Table 1.
Propranolol Use in 32 PHACE Syndrome Infants with Cervical and Intracranial Arterial Anomalies: Overall Results
| Category |
| Results: n (%) [case number(s)] |
| IH Location |
| S1 and/or S3: 30 (94%) |
| S4 only: 2 |
| Extensive, unilateral facial involving all 3 segments or bilateral facial: 17 (53%) |
| Scalp: 9 (28%) |
| Visceral: 15 (47%); airway: 10, CNS: 5, g.i.: 3, paraspinous muscle: 1, spleen: 1, liver: 1 |
| Cerebral or Cervical Arteriopathy: 32 (100%) |
| Dysplasia: 20 (63%) |
| Hypoplasia: 17 (53%) |
| Aberrant origin or course: 11 (34%) |
| Narrowing/stenosis: 12 (38%) [cases 2–5, 8, 11, 12 (severe, long-segment), 13, 22, 25, 28, 32] |
| Non-visualization/absence: 6 (19%): [cases 8, 11, 12, 18, 19, 32] |
| Other: persistent trigeminal a: 3, fetal posterior communicating a: 2, aneurysm [case 10], possible aneurysm [case 18], possible dural arteriovenous fistula [case 12] |
| Structural CNS Anomalies: 13 (41%) |
| Cardiovascular Anomalies: 11 (34%) |
| Aberrant rt subclavian artery: 7 (22%) |
| Aortic arch anomalies: 6; mild coarctation with dysplasia [case 2], severe coarctation requiring surgical repair [case 22], coarctation with transverse arch narrowing [case 28], rt aortic arch [case 25], rt aortic arch with dysplasia [case 3], small aneurysm versus pseudoaneurysm [case 32] |
| Other: Patent ductus arteriosus [cases 6, 10, 11], anomalous origin/course of cardiac vessel [cases 10, 15, 30], patent foramen ovale [cases 25, 30], pulmonary stenosis [cases 16, 21], atrial septal defect [case 21], ventricular septal defect [case 30] |
| Indications for Propranolol |
| High-risk for facial scarring/disfigurement: 22 (69%) |
| Visual compromise: 15 (47%) |
| Insufficient response to systemic corticosteroids: 9 (28%) |
| Ulceration (actual or potential): 8 (25%) |
| Other: side effects from systemic corticosteroids: 4 (hypertension in 3, cushingoid appearance in 1), airway compromise: 5, g.i. bleeding: 2, auditory obstruction: 1, persistent bulk/slow involution: 1, persistent intraoral IH: 1 |
| Propranolol Dosing |
| Avg age at initiation: 4.8 mos (range: 7 days to 24 mos) |
| Avg daily dose: 1.8 mg/kg/d |
| Frequency: t.i.d. in 23 (72%), b.i.d. in 9 (28%) |
| Avg duration: 12.3 mos in 19 patients who completed therapy (9 pts still on propranolol, 3 lost to follow-up) |
| Propranolol Side Effects |
| Sleep disturbance/night terrors: 2 |
| Single case each: g.i. upset, asymptomatic hypotension, periodic cold hands & feet, constipation |
| Physician’s Assessment of IH Response to Propranolol |
| Excellent: 23 (72%), excellent to moderate: 1, moderate: 6, moderate to mild: 1, mild: 1 |
| Atypical Events during Propranolol Therapy (see also Discussion) |
| Change in neuroimaging: progressive vessel narrowing [case 12] |
| Change in neurologic status: mild hemiparesis [case 11] |
| Worsening IH ulceration/tissue necrosis [cases 25, 28, 30] |
A = artery; avg = average; g.i. = gastrointestinal; IH = infantile hemangioma; mos = months; rt = right