A 6-month-old female infant with a large upper facial hemangioma presents for pulse-dye laser treatment. She is diagnosed with PHACE syndrome when workup reveals a small ventricular septal defect (VSD) and a right-sided aortic arch.
PHACE (or PHACES) syndrome is most commonly found in female, singleton, term, normal birth weight infants. It is diagnosed when a large facial hemangioma is associated with one or more of the congenital anomalies listed in the acronym:
P: Posterior fossa malformation. Dandy-Walker syndrome, cerebellar hypoplasia, hypoplasia of the corpus callosum, microcephaly, or absent pituitary.
H: Hemangioma. Large (>5 cm), segmental, extracutaneous hemangiomas on the head or neck; the most common site is subglottic, which may cause respiratory compromise.
A: Arterial anomalies of the head and neck. Abnormal cerebral arteries, saccular aneurysm, cerebral sinus malformation, dural arteriovenous malformation, Moyamoya disease, or acute arterial stroke.
C: Cardiac defect. Aortic coarctation and other aortic anomalies, patent ductus arteriosus, VSD, atrial septal defect, pulmonary stenosis, or cor triatriatum.
E: Eye abnormalities. Microphthalmos, optic nerve atrophy, coloboma, cataracts, third nerve palsy, or Horner’s syndrome; unique to PHACE is “morning glory” retinal deformity.
S: Ventral defects. Sternal clefting or pit, or supraumbilical raphe.
Other associated findings may include micrognathia, congenital hypothyroidism, spina bifida, esophageal diverticulum, and sensorineural hearing loss.
The majority of children have neurologic sequelae—mostly developmental delay and seizures, and less commonly migraine headaches ipsilateral to the hemangioma, hypotonia, and central apnea. Routine evaluation consists of ophthalmologic exam, MRI or MRA of the head and neck, and cardiac evaluation including an echocardiogram.
Hemangiomas grow rapidly in the first year and typically regress spontaneously over the next few years. Treatment is required when there is airway compromise, visual impairment, skin necrosis, or infection.
Antiepileptics should be continued throughout the perioperative period.
The IV line should be meticulously cleared of air bubbles in the presence of an intracardiac shunt.
Careful airway examination for micrognathia is needed, as this may necessitate preparation for a difficult mask ventilation and/or intubation.
If a subglottic hemangioma is suspected based on presentation or imaging, an ear, nose, and throat surgeon should be present for evaluation of the lesion and assistance with airway management.
Mask induction and maintenance with a volatile anesthetic is normally safe.
The airway can be managed with laryngeal mask anesthesia in older children, while endotracheal intubation may be more appropriate in infants.
Maintenance of adequate cerebral perfusion pressure in the presence of abnormal cerebral vasculature is necessary.
Use rectal acetaminophen at the start of the procedure.
Postoperative pain is minimal and can usually be managed without narcotics. Prolonged apnea monitoring may be required for children with a history of hypotonia and apnea.
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