Abstract
Introduction Children with PHACES syndrome (PS) and visual impairment or stridor show a dramatic
and immediate response to propranolol. However, this beta-blocking drug could be responsible
for an eventually increased risk of ischemic stroke due to the underlying cerebral
vascular disease. To more accurately understand the effects of propranolol on brain
vascularization, we examined PS patients treated with this drug for airway or visual
complications using brain perfusion SPECT (Single Photon Emission Computed Tomography).
In the past, this examination has been shown to be useful in the management of patients
with different neurovascular disorders.
Methods Clinical records and imaging studies were reviewed in 7 patients with a diagnosis
of PS. All patients underwent magnetic resonance angiography (MRA), echocardiography,
chest X-ray and ophthalmologic, neurological, and cardiologic assessments. They received
2–3 mg/kg/day propranolol in an attempt to treat stridor or avoid ophthalmologic occlusion.
We performed SPECT after 3–6 months of treatment.
Results SPECT showed a normal uptake in the frontal and temporal regions despite vascular
abnormalities found with MRA imaging. Significant improvements of symptoms and in
the volume of the hemangioma were noted in all cases without signs of a reduction
of brain blood perfusion.
Conclusions Propranolol treatment was safe in our patients who did not show signs of perfusion
changes. The high sensitivity for detecting functional impairment makes brain perfusion
SPECT useful in the diagnosis and follow-up of patients with PS considered at risk
of neurovascular impairment. Accurate knowledge of its pathophysiological basis, together
with the appropriate technique and careful interpretation of reporting, will enhance
the clinical use of brain SPECT in those patients.
Keywords
hemangioma - propranolol - neurocutaneous syndromes - stroke - child - intracranial
arteriovenous malformations/diagnosis
