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

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...

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Autores principales: Rokugo, Yuka, Ota, Chiharu, Kimura, Masato, Sasahara, Yoji
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6506039/
https://www.ncbi.nlm.nih.gov/pubmed/31036736
http://dx.doi.org/10.1136/bcr-2018-228117
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author Rokugo, Yuka
Ota, Chiharu
Kimura, Masato
Sasahara, Yoji
author_facet Rokugo, Yuka
Ota, Chiharu
Kimura, Masato
Sasahara, Yoji
author_sort Rokugo, Yuka
collection PubMed
description 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.
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spelling pubmed-65060392019-05-28 Preoperative administration of propranolol reduced the surgical risks of PHACES syndrome in a 14-month-old girl Rokugo, Yuka Ota, Chiharu Kimura, Masato Sasahara, Yoji BMJ Case Rep Rare Disease 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. BMJ Publishing Group 2019-04-29 /pmc/articles/PMC6506039/ /pubmed/31036736 http://dx.doi.org/10.1136/bcr-2018-228117 Text en © BMJ Publishing Group Limited 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
spellingShingle Rare Disease
Rokugo, Yuka
Ota, Chiharu
Kimura, Masato
Sasahara, Yoji
Preoperative administration of propranolol reduced the surgical risks of PHACES syndrome in a 14-month-old girl
title Preoperative administration of propranolol reduced the surgical risks of PHACES syndrome in a 14-month-old girl
title_full Preoperative administration of propranolol reduced the surgical risks of PHACES syndrome in a 14-month-old girl
title_fullStr Preoperative administration of propranolol reduced the surgical risks of PHACES syndrome in a 14-month-old girl
title_full_unstemmed Preoperative administration of propranolol reduced the surgical risks of PHACES syndrome in a 14-month-old girl
title_short Preoperative administration of propranolol reduced the surgical risks of PHACES syndrome in a 14-month-old girl
title_sort preoperative administration of propranolol reduced the surgical risks of phaces syndrome in a 14-month-old girl
topic Rare Disease
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6506039/
https://www.ncbi.nlm.nih.gov/pubmed/31036736
http://dx.doi.org/10.1136/bcr-2018-228117
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