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DOI: 10.1055/s-0042-1743902
Chronic Intraosseous Organizing Hematoma of the Calvarium in the Setting of Coagulopathy: Report of a Case and Review of the Literature
Objective: A calvarial intraosseous pseudotumor (CIP) is a progressive, organizing hematoma contained within the bone and connective tissue of the skull. There is a rare association between these lesions and the hereditary hemophilias, but reports in other coagulopathies are limited.
Methods: We present a case of a premature newborn with CIP in the setting of thrombocytopenia which, to the best of our knowledge, has not been described previously. Additionally, we systematically reviewed PubMed to find all cases of CIP in patients with coagulopathies.
Results: Including the present case, literature review identified 16 cases of CIP in the setting of coagulopathy. All were male with an average age of 20.9 years (range: 2 months–46 years). Fourteen (87.5%) exhibited hemophilia A, one (6.25%) had hemophilia B, and one (6.25%) had laboratory-confirmed thrombocytopenia. Nine patients (56.2%) reported prior head trauma. Thirteen (81.3%) underwent surgical resection after replacement of the appropriate clotting factor. Three of those thirteen (23.1%) developed an epidural hematoma during their hospital stay. Two patients (11.1%) underwent conservative treatment with Factor VIII replacement alone. All fifteen patients who received treatment experienced resolution of their lesions. The present case was not a surgical candidate due his tenuous clinical condition and was the only fatality.
Conclusion: Coagulopathy-related CIP is exceedingly rare. Diagnosis is made via CT scan visualization of bone remodeling, where biopsy is contraindicated given the risk of hemorrhage. Mindfulness of slow-growing calvarial lesions is important in coagulopathic patients, even in the absence of head trauma.
Publication History
Article published online:
15 February 2022
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