Preoperative administration of propranolol reduced the surgical risks of PHACES syndrome in a 14-month-old girl

Yuka Rokugo, Chiharu Ota, Masato Kimura, Yoji Sasahara

Research output: Contribution to journalArticlepeer-review

Abstract

PHACES syndrome is an uncommon neurocutaneous disorder first identified in 1996. Patients with PHACES syndrome often require surgical treatment for their anomalies, including intracranial vasculopathy, coarctation/interruption of the aorta, intracardiac defects, glaucoma/cataract and sternal defects. Risk factors associated with the symptoms of intraoperative/perioperative management include ischaemic stroke due to the cerebral vasculopathy, airway obstruction due to the subglottic/tracheal haemangiomas and massive bleeding due to the large haemangiomas. Recently, propranolol is considered as first-line therapy for patients with infantile haemangiomas (IHs). However, until now, there have been no reported cases of PHACES syndrome treated by propranolol to reduce the surgical risks associated with IH. In this report, we describe a case of a 14-month-old Japanese girl with PHACES syndrome treated by propranolol for IH before surgical closure of the ventricular septum defect. Oral administration of propranolol was effective in decreasing the size of IH, leading to the uneventful perioperative course.

Original languageEnglish
Article numbere228117
JournalBMJ case reports
Volume12
Issue number4
DOIs
Publication statusPublished - 2019 Apr 1

Keywords

  • congenital disorders
  • paediatrics

ASJC Scopus subject areas

  • Medicine(all)

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