Cerebrofacial venous metameric syndrome—spectrum of imaging findings

Brinjikji, Waleed; Nicholson, Patrick; Hilditch, Christopher Alan; Krings, Timo; Pereira, Vítor Mendes; Agid, Ronit

doi:10.1007/s00234-020-02362-7

Cited by 20 publications

(29 citation statements)

References 18 publications

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“…Of note, 2 of these neonates presented with clinical-neuroradiologic features consistent with CVMS, a rare craniofacial vascular malformation disorder characterized by a wide spectrum of slow-flow vascular lesions distributed along $1 of the 3 craniofacial metameres, further supporting this theory. 19 Remarkably, 1 neonate also presented with a superior orbital fissure AVF, suggesting that this complex disorder may actually be a continuum potentially affecting .1 vessel type.…”

Section: Discussionmentioning

confidence: 98%

“…[8][9][10][11][12][13][14][15][16] Moreover, a higher prevalence of DVAs has been described in patients with different pathologies and/or genetic conditions. [17][18][19][20][21] Although DVAs are widely described and characterized in adults, they remain under-reported in the pediatric population. Indeed, there are noticeably fewer studies focusing exclusively on DVAs in this age group, especially in the neonatal period.…”

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confidence: 99%

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Neonatal Developmental Venous Anomalies: Clinicoradiologic Characterization and Follow-Up

Geraldo

Messina²,

Tortora³

et al. 2020

AJNR Am J Neuroradiol

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BACKGROUND AND PURPOSE: Although developmental venous anomalies have been frequently studied in adults and occasionally in children, data regarding these entities are scarce in neonates. We aimed to characterize clinical and neuroimaging features of neonatal developmental venous anomalies and to evaluate any association between MR imaging abnormalities in their drainage territory and corresponding angioarchitectural features. MATERIALS AND METHODS: We reviewed parenchymal abnormalities and angioarchitectural features of 41 neonates with developmental venous anomalies (20 males; mean corrected age, 39.9 weeks) selected through a radiology report text search from 2135 neonates who underwent brain MR imaging between 2008 and 2019. Fetal and longitudinal MR images were also reviewed. Neurologic outcomes were collected. Statistics were performed using x 2 , Fisher exact, Mann-Whitney U, or t tests corrected for multiple comparisons. RESULTS: Developmental venous anomalies were detected in 1.9% of neonatal scans. These were complicated by parenchymal/ventricular abnormalities in 15/41 cases (36.6%), improving at last follow-up in 8/10 (80%), with normal neurologic outcome in 9/14 (64.2%). Multiple collectors (P ¼ .008) and larger collector caliber (P , .001) were significantly more frequent in complicated developmental venous anomalies. At a patient level, multiplicity (P ¼ .002) was significantly associated with the presence of $1 complicated developmental venous anomaly. Retrospective fetal detection was possible in 3/11 subjects (27.2%). CONCLUSIONS: One-third of neonatal developmental venous anomalies may be complicated by parenchymal abnormalities, especially with multiple and larger collectors. Neuroimaging and neurologic outcomes were favorable in most cases, suggesting a benign, self-limited nature of these vascular anomalies. A congenital origin could be confirmed in one-quarter of cases with available fetal MR imaging. ABBREVIATIONS: CCM ¼ cerebral cavernous malformation; c-DVA ¼ complicated developmental venous anomaly; cUS ¼ cerebral ultrasound; CVMS ¼ cerebrofacial venous metameric syndrome; DVA ¼ developmental venous anomaly; u-DVA ¼ uncomplicated developmental venous anomaly

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Section: Discussionmentioning

confidence: 98%

mentioning

confidence: 99%

Neonatal Developmental Venous Anomalies: Clinicoradiologic Characterization and Follow-Up

Geraldo

Messina²,

Tortora³

et al. 2020

AJNR Am J Neuroradiol

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“…68 CVMS is an overarching term that encompasses multiple syndromes, the most common of which is Sturge-Weber syndrome. 67 Sturge-Weber syndrome, otherwise known as encephalotrigeminal angiomatosis, is a sporadic congenital neurocutaneous disorder that results from a somatic GNAQ gene mutation. 52 It is considered a variant of CVMS 1 and/or 2.…”

Section: Syndromes With Low-flow Vascular Malformationsmentioning

confidence: 99%

“…Cerebrofacial venous metameric syndrome (CVMS) mirrors CAMS with slow-flow venous vascular malformations instead of high-flow arterial malformations. 67,68 Common slow-flow vascular malformations seen in CVMS include cavernous malformations and developmental venous anomalies of the brain and craniofacial area. 68 CVMS is an overarching term that encompasses multiple syndromes, the most common of which is Sturge–Weber syndrome.…”

Section: Low-flow Vascular Malformations and Associated Syndromesmentioning

confidence: 99%

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Imaging of benign cervicofacial vascular anomalies and associated syndromes

et al. 2021

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Cervicofacial vascular anomalies can result in morbidity, pain, and cosmetic concerns in affected individuals. Each anomaly has its own unique natural history, treatment, and associations with underlying genetic syndromes. For optimal patient care, it is important for the neuroradiologist to accurately recognize and characterize these entities to ensure appropriate treatment and management. In this review, we discuss the general characteristics, classifications, and imaging features associated with the most common vascular anomalies such as hemangiomas, arteriovenous malformations and fistulas, capillary malformations, venous malformations, and lymphatic malformations in the context of associated syndromes. Additionally, we discuss novel imaging techniques that aid in identifying these vascular anomalies.

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Pediatric Dural Arteriovenous Shunts

Zhang¹,

Lv²

2022

Intracranial and Spinal Dural Arteriovenous Fistulas

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Cerebrofacial venous metameric syndrome—spectrum of imaging findings

Cited by 20 publications

References 18 publications

Neonatal Developmental Venous Anomalies: Clinicoradiologic Characterization and Follow-Up

Neonatal Developmental Venous Anomalies: Clinicoradiologic Characterization and Follow-Up

Imaging of benign cervicofacial vascular anomalies and associated syndromes

Pediatric Dural Arteriovenous Shunts

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