Otolaryngologic manifestations of PHACE syndrome

Dana S. Smith, Ken K. Lee, Henry Milczuk

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Two case reports of PHACE syndrome (posterior fossa malformations (P), hemangiomas (H), arterial anomalies (A), coarctation of the aorta and cardiac defects (C), and eye abnormalities (E)) are presented. Clinical characteristics consisted of cutaneous and airway hemangiomas, aortic coarctation, and left superior vena cava in one child and cutaneous and airway hemangiomas, sternal clefting, and supraumbilical raphe in the other child. Treatment modalities included systemic and intralesional steroids for cutaneous and airway hemangiomas, submucosal resection and laryngotracheal reconstruction for airway hemangiomas, repair of aortic coarctation, and laser treatment of cutaneous hemangiomas. PHACE syndrome poses a significant potential for airway compromise from hemangiomas, which may require multimodality treatment.

Original languageEnglish (US)
Pages (from-to)1445-1450
Number of pages6
JournalInternational Journal of Pediatric Otorhinolaryngology
Volume68
Issue number11
DOIs
StatePublished - Nov 2004

Fingerprint

Hemangioma
Aortic Coarctation
Skin
Eye Abnormalities
Superior Vena Cava
Lasers
Therapeutics
Steroids

Keywords

  • Airway hemangioma
  • Neurocutaneous syndrome
  • Pediatric

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine
  • Surgery

Cite this

Otolaryngologic manifestations of PHACE syndrome. / Smith, Dana S.; Lee, Ken K.; Milczuk, Henry.

In: International Journal of Pediatric Otorhinolaryngology, Vol. 68, No. 11, 11.2004, p. 1445-1450.

Research output: Contribution to journalArticle

@article{bda49328b5bd4cbcbceb320b5281c0f5,
title = "Otolaryngologic manifestations of PHACE syndrome",
abstract = "Two case reports of PHACE syndrome (posterior fossa malformations (P), hemangiomas (H), arterial anomalies (A), coarctation of the aorta and cardiac defects (C), and eye abnormalities (E)) are presented. Clinical characteristics consisted of cutaneous and airway hemangiomas, aortic coarctation, and left superior vena cava in one child and cutaneous and airway hemangiomas, sternal clefting, and supraumbilical raphe in the other child. Treatment modalities included systemic and intralesional steroids for cutaneous and airway hemangiomas, submucosal resection and laryngotracheal reconstruction for airway hemangiomas, repair of aortic coarctation, and laser treatment of cutaneous hemangiomas. PHACE syndrome poses a significant potential for airway compromise from hemangiomas, which may require multimodality treatment.",
keywords = "Airway hemangioma, Neurocutaneous syndrome, Pediatric",
author = "Smith, {Dana S.} and Lee, {Ken K.} and Henry Milczuk",
year = "2004",
month = "11",
doi = "10.1016/j.ijporl.2004.05.013",
language = "English (US)",
volume = "68",
pages = "1445--1450",
journal = "International Journal of Pediatric Otorhinolaryngology",
issn = "0165-5876",
publisher = "Elsevier Ireland Ltd",
number = "11",

}

TY - JOUR

T1 - Otolaryngologic manifestations of PHACE syndrome

AU - Smith, Dana S.

AU - Lee, Ken K.

AU - Milczuk, Henry

PY - 2004/11

Y1 - 2004/11

N2 - Two case reports of PHACE syndrome (posterior fossa malformations (P), hemangiomas (H), arterial anomalies (A), coarctation of the aorta and cardiac defects (C), and eye abnormalities (E)) are presented. Clinical characteristics consisted of cutaneous and airway hemangiomas, aortic coarctation, and left superior vena cava in one child and cutaneous and airway hemangiomas, sternal clefting, and supraumbilical raphe in the other child. Treatment modalities included systemic and intralesional steroids for cutaneous and airway hemangiomas, submucosal resection and laryngotracheal reconstruction for airway hemangiomas, repair of aortic coarctation, and laser treatment of cutaneous hemangiomas. PHACE syndrome poses a significant potential for airway compromise from hemangiomas, which may require multimodality treatment.

AB - Two case reports of PHACE syndrome (posterior fossa malformations (P), hemangiomas (H), arterial anomalies (A), coarctation of the aorta and cardiac defects (C), and eye abnormalities (E)) are presented. Clinical characteristics consisted of cutaneous and airway hemangiomas, aortic coarctation, and left superior vena cava in one child and cutaneous and airway hemangiomas, sternal clefting, and supraumbilical raphe in the other child. Treatment modalities included systemic and intralesional steroids for cutaneous and airway hemangiomas, submucosal resection and laryngotracheal reconstruction for airway hemangiomas, repair of aortic coarctation, and laser treatment of cutaneous hemangiomas. PHACE syndrome poses a significant potential for airway compromise from hemangiomas, which may require multimodality treatment.

KW - Airway hemangioma

KW - Neurocutaneous syndrome

KW - Pediatric

UR - http://www.scopus.com/inward/record.url?scp=5644290750&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=5644290750&partnerID=8YFLogxK

U2 - 10.1016/j.ijporl.2004.05.013

DO - 10.1016/j.ijporl.2004.05.013

M3 - Article

C2 - 15488979

AN - SCOPUS:5644290750

VL - 68

SP - 1445

EP - 1450

JO - International Journal of Pediatric Otorhinolaryngology

JF - International Journal of Pediatric Otorhinolaryngology

SN - 0165-5876

IS - 11

ER -