Clinical characteristics of oral Propranolol in the treatment of 4 patients with PHACES syndrome and literature review / 中华实用儿科临床杂志

ABSTRACT

Objective:To summarize the clinical data and imaging characteristics of patients with PHACES syndrome treated with oral Propranolol.Methods:The clinical data of 4 cases of PHACES syndrome treated with oral Propranolol in Children′s Hospital, Capital Institute of Pediatrics from October 2018 to October 2022 were retrospectively analyzed.Relevant studies reporting the treatment of PHACES with Propranolol were retrieved in PubMed, China National Knowledge Infrastructure (CNKI) and Wanfang Data.Results:Three cases of the 4 patients with PHACES syndrome treated with Propranolol obtained relieved symptoms of facial hemangioma, and 1 case died due to late treatment, complication of severe cardiovascular malformation, and treatment abandonment by parents.A total of 7 clinical studies on the use of Propranolol in the treatment of PHACES were retrieved, including 6 retrospective studies and 1 observational study.Most studies have shown that Propranolol is well tolerated in the treatment of PHACES syndrome, and most of cases have relieved facial hemangioma.The main factors affecting the prognosis are the degree of damage to middle and small arteries such at brain, aorta, chest and neck.Propranolol treatment can improve the prognosis.Conclusions:Oral Propranolol is currently the first-line treatment for PHACES syndrome, and most patients tolerate oral Propranolol well.