We report a child presenting with a triad of congenital third nerve palsy, cerebellar hypoplasia and facial capillary hemangioma. [ncbi.nlm.nih.gov]

This review highlights the diagnostic criteria for PHACE syndrome, the imaging workup for extracutaneous involvement, the treatment of infantile hemangioma, and the importance of a multidisciplinary approach in the management of these patients. [ncbi.nlm.nih.gov]

Cardiac and hypercoagulable workups were negative. Stroke etiology was determined to be PHACE syndrome. Conclusions: Neurocutaneous syndromes are typically diagnosed in childhood and can be a rare cause of stroke in young. [n.neurology.org]

In this case, a full-term 5-kg infant with eye swelling and a facial hemangioma presented for a sedated echocardiogram and MRI of the brain and neck for workup of PHACE syndrome. [journals.lww.com]

Treatment modalities included systemic and intralesional steroids for cutaneous and airway hemangiomas, submucosal resection and laryngotracheal reconstruction for airway hemangiomas, repair of aortic coarctation, and laser treatment of cutaneous hemangiomas [ncbi.nlm.nih.gov]

The child may also need other treatments, depending on which other organs or structures are involved. PHACE syndrome requires evaluation and treatment by a multidisciplinary team of experts. [cincinnatichildrens.org]

Standard Therapies Treatment The treatment of PHACE syndrome is directed toward the specific symptoms that are apparent in each individual. Treatment may require the coordinated efforts of a team of specialists. [rarediseases.org]

Prognosis in this condition depends primarily on cardiovascular and cerebral artery complications associated with the syndrome. [ncbi.nlm.nih.gov]

Prognosis The prognosis depends on the severity of the clinical signs associated with the cerebral and arterial anomalies. Neurological sequelae are frequent. The documents contained in this web site are presented for information purposes only. [orpha.net]

Cardiovascular abnormalities may require early surgical intervention.[10] Differential Diagnosis Sturge-Weber syndrome Infantile hemangioma Visceral hemangioma Wyburn-Mason syndrome LUMBAR syndrome Prognosis The prognosis is variable. [statpearls.com]

The prenatal presence of 2 PHACE criteria led to the suspicion of this syndrome, and prenatal diagnostic criteria might be developed to improve information regarding the prognosis of cerebellar malformations. References 1. [journals.sagepub.com]

Further research is needed to determine possible etiologies, optimal evaluation, and outcomes. 2006 Wiley-Liss, Inc. [ncbi.nlm.nih.gov]

Stroke etiology was determined to be PHACE syndrome. Conclusions: Neurocutaneous syndromes are typically diagnosed in childhood and can be a rare cause of stroke in young. [n.neurology.org]

Grimmer;Tracey Lewis;Denise Metry;Ilona Frieden;Francine Blei;Hulya Kayserili;Beth Drolet;Pinar Bayrak-Toydemir; Author Information Checking for direct PDF access through Ovid Abstract PHACE syndrome (OMIM #606519) is a neurocutaneous syndrome of unknown etiology [insights.ovid.com]

Summary Epidemiology This association of manifestations is rare and less than 100 cases have been reported in the literature so far. The syndrome is much more common in females than in males (8:1). [orpha.net]

(See "Infantile hemangiomas: Epidemiology, pathogenesis, clinical features, and complications" .) (See "Infantile hemangiomas: Evaluation and diagnosis" .) [uptodate.com]

Epidemiology PS is more prevalent in the Caucasian and Hispanic populations. [statpearls.com]

Tasnádi Epidemiology and etiology of congenital vascular malformations Sem Vasc Surg, 6 (1993), pp. 200-203 [52.] E. Quecedo, M.P. Gil-Mateo, V. Pont, M.I. Febrer, A. [analesdepediatria.org]

We made an epidemiological investigation in our hospital, concerning the collection of haemocultures, because in a short period of time, from 255 haemocultures, 33 positive haemocultures were reported, 11 of them being positive with the same germs: aerobic [biochemia-medica.com]

The high prevalence of arterial anomalies in this cohort has implications for clinical management and future research regarding the pathophysiology of PHACE. [ncbi.nlm.nih.gov]

1] Approximately, 20 % of children with facial hemangioma will develop one of the abnormalities of PS.[4]The risk of extracutaneous abnormalities increases with increasing hemangioma size and when the hemangioma involves multiple facial segments.[5] Pathophysiology [statpearls.com]

The rarity of the syndrome, the lack of a unifying pathophysiologic mechanism, and the marked variety of clinical and imaging manifestations all lead to potential controversy. [ajnr.org]

Understanding the pathophysiological link between cutaneous infantile hemangiomas and the cerebral vasculopathy of PHACES may help to elucidate the underlying cause of the neurocutaneous disorder. [ahajournals.org]

As children with PHACE syndrome are frequently accompanied with cerebrovascular anomalies and at a risk of cerebral hypoperfusion, prevention of cerebral hypoperfusion is crucially important during general anesthesia. [ncbi.nlm.nih.gov]

Management and treatment Management should be multidisciplinary with the aim of stabilising the cardiac and neurological manifestations and preventing angioma growth. [orpha.net]

Early identification of neurocutaneous syndromes allows for proper surveillance, evaluation, and preventative education for development of complications. Disclosure: Dr. Fitzgerald has nothing to disclose. Dr. Chandra has nothing to disclose. Dr. [n.neurology.org]