PHACES Association: A Neuroradiologic Review of 17 Patients

Oza, Vikash S.; Wang, E.; Berenstein, Alex; Waner, Milton; Lefton, Daniel R.; Wells, Jack A.; Blei, Francine

doi:10.3174/ajnr.a0937

Cited by 74 publications

(50 citation statements)

References 32 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…They have been described as having a predilection for the posterior fossa, especially the fourth ventricle and the cerebellopontine angle cistern. [3][4][5][6][7][8] Other reported sites include the pineal region, the hypothalamus, the hippocampus, and along the cerebellar convexity. 4,5 There is 1 recent case report of diffuse neonatal hemangiomatosis that involved the brain and cervical spinal cord.…”

Section: Discussionmentioning

confidence: 99%

“…9 Other associated findings reported included cerebellar hypoplasia and atrophy, persistent trigeminal artery, periventricular gray matter heterotopia, cerebral AVM in 1 patient, Dandy-Walker complex, and multifocal hepatic hemangiomas. [3][4][5]8,24 Reported examples of intraspinal hemangiomas are even rarer, numbering 5.…”

Section: Discussionmentioning

confidence: 99%

“…3 We would recommend contrast-enhanced brain MR imaging and intracranial MR angiography in this setting. Imaging has also been recommended whenever a cutaneous hemangioma lies over the lumbosacral spine, in perineal hemangiomas with associated urogenital or anal abnormalities, when more than 4 cutaneous hemangiomas are present, and for hemangioma with atypical presentation such as thrombocytopenia.…”

Section: Discussionmentioning

confidence: 99%

“…There are only scattered reports of true infantile hemangiomas involving the central nervous system (CNS). [3][4][5][6][7][8][9][10][11][12] Approximately 60% of infantile hemangiomas occur in the craniofacial region. 13 The lesions are described as superficial, deep, or of combined depth.…”

Section: T He Classification Of Vascular Anomalies Proposed By Mullikmentioning

confidence: 99%

See 3 more Smart Citations

Infantile Hemangiomas Involving the Neuraxis: Clinical and Imaging Findings

Venkataraman¹,

Smith²,

Mulliken³

et al. 2009

AJNR Am J Neuroradiol

View full text Add to dashboard Cite

BACKGROUND AND PURPOSE:The neuroradiology and neurosurgery literature is replete with references to "hemangioma" involving the central nervous system (CNS). However, the number of cases of true infantile hemangiomas in the CNS reported to date is 15. Our purpose was to delineate the definition of infantile hemangiomas, determine their prevalence in the neuraxis, and describe their imaging characteristics and associations in this location.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: T He Classification Of Vascular Anomalies Proposed By Mullikmentioning

confidence: 99%

See 2 more Smart Citations

Infantile Hemangiomas Involving the Neuraxis: Clinical and Imaging Findings

Venkataraman¹,

Smith²,

Mulliken³

et al. 2009

AJNR Am J Neuroradiol

View full text Add to dashboard Cite

show abstract

“…3,4 More recently, attention has been drawn to the incidence of intracranial hemangiomas, especially in the internal auditory canal (IAC) and cerebellopontine angle cistern.…”

Section: Abbreviationsmentioning

confidence: 99%

Enlargement of the Internal Auditory Canal and Associated Posterior Fossa Anomalies in PHACES Association

Meltzer

Robson

Blei

et al. 2015

AJNR Am J Neuroradiol

Self Cite

View full text Add to dashboard Cite

SUMMARY:We noted enlargement of the internal auditory canal in several of our patients with posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, and sternal or supraumbilical defects (PHACES) association and hence evaluated children with PHACES for the presence of an enlarged internal auditory canal and potential associated findings, including infantile hemangioma within the internal auditory canal, to understand the genesis of this enlargement. We reviewed our records to identify children with PHACES association who had been evaluated with MR imaging at our institutions. Imaging was reviewed for abnormal enhancement in the internal auditory canal, internal auditory canal enlargement, cerebellar hypoplasia, prominence of the petrous ridge, and deformity of the calvarium. We raise the possibility of an association between enlargement of the internal auditory canal in PHACES and a generalized malformation of the posterior fossa with cerebellar and calvarial hypoplasia. ABBREVIATIONS:IAC ϭ internal auditory canal; PHACES ϭ posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, and sternal or supraumbilical defects P osterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, and sternal or supraumbilical defects compose the PHACES association.1,2 The regional facial infantile hemangiomas of PHACES association are frequently associated with ipsilateral cerebellar hypoplasia and ipsilateral anomalies of the intracranial internal carotid artery. 3,4 More recently, attention has been drawn to the incidence of intracranial hemangiomas, especially in the internal auditory canal (IAC) and cerebellopontine angle cistern.5-8 The purpose of this study was to document the incidence and imaging appearance of anomalies of the IAC and the potential for associated posterior fossa anomalies in patients with PHACES association. CASE SERIESThe index patient was a 25-year-old woman who presented with chronic hearing loss and a history of a facial hemangioma in infancy. Initial temporal bone CT revealed a funnel-shaped configuration of the prominent left IAC, prominence of the left posterior petrous ridge, hypoplasia of the left occipital bone, and findings consistent with a persistent left stapedial artery (Fig 1). Subsequent MR imaging demonstrated left cerebellar hypoplasia, enlargement of the left IAC, and an asymmetrically prominent left retrocerebellar CSF space (Fig 2). No abnormal enhancement was observed in the IAC or posterior fossa.Subsequent review of clinical data bases at 2 institutions identified 44 patients (37 male, 7 female) diagnosed with PHACES association who underwent diagnostic MR imaging of the brain between 2003 and 2012. Institutional review board approval was obtained from the 2 participating institutions for this retrospective review. A neuroradiologist with 9 years of experience and a pediatric neuroradiologist with 20 years of experience performed a consensus review of MR imag...

show abstract

Central nervous system vascular malformations: A clinical review

Sabayan

Lineback

Viswanathan

et al. 2021

Ann Clin Transl Neurol

View full text Add to dashboard Cite

CNS vascular malformation is an umbrella term that encompasses a wide variety of pathologies, with a wide range of therapeutic and diagnostic importance. This range spans lesions with a risk of devastating neurological compromise to lesions with a slow, static or benign course. Advances in neurovascular imaging along with increased utilization of these advances, have resulted in more frequent identification of these lesions. In this article, we provide an overview on definitions and classifications of CNS vascular malformations and outline the etiologic, diagnostic, prognostic, and therapeutic features for each entity. This review covers intracranial and spinal cord vascular malformations and discusses syndromes associated with CNS vascular malformations.

show abstract

PHACES Association: A Neuroradiologic Review of 17 Patients

Cited by 74 publications

References 32 publications

Infantile Hemangiomas Involving the Neuraxis: Clinical and Imaging Findings

Infantile Hemangiomas Involving the Neuraxis: Clinical and Imaging Findings

Enlargement of the Internal Auditory Canal and Associated Posterior Fossa Anomalies in PHACES Association

Central nervous system vascular malformations: A clinical review

Contact Info

Product

Resources

About