Bilateral Facial Capillary Malformation Associated With Eye and Brain Abnormalities

Mazereeuw‐Hautier, J.; Syed, Samira; Harper, John

doi:10.1001/archderm.142.8.994

Cited by 33 publications

(10 citation statements)

References 7 publications

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“…Of those babies who have a PW birthmark that involves the upper eyelid or forehead, roughly 15-52% (depending on size and extent of PW birthmark) will develop leptomeningeal angioma and the neurological and/or ophthalmic dysfunction that is characteristic of Sturge-Weber syndrome (SWS) (Mazereeuw-Hautier et al, 2006, Tallman et al, 1991); the rest remain neurologically asymptomatic. Clinical manifestations of SWS brain involvement include seizures, hemiparesis, visual field deficits and cognitive impairment.…”

Section: Introductionmentioning

confidence: 99%

Use of quantitative EEG in infants with port-wine birthmark to assess for Sturge–Weber brain involvement

Ewen

Kossoff

Crone

et al. 2009

Clinical Neurophysiology

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Objective-Many infants born with a facial port-wine (PW) birthmark will not develop brain involvement of Sturge-Weber syndrome (SWS). Previous studies have shown asymmetry in quantitative EEG (qEEG) correlates with degree of clinical impairment in children and adults with known SWS. We hope to determine if quantitative qEEG can be used as a method to predict which infants are most likely to develop SWS brain involvement on MRI. The current study looks at the ability of qEEG to differentiate between infants with radiographically-demonstrated SWS and those without.Methods-We first performed an observational study of qEEG results on 8 infants with facial PW birthmark (4 had SWS brain involvement). We recorded standard clinical EEGs and then derived a measure of asymmetry. We subsequently validated this threshold through a study of an additional 9 infants with PW birthmark (5 with SWS brain involvement).Results-Quantitative EEG correctly identified infants with SWS brain involvement in all cases in the validation cohort. This technique was at least as good as a pediatric electroencephalographer with extensive experience reading SWS EEGs.Conclusions-This study demonstrates the ability for qEEG to discriminate between those infants with SWS brain involvement and those with neurologically asymptomatic PW birthmark.Significance-This study represents an important step toward the development of a qEEG technique able to predict which infants with PW birthmark will develop SWS brain involvement.

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Section: Introductionmentioning

confidence: 99%

Use of quantitative EEG in infants with port-wine birthmark to assess for Sturge–Weber brain involvement

Ewen

Kossoff

Crone

et al. 2009

Clinical Neurophysiology

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“…V1 : territoire de la branche ophtalmique ; V2 : territoire de la branche maxillaire ; V3 : territoire de la branche mandibulaire Syndrome de Sturge-Weber partielle et surtout complète, et le caractère étendu de l'APF : extension aux autres territoires du trijumeau (V1-V2, V1-V3, V1-V2-V3), extension à l'hémiface controlatérale[5][6][7].…”

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Syndrome de Sturge-Weber

Maruani

2010

La Presse Médicale

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“…In case of such a CM, in addition to an ophthalmological evaluation, a cranial MRI at age 6–12 months is indicated. In patients with unilateral V1 involvement, the risk of SWS is 40–60%, while in patients with bilateral V1 involvement it is nearly 100% .…”

Section: Vascular Malformationsmentioning

confidence: 99%

Vascular anomalies – a practical approach

Theiler

Wälchli

Weibel

2013

J Deutsche Derma Gesell

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SummaryVascular anomalies are common clinical problems (around 4.5% of all patients) in pediatric dermatology. A correct diagnosis is possible on clinical grounds in around 90 % of cases; the remaining patients may require radiologic evaluations (duplex ultrasonography, MRI scan) and, rarely, histology.Vascular anomalies are divided into tumors and vascular malformations. This clear division reflects the different biological behaviors of these two groups. The infantile hemangioma represents by far the most common vascular tumor and is characterized by a typical growth cycle consisting of rapid proliferation, plateau phase, and finally slow regression. The discovery in 2008 of the efficacy of beta blockers in this disease is a therapeutic milestone.Vascular malformations can affect all types of vessels (capillaries, veins, arteries and lymphatic vessels). They usually manifest at birth and grow proportionally with the affected child. Some show marked progression especially during puberty. Considerable progress has been made with innovative interventional therapies in recent years, but surgery remains an important option.Basic knowledge of these diseases is important to every dermatologist in order to be able to counsel and manage affected patients correctly.

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Bilateral Facial Capillary Malformation Associated With Eye and Brain Abnormalities

Cited by 33 publications

References 7 publications

Use of quantitative EEG in infants with port-wine birthmark to assess for Sturge–Weber brain involvement

Use of quantitative EEG in infants with port-wine birthmark to assess for Sturge–Weber brain involvement

Syndrome de Sturge-Weber

Vascular anomalies – a practical approach

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