Article Figures & Data
Figures
- FIG 1.
Segmental IH. A, A 3-month-old girl with PHACE syndrome. Clinical photograph shows a bilateral segmental IH in an S1 distribution predominantly and with minimal S2 involvement. B, Diagram of the Haggstrom classification, used for clinical assessment of superficial IH distribution. It divides the face on the 4 following segments: S1 = frontotemporal, S2 = maxillary, S3 = mandibular, S4 = frontonasal.
- FIG 2.
Intracranial infantile hemangioma. A and B, A 1.5-month-old girl with segmental IH of the right face and PHACE syndrome. A, Axial T2-weighted image shows an extra-axial T2-hyperintense mass in the right cerebellopontine angle cistern (small arrow) and a similar lesion in the enlarged right MC (arrowhead). A long arrow points to an asymmetrically small right cerebellar hemisphere, which is an additional common manifestation of PHACE syndrome. Both lesions reveal diffuse enhancement on postcontrast imaging and markedly increased perfusion on arterial spin-labeling imaging (B). C, A 3-month-old girl with right orbital segmental IH and PHACE syndrome. Axial postcontrast T1-weighted image with fat suppression shows linear enhancement in the right IAC (large arrow) and linear enhancement of the cochlear basal turn (arrowhead). D, A 2-month-old girl with segmental IH of the right face and PHACE syndrome. Coronal postcontrast T1-weighted image with fat suppression shows a dural base extra-axial enhancing mass in the right frontal lobe undersurface (arrowhead). Additional IHs are demonstrated in the right face (large arrow) and the right skull base and inferior orbit (small arrow).
- FIG 3.
Intracranial infantile hemangioma. A and B, A 2-month-old girl, born with a very large segmental IH of the forehead. A, Clinical photograph shows an IH in the S4 segment (according to the Haggstrom classification). B, Axial postcontrast T1-weighted image with fat suppression of the same patient shows a lobulated enhancing interhemispheric mass (arrow). A superficial forehead IH is indicated by arrowheads. C, A different 2-month-old girl with right facial IH and PHACE syndrome. Coronal postcontrast T1-weighted image with fat suppression shows focal leptomeningeal enhancement along the right tentorium (arrowhead). D, A 1.5-month-old boy with left facial IH. Axial postcontrast T1-weighted image with fat suppression shows enhancing hemangiomas in the left cerebellopontine angle cistern (white arrow) and in the enlarged left MC (small white arrowhead) and enhancing vascular tissue along the lateral orbital wall (black arrow), which seems to communicate between the intra- and extracranial components.
- FIG 4.
Paraspinal IH. A 2-month-old girl with face and neck segmental IH. Coronal (A) and axial (B) postcontrast T1-weighted images with fat suppression show extensive bilateral enhancing paraspinal masses along the thoracic spine (arrows), with extension into the spinal canal and focal cord compression (arrowhead).
- FIG 5.
A, Asymmetrical enlargement of Meckel’s cave in patients with PHACE syndrome, an illustration showing a relationship between skull base hemangioma and adjacent nerves and vascular structures. Modified from a previously published image.33 B, Posterior fossa abnormality in patients with PHACE syndrome. A 6-week-old girl with a left facial IH. An axial T2-weighted image shows an enlarged retrocerebellar CSF space (black arrow), an asymmetrically small left cerebellar hemisphere (white arrow), and a small posterior vitreous coloboma in the left globe (arrowhead), which is another infrequent feature of PHACE syndrome. C, Arteriopathy of PHACE syndrome. A 6-month-old girl with a right-face IH and PHACE syndrome. Coronal TOF-MRA reconstructed image shows the diffusely small caliber of intracranial right ICA (arrow). Fig 5A courtesy of Malhotra, A., Tu, L., Kalra, V.B. et al. Neuroimaging of Meckel’s cave in normal and disease conditions. Insights Imaging 9, 499–510 (2018). https://doi.org/10.1007/s13244-018-0604-7.
Tables
- Table 1:
Characteristics of subjects with IH as part of a hemangioma syndrome compared with isolated IH
Hemangioma Syndrome (n = 42) Isolated Superficial IH (n = 53) P Value Age (median) (IQR) (mo) 2 (3–2) 2 (3-2) .53 Female sex (%) (n) 74% (31) 86% (46) .12 Side/location of cutaneous IH (%) (n) .78 Right face only 45% (19) 40% (21) Left face only 33% (14) 41% (22) Bilateral face 14% (6) 15% (8) Other 7% (3) 4% (2) Facial segment (%) (n) S1 65% (27) 30% (16) .002 S2 50% (21) 66% (35) .14 S3 43% (18) 28% (15) .19 S4 21% (9) 4% (2) .010 Multiple facial segments 57% (24) 34% (18) .037 All facial segments 14% (6) 0% (0) .006 Intracranial IH (%) (n) 45% (19) 0% (0) <.001 Paraspinal IH (%) (n) 12% (5) 0% (0) .015 MC enlargement (%) (n) 67% (28) 0% (0) <.001 Posterior fossa abnormality (%) (n) 48% (20) 0% (0) <.001 Cardiovascular abnormalities (%) ((n) 95% (40) 0% (0) <.001 Note:—IQR indicates Interquartile range.
Neuroaxial IH Location (n = 95) Reader 1 Reader 2 Reader 3 κ P Value Consensus IAC 15 15 15 1.00 <.001 15 Cochlea 3 4 3 0.85 <.001 3 Cerebellopontine angle 5 5 5 1.00 <.001 5 MC 2 2 2 1.00 <.001 2 Cavernous sinus 2 2 2 1.00 <.001 2 Pterygopalatine fossa 1 1 1 1.00 <.001 1 Vidian canal 1 1 1 1.00 <.001 1 Leptomeningeal 10 10 8 0.89 <.001 10 Dural base 4 4 4 1.00 <.001 4 Intra-/paraspinal 5 5 5 1.00 <.001 5
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