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Haemophilic Pseudotumor a rare differential for swelling of the great toe A case report.

ANAND A

Abstract


Haemophilic pseudotumor is a rare complication in haemophiliacs with high recurrence rates. Involvement of the bones prone for trauma is common. Early surgical                   intervention with adequate factor cover proves beneficial in these patients. An 18-year old male with haemophilia A (negative for factor VIII inhibitor assay), presented with a swelling of the left great toe, one year following trauma. It was diagnosed to be a pseudotumor of the left first distal phalanx based on clinical and radiological findings. Amputation of the great toe at the level of first inter-phalangeal joint was           performed under factor cover. Post operatively, he was found to have a positive factor VIII inhibitor assay, which warranted increased factor VIII transfusions. 2 years following this, the patient presented with one month history of rapidly increasing swelling of the left great toe. It was a recurrence. 1st ray  amputation was performed under factor cover with no post-op complications. This case reinforces that pseudotumor should be considered as one of the differentials for expansile lytic lesions of small bones in the background of haemophilia and suggests close follow up of patients with positive inhibitor assay, considering the high recurrence rate in such patients and the invasiveness of the condition.

 


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References


Ahlberg AK. On the natural history of hemophilic pseudotumor. J Bone Joint Surg Am. 1975; 57 :1133–6.

Heim M, Martinowitz U. Pseudotumours in patients with haemophilia. In: Lee CA, Berntorp EE, Hoots WK, Aledort LM, editors. Textbook of Hemophilia. Oxford, UK:

Blackwell Publishing Ltd; 2007.

A. Pakala, J. Thomas and P. Comp, "Hemophilic Pseudotumor: A Case Report and Review of Literature,"International Journal of Clinical Medicine, Vol. 3 No. 3, 2012, pp. 229-233.

Zhai J, Weng X, Zhang B, Peng H, Bian Y, Zhou L. Surgical management of hemophilic pseudotumor complicated by destructive osteoarthropathy: Blood Coagul Fibrinolysis. 2015 Jun;26(4):373–7.

Starker L. Knochenusur durch ein hamophiles subperiostales hamatom. Mitt Med Chir. 1918; 31 :381.

Steel WM, Duthie RB, O’Connor BT. Haemophilic cysts. Report of five cases. J Bone Joint Surg Br. 1969; 51 :614–26.

Gilbert MS. The hemophilic pseudotumor. Prog Clin Biol Res. 1990; 324 :257–62.

Heeg M, Smit WM, van der Meer J, van Horn JR. Excision of a haemophilic pseudotumour of the ilium, complicated by fistulation. Haemophilia. 1998;4 :132–5.

Rodriguez-Merchan EC. The haemophilic pseudotumour. Haemophilia. 2002;8 :12–6.

Brant EE, Jordan HH. Radiologic aspects of hemophilic pseudotumors in bone. Am J Roentgenol Radium Ther Nucl Med. 1972;115:525-39

Fechner RE, Mills SE. Atlas of tumor pathology: tumors of the bone and joints. 3rd ed. Washington, DC: Armed Forces Institute of Pathology; 1993.

Gaary E, Gorlin JB, Jaramillo D. Pseudotumor and arthropathy in the knees of a hemophiliac. Skeletal Radiol. 1996;25:85-7.


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