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.
