Haematologica 2002; 87:(01)ELT03
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Spontaneous resolution of subdural hematoma in hemophilia
Keechilat Pavithran*, Mathew Thomas**
Department of Hematology and Internal Medicine, Medical College Hospital, Thiruvananthapuram-695011, India


Correspondence: Dr.K.Pavithran. MD, DM, Rose Gardens, 247, Krishnasumam, Kochulloor, P.O.Medical College, Thiruvananthapuram-695011, India. Phone: 91-471-441602. Email: drkpavithran@hotmail.com
Intracranial hemorrhage is a life-threatening complication of hemophilia. The site of intracranial bleeding in hemophilia is about equally distributed between subdural hematoma and intracranial hemorrhage. The prevalence of ICH in hemophiliacs varies from 4-12% with an equal frequency among hemophilia A and B. Surgical evacuation is the treatment of choice. Here we report a case of acute subdural hematoma in an 18-year-old boy with hemophilia A who was managed conservatively.

Intracranial hemorrhage in bleeding disorders may be subdural, subarachnoid or intracerebral and at times occurring in combination. The frequency of bleeding disorders as a cause of intracranial hemorrhage varies among authors, from 1.6 to 18%.1 Risk factors for intracranial bleeding are severity of disease, young age and trauma. Here we report an adolescent boy with moderate hemophilia presenting with spontaneous subdural hematoma.

An 18-year-old male presented with a history of fever, headache and vomiting of 1-week duration. He was a known hemophiliac (factor VIII deficiency) diagnosed at the age of 4 years, but bleeding episodes were only occasional. There was no history of any injury or any drug intake. His brother also had hemophilia. On physical examination he was stuporous and ocular fundi were normal. There was no focal neurological deficit or neck stiffness. Factor VIII level was 3%. CT scan showed an acute subdural hematoma in the temperoparietal region with a mass effect on the right lateral ventricle with a shift of the midline structures to the left. There was no evidence of any parenchymal bleed (Figure 1). He was treated with antiedema measures and infusion of factor VIII concentrate (1500 units). As he showed symptomatic improvement relatives of the patient were not willing for surgical evacuation. So conservative management was continued with factor VIII concentrate and fresh frozen plasma (due to cost considerations) for 5 days. During this period his factor VIII level could be maintained around 30%. Repeat CT scan after 4 weeks showed complete resolution of hematoma. He made a complete recovery without any neurological sequelae.
Intracranial hemorrhage (ICH) is a life-threatening complication of hemophilia. The site of intracranial bleeding in hemophilia is about equally distributed between subdural hematoma and intracranial hemorrhage. Subarachnoid hemorrhage is the least common. The prevalence of ICH in hemophiliacs varies from 4-12%.2,3 There was no significant difference between the prevalence of patients with hemophilia A and B (3.5% vs. 6.3%) and among the age groups in the study by Klinge et al.2 The reported annual incidence of ICH in hemophilia varies according to different authors, from 0.27- 2%.3,4 Even with the improvements in treatment, mortality is still substantial (33-57%).1 Subdural haematoma and subarachnoid hemorrhage have got a better prognosis, with a mortality rate of 10%. In most circumstances, the optimal treatment for acute subdural hematomas is surgical evacuation.5 However, as there was marked improvement with factor replacement therapy alone, surgical evacuation was not attempted in our case. This case demonstrates that in selected cases conservative management is feasible, especially in countries with limited resources.

 

References

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  2. Klinge J, Auberger K, Auerswald G, Brackmann HH, Mauz-Korholz C, Kreuz W. Prevalence and outcome of intracranial haemorrhage in haemophiliacs--a survey of the paediatric group of the German Society of Thrombosis and Haemostasis (GTH). Eur J Pediatr 1999 ;158 Suppl 3:S162-5.
  3. Nelson MD Jr, Maeder MA, Usner D, Mitchell WG, Fenstermacher MJ, Wilson DA, et al. Prevalence and incidence of intracranial haemorrhage in a population of children with haemophilia. The Hemophilia Growth and Development Study. Haemophilia 1999 ;5:306-12.
  4. Martinowitz U, Heim M, Tadmor R, Eldor A, Rider I, Findler G, et al. Intracranial hemorrhage in patients with hemophilia. Neurosurgery 1986;18:538-41.
  5. Yue CP, Mann KS.The surgical management of intracranial hematomas in hemophiliac children. A prospective study. Childs Nerv Syst 1986;2:5-9.