Limb salvage in Christmas disease masquerading as fungating aneurysmal bone cyst

A boy in middle childhood presented with complaints of swelling and pain in his right forearm for 4 months. He had previously undergone left above-elbow amputation due to a suspected malignant tumour in that arm. Radiological imaging revealed a locally aggressive bone tumour of the right distal radius. Two biopsies failed to provide a diagnosis, and in the meantime the lesion became fungating. Consequently, it was decided to perform wide resection together with wrist arthrodesis. Postoperatively, on day 4, the patient developed wound dehiscence and impending compartment syndrome. During revision surgery, a significant quantity of blood clots was removed, without any active bleeders. Due to the uncertain diagnosis, thromboelastography (TEG) analysis was done, which showed a fibrinolytic pattern, and the patient's factor IX level was found to be 4% of the normal value. As a result, a diagnosis of haemophilia B was established. The patient was started on tranexamic acid and factor IX replacement therapy.

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Publication Details

Journal
BMJ Case Reports
Published
2024-12-01
DOI
https://doi.org/10.1136/bcr-2024-262163
Primary Topic
Hemophilia Treatment and Research
Type
article
Field-Weighted Citation Impact
0.00
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article

Limb salvage in Christmas disease masquerading as fungating aneurysmal bone cyst

Tulika Seth, Venkatesan Sampath Kumar, Sandeep Sehrawat
BMJ Case Reports
Hemophilia Treatment and Research
article

Limb salvage in Christmas disease masquerading as fungating aneurysmal bone cyst

Tulika Seth, Venkatesan Sampath Kumar, Sandeep Sehrawat
article en

Abstract

A boy in middle childhood presented with complaints of swelling and pain in his right forearm for 4 months. He had previously undergone left above-elbow amputation due to a suspected malignant tumour in that arm. Radiological imaging revealed a locally aggressive bone tumour of the right distal radius. Two biopsies failed to provide a diagnosis, and in the meantime the lesion became fungating. Consequently, it was decided to perform wide resection together with wrist arthrodesis. Postoperatively, on day 4, the patient developed wound dehiscence and impending compartment syndrome. During revision surgery, a significant quantity of blood clots was removed, without any active bleeders. Due to the uncertain diagnosis, thromboelastography (TEG) analysis was done, which showed a fibrinolytic pattern, and the patient's factor IX level was found to be 4% of the normal value. As a result, a diagnosis of haemophilia B was established. The patient was started on tranexamic acid and factor IX replacement therapy.

BMJ Case ReportsVol. 17(12)
All India Institute of Medical Sciences Raipur (IN), All India Institute of Medical Sciences Bhopal (IN), All India Institute of Medical Sciences (IN)
Good health and well-being
Openalex Percentile: Top 57%
Hemophilia Treatment and Research
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