neurol India [serial online] 2003 [cited 2023 Mar 21];51:114-5. Available from: https://www.neurologyindia.com/text.asp?2003/51/1/114/1055

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.

  »   Case Report

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.

  »   Discussion Top

Spontaneous acute subdural hematoma is a rare condition and multiple, etiological factors have been identified.[1],[2],[3],[4],[5],[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 abuse[3] 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]  

 » References Top

1.Avis SP. Nontraumatic acute Subdural Hematoma: A case report and review of the literature. Am J Forensic Med Pathol 1993;14:130-4.  Back to cited text no. 1  [PUBMED]  
2.Drake CG. Subdural hematoma from arterial rupture. J Neurosurg 1961;18:597-601.  Back to cited text no. 2  [PUBMED]  
3.Keller TM, Chappel ET. Spontaneous acute Subdural Hematoma precipitated by Cocaine abuse: Case report. Surg Neurol 1997;47:12-5.  Back to cited text no. 3    
4.Matsuyama T, Shmourura T, Okumura Y, Sakaki T. Acute Subdural hematoma due to rupture of cortical arteries. A study of the points and rupture in 19 cases. Surg Neurol 1997;47:423­7.  Back to cited text no. 4    
5.Tatalla A, Mckissodk W. Acute spontaneous Subdural Hemorrhage:an unusual form of cerebro-vascular accident. Neurology 1971;21:19-25  Back to cited text no. 5    
6.Tokoro K, Nakajiwa F, Yaurataki A. Acute spontaneous Subdural Hematoma of arterial origin. Surg Neurol 1989;29:159-63.  Back to cited text no. 6    
7.Tonga G, Jempeska F, Tonga AR, et al. Platelet responsiveness and biosynthesis of thromboxane and prostacyclin in response to in vitro cocaine treatment. Hemostasis 1985;15:1000-7.  Back to cited text no. 7    
8.Biggs F. Recent advances in the management of hemophilia and Christmas disease. Clinics in hematology 1979;8:95-114.  Back to cited text no. 8    
9.Marlinowitz V, Heem M, Tadmov R, et al. Intracranial hemorrhage in patients with hemophilia. Neurosurgery 1986;18:538-41.  Back to cited text no. 9    
10.Eyster ME, Gill FM, Blatt PM, et al. Central nervous system bleeding in hemophiliacs. Blood 1978;51:1179-88.  Back to cited text no. 10  [PUBMED]  
11.Davies SH, Turner JW, Cumming RA, et al. Management of intracranial hemorrhage in hemophilia. Br Med J 1966;2:1627-30.  Back to cited text no. 11  [PUBMED]  
12.Yoshida M, Hayashi T, Kuramoto S, et al. Traumatic intracranial hematoma in hemophiliac children. Surg Neurol 1979;12:115-8.  Back to cited text no. 12    


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