Neuroaxial Infantile Hemangiomas: Imaging Manifestations and Association with Hemangioma Syndromes

Abstract

BACKGROUND AND PURPOSE: Infantile hemangiomas are common lesions in the pediatric population; in rare cases, an infantile hemangioma can be detected along the neural axis. The purposes of our study included determination of the incidence, location, and imaging appearance of neuroaxial infantile hemangiomas and their syndromic association. We also assessed additional features of cerebral and cardiovascular anomalies that may be associated with neuroaxial lesions. MATERIALS AND METHODS:A retrospective cohort study was performed, searching the radiology database for patients with segmental infantile hemangiomas referred for assessment of possible hemangioma syndromes. We retrospectively reviewed brain and spine MR imaging studies, with particular attention paid to neuroaxial vascular lesions, as well as the relevant clinical data. Neuroaxial hemangioma imaging findings were described, and comparison of segmental cutaneous infantile hemangioma location with the imaging findings was performed in patients with confirmed hemangioma syndromes and in patients with isolated skin infantile hemangioma.RESULTS: Ninety-five patients with segmental infantile hemangioma were included in the study, 42 of whom had a hemangioma syndrome; of those, 41 had posterior fossa brain malformations, hemangioma, arterial lesions, cardiac abnormalities, and eye abnormalities (PHACE) syndrome and 1 had diffuse neonatal hemangiomatosis. Neuroaxial involvement was detected in 20/42 patients (48%) with hemangioma syndromes and in no subjects with isolated segmental infantile hemangioma (P , .001). The most common intracranial hemangioma location was within the ipsilateral internal auditory canal (83%).CONCLUSIONS: Many pediatric patients with segmental infantile hemangioma in the setting of hemangioma syndromes, especially those with PHACE, had neuroaxial hemangiomas. This finding may potentially lead to requiring additional clinical evaluation and management of these patients.ABBREVIATIONS: IAC ¼ internal auditory canal; IH ¼ infantile hemangioma; MC ¼ Meckel cave; PHACE ¼ posterior fossa brain malformations, hemangioma, arterial lesions, cardiac abnormalities, and eye abnormalities I nfantile hemangiomas (IHs) are benign vascular tumors common in infants. [1][2][3] IHs express endothelial glucose transporter 1, differentiating them from other pediatric vascular tumors. 4 Unlike congenital hemangiomas, IHs are not

Non‐vascular intracranial lesions in three children with PHACE association

“…9 In PHACE patients, they are often localized to the cerebellopontine angle and internal auditory canal. 5,11,12 Suspected intracranial IHs are biopsied infrequently.…”

“…They are observed with increased frequency in patients with PHACE association 9 . In PHACE patients, they are often localized to the cerebellopontine angle and internal auditory canal 5,11,12 . Suspected intracranial IHs are biopsied infrequently.…”

“…Its most frequent extracutaneous manifestations are cerebrovascular and cardiac malformations, occurring in up to 91% and 67% of patients, respectively 4 . Intracranial IH are also observed 5 . Non‐vascular intracranial lesions have been reported in only two PHACE patients with astrocytomas 6,7 .…”