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Hereditary intraosseous vascular malformation of the craniofacial region: imaging findings

Hacettepe University Hospitals, ¹ Plastic and Reconstructive Surgery, Departments of ² Radiology and ³ Nuclear Medicine, 06100, Samanpazari, Ankara, Turkey

View larger version (104K): [in a new window]   Figure 1. (a) Direct lateral and (b) anteroposterior radiographs show expansion of all craniofacial bones, rarefaction, coarse trabecular pattern and "soap bubble" appearance (Case 1).

View larger version (85K): [in a new window]   Figure 2. (a) Axial CT sections in bone settings at the level of foramen magnum shows lytic expansion of the maxilla and paranasal bones, obliteration of the nasal cavity and mastoid cells on the left side and severe exophthalmia due to expansion of the bony orbit on the left. (b) CT section at the level of parietal bones shows marked increase in the diploic space and patchy cavern-like lucent areas (Case 1).

View larger version (111K): [in a new window]   Figure 3. (a) Coronal T1W cranial MRI demonstrates high signal intensity of the involved cranial bones with nodular cavern-like low signal intensity areas on the frontal bone and posterior part of the parietal bone. Involvement of the maxilla and mandible is also seen. (b) On post-contrast image of the same plane demonstrates contrast enhancement of these cavern-like nodular areas. Contrast enhancement of the involved maxilla and mandible is also seen (Case 1).

View larger version (130K): [in a new window]   Figure 4. Axial T2W cranial MR section at the level of centrum semiovales demonstrates high signal intensity characteristic of the involved cranial bones and indentations of expanded bones on brain parenchyma (Case 1).

View larger version (61K): [in a new window]   Figure 5. (a) Right external carotid arteriogram shows enlargement of the external carotid artery and its branches, multiple areas of abnormal staining of the craniofacial bones and (b) on late phase images, multiple areas of pooling of the contrast material (Case 1).

View larger version (80K): [in a new window]   Figure 6. 1st and 10th min anterior dynamic images with semi-in vivo labelled red blood cells by technetium-99m pertechnetate demonstrate accumulation of the radioactivity in multiple craniofacial bones mainly in the left orbital, maxillary and mandible regions.

View larger version (150K): [in a new window]   Figure 7. Haematoxylin-eosin stained pathological specimen (mandible, Case 2) demonstrates innumerable varying calibre vascular channels within bone tissue, with thinning of and decreased number of bony trabecula. The walls of the blood vessels were usually very thin, single cell layer and no muscular coat. (MB, mandibular bone; ICVC, innumerable calibre vascular channels).

View larger version (64K): [in a new window]   Figure 8. (a) Right external carotid arteriogram shows enlargement of the external carotid artery branches supplying the involved maxillofacial bones, abnormal staining of the involved bones and (b) contrast pooling on late phase images (Case 2).

View larger version (138K): [in a new window]   Figure 9. Coronal reconstruction of the CT scan demonstrates severe expansion of the maxillae and zygomatic bones, obliteration of nasal cavity, obliteration of maxillary sinuses due to secretions, and decrease of the orbital volumes (Case 3).

View larger version (98K): [in a new window]   Figure 10. (a) Axial T1W MRI of the mandible shows mixed signal intensity of the expanded and deformed mandible. (b) On post-contrast image, marked contrast enhancement is seen (Case 3).