Objectives: To report the clinical features, nature of vasculopathy observed and the management instituted in three cases of PHACE syndrome.
Results: All three patients were noted to have diffuse vasculopathy and aortic arch hypoplasia in addition to aortic coarctation adding substantial surgical risk or making surgery palliative.
Conclusion: PHACE syndrome should be considered in the differential diagnosis of aortic coarctation associated with aortic arch hypoplasia.
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| http://dx.doi.org/10.1111/j.1747-0803.2008.00193.x | DOI Listing |
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'PHACE' on the 'face'.
BMJ Case Rep
January 2025
Paediatric Department, SJOG Midland Public Hospital, Midland, Western Australia, Australia.
Infantile haemangiomas are a common presentation in infants within the first few months of life. The majority of haemangiomas are benign; however, large haemangiomas (≥5 cm), especially those involving the face, may indicate a more serious underlying neurocutaneous disorder known as PHACE (Posterior fossa malformations, Haemangioma, Arterial anomalies, Coarctation of the aorta/Cardiac defects and Eye abnormalities) syndrome. The authors report an unusual case of possible PHACE syndrome in a young male toddler with a large facial haemangioma.
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Division of Dermatology, Department of Medicine, University of Toronto, Toronto, ON, Canada; Division of Dermatology, The Hospital for Sick Children, Toronto, ON, Canada.
Contralateral oculomotor nerve palsy associated with PHACES syndrome.
J AAPOS
December 2024
Ophthalmology and Visual Sciences, University of British Columbia, Vancouver, Canada.
A 13-month-old boy presented to the pediatric ophthalmology clinic at BC Children's Hospital for strabismus assessment. On examination he had a right facial hemangioma, left ptosis, and left exotropia and hypotropia. Magnetic resonance imaging and magnetic resonance angiography of his head demonstrated posterior cerebral artery tortuosity producing mild mass effect on the left oculomotor nerve.
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Department of Oral Pathology, Wroclaw Medical University, Poland.
Symmetry is present in various aspects of everyday life. A symmetrical face is considered attractive, whereas a lack of facial symmetry is regarded as a source of functional and aesthetic problems. Most of the people exhibit slight asymmetries, but some of them reveal severe asymmetries.
View Article and Find Full Text PDFPHACEing a challenging diagnosis: Should we expand the phenotype?
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Paediatric Neurology/Neurogenetics Units, Monash Health, Melbourne, Victoria, Australia.
Article Synopsis
- PHACE syndrome is a complex prenatal condition that can have significant health implications, often identified through specific signs observed in prenatal imaging.
- The 'tilted telephone receiver' sign suggests a strong link to PHACE, but recent cases show it can also appear in less severe forms, prompting discussions about expanding how we recognize this syndrome.
- A particular case with mild symptoms resembling PHACE did not actually result in the condition after birth, indicating that expanded criteria for diagnosis could lead to incorrect negative predictions for affected pregnancies.
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