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Intracranial infantile hemangiomas associated with PHACE syndrome
C D Judd1, P R Chapman, B Koch
1Inland Imaging, Spokane, Washington 99202, USA.
Insights
Infantile hemangiomas rarely occur in the brain. This study reports four cases of orbital infantile hemangiomas with associated intracranial lesions, suggesting a link between these tumors and PHACE syndrome.
Area of Science:
- Neurology
- Pediatrics
- Radiology
Background:
- Infantile hemangiomas are common vascular tumors, typically found in the head and neck.
- Intracranial infantile hemangiomas are rare, with limited case reports in medical literature.
Purpose of the Study:
- To report four cases of orbital infantile hemangiomas with concurrent intracranial lesions.
- To investigate the imaging characteristics and potential association with PHACE syndrome.
Main Methods:
- Retrospective review of imaging studies and medical records of four infants.
- Evaluation of intracranial lesions for location, signal intensity, enhancement, and treatment response.
- Assessment for PHACE syndrome features and literature review.
Main Results:
- Intracranial lesions were located near the internal auditory canal, mirroring orbital hemangioma characteristics.
- All patients exhibited ipsilateral internal carotid artery and/or cerebellar hemisphere hypoplasia.
- Associated head and neck hemangiomas were noted in all cases.
Conclusions:
- Findings support the diagnosis of intracranial infantile hemangiomas.
- A unique radiographic association between PHACE syndrome and intracranial infantile hemangiomas is suggested.
Background And Purpose:
Head and neck infantile hemangiomas are common lesions that are rarely observed in an intracranial location. We report 4 patients with orbital infantile hemangiomas and ipsilateral enhancing intracranial lesions, presumed to be infantile hemangiomas.
Methods:
Imaging studies and medical records of 4 infants with orbital hemangiomas and enhancing intracranial lesions were reviewed. The intracranial lesions were evaluated in terms of their location, signal intensity characteristics, enhancement pattern, and degree of involution following treatment. Additional findings associated with PHACE syndrome were also noted and a literature review of intracranial infantile hemangiomas and PHACE syndrome was also performed.
Results:
The intracranial masses were primarily in or adjacent to the internal auditory canal and demonstrated imaging characteristics and treatment response similar to the ipsilateral orbital lesions. Ipsilateral internal carotid artery hypoplasia, ipsilateral cerebellar hemisphere hypoplasia, and/or other head and neck hemangiomas were present in all patients.
Conclusion:
These cases collectively support the diagnosis of intracranial infantile hemangiomas and suggest a unique radiographic association between PHACE syndrome and intracranial infantile hemangiomas.
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