British Journal of Neurosurgery, August 2009; 23(4): 455–457 CASE REPORT Br J Neurosurg Downloaded from informahealthcare.com by SUNY State University of New York at Stony Brook on 10/25/14 For personal use only. Intracranial haemophilic pseudotumor associated with factor VIII deficiency XIAO-JUN WU, HUAI-RUI CHEN, JU-XIANG CHEN, CHENG-GUANG HUANG, XI HAN, YI-CHENG LU & CHUN LUO Department of Neurosurgery, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, China Abstract Haemophilic pseudotumor is a rare complication of haemophilia occurring in 1–2% of patients and is more frequently located is in the long bones of the lower extremities and in the pelvis.. We present the first case of an intracranial haemophilic pseudotumor in a patient with factor VIII deficiency. Key words: hemophilia, pseudotumor, factor VIII deficiency. Clinical details A 12-year-old male patient was admitted with symptoms of headache and seizures. Neurological examination showed no abnormalities except for the malformation of the right parital bone of approximately 3 cm in diameter. Routine laboratory data showed the following results: Red blood cell count, 4.53 6 1012/L (normal range 3.5–5.5); Hemoglobin 96 g/L (normal range 110–160); Hematocrit 28.7% (normal range 37–49); Platelet count 2816109/L (normal range 100–300); Prothrombin time 12.5 s; Activated partial thromboplastin time 55.9 s.Thromboplastin time 14.3 s; Fibrinogen 3.6 (normal range 2–4.5). Plain radiographs and CT of the skull showed irregular erosion of the right parietal bone (Fig. 1). An MRI scan revealed a heterogeneous intracranial lesion in the right parietal region with areas ofisointensity and hyperintensity on T1 weighted images, mainly hypointense on T2 weighted images and a rim of hypointense singanal between the neoplasm margin and the underlying cortex. There was peritumoral oedema and the lesion showed nonhomogeneous enhancement following administration of gadolinium. A craniotomy was undertaken anda dark-colored neoplasm based on the dura was observed with mainly subdural extension along with osteolytic skull changes. The neoplasm was removed totally without difficulty (Fig. 2). Histology demonstrated the neoplasm to be an intracranial haemophilic A pseudotumor. Cerebral hemorrhage on the 2nd postoperative day was observed. Factor VIII analysis showed 21.6% of normal activity (normal range 50–150), leading to the diagnosis of haemophilia associated with factor VIII deficiency. The patient received an intravenous infusion factor VIII in divided doses, which increased his factor VIII level to normal. Further surgery was not required. He was discharged without any new deficits. One month postoperatively his epilepsyhad substantially improved and he remained well 2 years after surgery. Discussion Haemophilic pseudotumor is an unusual complication occurring in only 1% to 2% of patients with severe factor VIII or IX deficiency, which is defined as an encased hematoma that increases in volume progressively by episodes of recurrent hemorrhage, Its more frequent location is in the long bones of the lower extremities often resulting in soft tissue destruction, bone erosion, and neurovascular compromise. Early diagnosis and treatment are essential to decrease morbidity and mortality.1 There are only 5 previously reported cases of lesions located in the skull in patients with with factor VIII/IX deficiency,2 whereas the present patient with factor VIII deficiency had a haemophilic pseudotumor located intracranially. This case shows that indicates that intracranial haemophilic pseudotumor can occur and surgical removal can be performed in this case. Surgical Correspondence: Chun Lo, Department of Neurosurgery, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, China. E-mail: boyluochun@126.com Received for publication 11 November 2008. Accepted 18 January 2009. ISSN 0268-8697 print/ISSN 1360-046X online ª The Neurosurgical Foundation DOI: 10.1080/02688690902774754 Br J Neurosurg Downloaded from informahealthcare.com by SUNY State University of New York at Stony Brook on 10/25/14 For personal use only. 456 X.-J. Wu et al. FIG. 1. Plain radiographs of the skull image showing irregular erosion of the right parietal bone. Computed tomographic scan showing a intracranial heterogeneous high-density area. Computed tomographic scan with bone window setting demonstrating the slightly eroded right parietal bone. FIG. 2. MRI showing a heterogeneous intracranial lesion in the right parietal region with mixed intensity on T1 weighted images, mainly hypointense on T2 weighted images, and a rim of hypointense signal between the neoplasm margin and the underlying cortex, Gadolinium enchancement demonstrates rapid, non-homogeneous enhancement. Petritumoral edema surrounds this neoplasm. Cerebral hemorrhage on the 2nd postoperative day was observed after a CT scan. Micrographs of the lesion showed old blood coagulum with fibrosis. Intracranial hemophilic pseudotumor Br J Neurosurg Downloaded from informahealthcare.com by SUNY State University of New York at Stony Brook on 10/25/14 For personal use only. excision is considered to be the most effective treatment3 and biopsy or partial excision should be avoided to avoid the risk of serios haemorrhage. Surgery for pseudotumors has a high complication rate including massive haemorrhage, vascular and neurologic damage, and infection, so adequate factor replacement and assessment of any factor inhibitors must be performed before treatment. Standard use of blood products, including fresh-frozen plasma or prothrombin complexes, to correct the coagulopathy leads to the success of treatment. Conclusion Intracranial haemophilic pseudotumor should be included in the differential diagnosis for a haemophilic patient presented with headache or epilepsy. 457 Microneurosurgical removal requires careful perioperative management and cooperation with haematologists ad neurosurgeons. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this paper. References 1 Paula HB, Bolton-Maggs K, Pasi J. Haemophilias A and B. Lancet 2003;24:1801–9. 2 Inoue T, Suzuki Y, Nishimura S. Cranial hemophilic pseudotumor associated with factor IX deficiency: case report. Surg Neurol 2008;69:647–51. 3 Rodriguez-Merchan EC. The haemophilic pseudotumour. Haemophilia 2002;8:12–6.