Table 4.

PHACE Patients on Propranolol with Higher Risk Imaging Features for AIS

Case	IH Location(s)	Cerebro- vascular Anomalies	CNS Anomalies	Cardiovascular Anomalies	Other Medical	Indication(s) for Propranolol	Prop Initiation	Propranolol: Age initiated/ Duration	Propranolol dose	Repeat Cerebro- vascular imaging	Atypical Events	Prop side effects/ IH response
1	Rt S1, S3, S4, Rt chest, airway	Hypoplastic, dysplastic Rt PCA w focal stenosis in P2 segment, absent Rt ICA, hypoplastic vertebrobasilar w aberrant origin/course	N	N	Sternal defect/pit	Visual compromise, high risk of facial disfigurement , CS side effects (cushingoid features, rebound IH growth w taper)	CS started 1st (2 mg/kg/d from 3 wks-11 mos), neurology input, hosp admit for initiation	13 mos/14.5 mos	1.5 mg/kg/d divided t.i.d.	MRA X 2 (baseline & at 1 yr of age), stable	N	Episodes of asymptomatic hypotension led to dose decrease, f/up vitals WNL; mod-E
2	Rt S1, Rt hemi- diaphragm , multiple abdominal : spleen, small bowel mesentery , upper anterior abdomen, anterior abdominal wall, bil hepatic lobes	Aberrant origin/course of Rt cavernous carotid a, severe dysplasia and/or dural AVF of vessels superomedial & lateral to Rt orbital IH, absent Lt PCA, long- segment stenosis of Rt ICA (initially mild)	N	N	N	Rapid growth, severe visual compromise, high risk of facial disfigurement , insufficient response to steroids	CS started 1st (1- 3 mg/kg/d from 1–9 mos), followed by vincristine X 1 dose; neurology input, slower upward taper w more frequent f/up	7 mos/1st course X 8 mos; restarted at 19 mos X 2 mos; restarted again at 2.5 yrs	2.0 mg/kg/d divided b.i.d	MRA X 4 (6 mos, 15 mos, 19 mos & 2.5 yrs of age); MRP at 15 mos, interval vessel changes noted	Repeat MRA at 15 mos showed progressive vessel stenosis from mild to severe (confirmed with MRP); propran olol was d/c; at 19 mos imaging stable, prop restarte d X 2 mos w/out incident ; repeat MRA at 2.5 yrs showed interval improve ment in degree of stenosis ; pt remained neurologically stable through out course	Mild sleep disturbance/mild
3	Rt S1-S3, partial Lt S1 & S2	Absent Lt ICA w the Lt ACA supplied by the Rt ACA & the Lt ophthalmic and bil MCAs supplied from a Lt PCOM, dysplastic PTA w possible saccular aneurysm, dysplastic Rt ICA, hypoplastic Rt VA, Lt parietal developmental venous anomaly, poss old hemorrhage Rt cerebello- pontine angle	Dandy- Walker Malformation	N	N	Rapid growth, visual compromise, high risk of facial disfigurement	Neurology input	10 mos/22 mos	2.0 mg/kg/d divided t.i.d	MRA at baseline & every 6 mos until 2 yrs, stable	N	N/E
4	Rt S1, S2	Absent Rt ICA w hypoplastic Rt MCA & hypoplastic or absent A1 segment of Rt ACA, dysplastic Lt intracranial ICA, hypoplastic Rt CCA w aberrant origin/course	N	N	N	Rapid growth, visual compromise	Initiation by outside practice, intralesional CS X 1 1st, hosp admit for initiation	5 wks/present (35 mos)	2.0 mg/kg/d divided b.i.d	MRA X 3 (6 wks, 6 mos, 1 yr of age), stable	N	N/E
5	Bil S3, Lt scalp, G.I. tract, airway (subglottic)	Dysplastic Lt ICA, MCA narrowing	N	Coarctation of the aorta, required surgical repair	Sternal scar	Airway compromise, insufficient response to CS (2nd course), G.I. bleeding	Initiation by outside practice; prop d/c at 3 mos & CS started (2 mg/kg/d from 3- 9 mos) b/c of stroke risk, but then restarted at 5 mos due to worsening airway	2 wks/u/k	2.0 mg/kg/d divided t.i.d	N	N	N/E
6	Lt S1, S2, S3, scalp, neck, upper back	Dysplastic & narrowed Lt ICA, narrowed Lt MCA	Unilateral cerebellar hypoplasia/ dysplasia	Coarctation of the aorta, Lt transverse arch narrowing	N	Rapid growth, high risk of facial disfigurement , ulceration	Lower & t.i.d. dosing in combination w CS (2 mg/kg/d)	3 wks/present (18 mos)	1.0 mg/kg/d divided t.i.d	MRA X 2 (baseline & after 4 mos; narrowing improved)	Very severe scalp & ear ulceration that destroyed upper half of ear	? worsened tissue necrosis/mod
7	Lt S2, Bil S3, partial S4, intraoral, neck, chest, gluteal cleft/buttocks, airway	Absent lt A1, narrow & dysplastic lt ICA & MCA, marked dysplasia top of basilar a, dysplastic PCOM	N	Small focal outpouching at lateral distal aortic arch, differential diagnosis = atypical ductus bump versus aortic aneurysm or pseudoaneurysm	Hamartom atous growth at chin and Lt tongue, poor oral intake required G-tube placement, hypotonia in trunk & legs, borderline gross motor delay at 6 mos	Rapid growth, high risk of facial disfigurement , ulceration, airway compromise	Slow upward taper; increased from 1 mg/kg/d to 2 mg/kg/d for proliferation, then 3 mg/kg/d for airway compromise at which time CS 1 mg/kg/d divided b.i.d. also added	1 wk/present	3.0 mg/kg/d divided t.i.d	MRA X 2 (baseline & 4.5 mos of age, stable)	N	N/E

A = artery; ACA = anterior cerebral artery; AVF = arteriovenous fistula; Avg = average; b/c = because; bil = bilateral; CCA = common carotid artery; CNS = central nervous system; CS = corticosteroids; CTA = computed tomography angiography; d/c = discontinued; E = excellent; f/up = follow-up; G.I. = gastrointestinal; hosp = hospital; hrs = hours; HTN = hypertension; hosp = hospital; ICA = internal carotid artery; IH = infantile hemangioma; Lt = left; MCA = middle cerebral artery; med = medication; mg/kg/d = milligrams per kilogram per day; mod = moderate; mo(s) = month(s); MRA = magnetic resonance angiography; N = none; PCA = posterior cerebral artery; PCOM = posterior communicating artery; PDA = patent ductus arteriosus; pt = patient, post = posterior; PTA = persistent trigeminal artery; prop = propranolol; Rt = right; S = segment; t.i.d. = three times daily; txd = treated; u/k = unknown; VA = vertebral artery; wks = weeks; w = with; WNL = within normal limits; w/u = work-up; yr = year