High risk of cerebrospinal fluid leakage in surgery of a rare primary intraosseous cavernous hemangioma of the clivus showing meningeal infiltration: A case report and review of the literature

Abstract

Background: Primary intraosseous cavernous hemangiomas (PICH) of the skull represent an infrequent bone tumor. Although some rare cases of PICHs located in the skull base have been published, to our concern only three cases have been reported in the English literature of PICHs arising within the clivus.

Case description: We present the case of a patient presenting an isolated abducens paresis due to a rare PICH of the clivus showing also an unusual destruction of the inner table as well as infiltration of the dura mater. Due to this uncommon infiltrative pattern of an otherwise expected intraosseous tumor, a cerebrospinal fluid (CSF)-fistula occurred while performing a transnasal biopsy. The patient recovered successfully without need of lumbar drainage or re-surgery. Additionally, intratumoral decompression was sufficient to relief the abducens paresis.

Conclusions: Our case provides new and meaningful information about clinical features as well as growth pattern of these rare clival tumors. We also discuss the importance of knowing these peculiarities before surgery in order to plan the optimal operative management as well as to avoid complications while approaching PICHs localized in such a delicate cranial region.

Keywords: Clivus; cavernous hemangioma; cerebrospinal fluid leakage.

Figure 1

Axial (a-h) and sagittal (i, j, k) reconstructed CT images showing a clear involvement of bone structures within the skull base. It is remarkable that the tumor showed infiltration and destruction of the tabula interna in many sites (arrows), (i, j, k) which corresponds to the extension and adhesion to the dural layer

Figure 2

Axial (a_) and sagittal (s_) contrast enhanced T1-weigthed (ceT1) and T2-weighted MRI images showing a heterogeneous lesion involving the clivus, (a, b, j, k, n, o) sphenoidal bone, (c, d, g, h, k, o) sphenoidal wing, (b, i) as well as extending to the left petrosal apex and cavernous (b, d, g, h, i, m) sinus

Figure 3

MRI angiographic scans show the relationship between the tumor and major vessels of the skull base. The intracavernous and supraclinoidal segment of the left internal carotid was surrounded and compressed by the tumor (c, d, e, g). However, sufficient blood supply to the left medial and anterior brain arteries provided by the right carotid circulation through the Willis circle (e, f, g), as well as a slow tumor growth enabling collateralization, can explain the lack of neurological deficits besides the ones produced by a direct tumor compression of cranial nerves

Figure 4

Microsurgical tumor biopsy performed with neuronavigation (a) through a transnasal transesphenoidal approach. After osteotomy of the esphenoidal sinus (b), the tissue showed a strong tendency to bleed (c). The destruction of the tabula interna and adhesions to the dura made the opening of subarachnoidal space almost unavoidable (d) and muscle (e) was used to close the dural defect. Esphenoidal bone (1), tumor (2), subarachnoidal space (3), and muscle graft (4)