PHACE without Face? Infantile hemangiomas of the upper body region with minimal or absent facial hemangiomas and associated structural malformations

Adam S. Nabatian, Sandy S. Milgraum, Christopher P. Hess, Anthony J. Mancini, Alfons Krol, Ilona J. Frieden

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

Infantile hemangiomas can be associated with congenital anomalies such as PHACE syndrome with facial hemangiomas and genitourinary and spinal anomalies in the setting of lower body hemangiomas. We describe five infants in whom segmental hemangiomas involving the upper torso and extremities with absent or small facial hemangiomas were associated with structural anomalies similar to those reported with PHACE syndrome, including three with structural arterial anomalies of the subclavian arteries, three with aortic arch anomalies (right sided or narrowed arch), two with congenital heart disease (atrial septal defect and ventricular septal defect; tetralogy of Fallot), one with a retinal scar, and one with a sternal defect (scar). Two of five had small facial hemangiomas of the lower lip, but none had large segmental hemangiomas of the face. Three of five would have met diagnostic criteria for PHACE but lacked a facial hemangioma of 5 cm in diameter or greater. Patients with segmental arm and thorax hemangiomas may have associated structural abnormalities with overlapping features of PHACE, suggesting that a similar syndrome can occur in this clinical setting.

Original languageEnglish (US)
Pages (from-to)235-241
Number of pages7
JournalPediatric Dermatology
Volume28
Issue number3
DOIs
StatePublished - May 2011

Fingerprint

Body Regions
Hemangioma
Cicatrix
Torso
Subclavian Artery
Tetralogy of Fallot
Atrial Heart Septal Defects
Ventricular Heart Septal Defects
Lip
Thoracic Aorta
Upper Extremity
Heart Diseases
Arm
Thorax

ASJC Scopus subject areas

  • Dermatology
  • Pediatrics, Perinatology, and Child Health

Cite this

PHACE without Face? Infantile hemangiomas of the upper body region with minimal or absent facial hemangiomas and associated structural malformations. / Nabatian, Adam S.; Milgraum, Sandy S.; Hess, Christopher P.; Mancini, Anthony J.; Krol, Alfons; Frieden, Ilona J.

In: Pediatric Dermatology, Vol. 28, No. 3, 05.2011, p. 235-241.

Research output: Contribution to journalArticle

Nabatian, Adam S. ; Milgraum, Sandy S. ; Hess, Christopher P. ; Mancini, Anthony J. ; Krol, Alfons ; Frieden, Ilona J. / PHACE without Face? Infantile hemangiomas of the upper body region with minimal or absent facial hemangiomas and associated structural malformations. In: Pediatric Dermatology. 2011 ; Vol. 28, No. 3. pp. 235-241.
@article{30cb02f308714de2abd15447246f55de,
title = "PHACE without Face? Infantile hemangiomas of the upper body region with minimal or absent facial hemangiomas and associated structural malformations",
abstract = "Infantile hemangiomas can be associated with congenital anomalies such as PHACE syndrome with facial hemangiomas and genitourinary and spinal anomalies in the setting of lower body hemangiomas. We describe five infants in whom segmental hemangiomas involving the upper torso and extremities with absent or small facial hemangiomas were associated with structural anomalies similar to those reported with PHACE syndrome, including three with structural arterial anomalies of the subclavian arteries, three with aortic arch anomalies (right sided or narrowed arch), two with congenital heart disease (atrial septal defect and ventricular septal defect; tetralogy of Fallot), one with a retinal scar, and one with a sternal defect (scar). Two of five had small facial hemangiomas of the lower lip, but none had large segmental hemangiomas of the face. Three of five would have met diagnostic criteria for PHACE but lacked a facial hemangioma of 5 cm in diameter or greater. Patients with segmental arm and thorax hemangiomas may have associated structural abnormalities with overlapping features of PHACE, suggesting that a similar syndrome can occur in this clinical setting.",
author = "Nabatian, {Adam S.} and Milgraum, {Sandy S.} and Hess, {Christopher P.} and Mancini, {Anthony J.} and Alfons Krol and Frieden, {Ilona J.}",
year = "2011",
month = "5",
doi = "10.1111/j.1525-1470.2011.01407.x",
language = "English (US)",
volume = "28",
pages = "235--241",
journal = "Pediatric Dermatology",
issn = "0736-8046",
publisher = "Wiley-Blackwell",
number = "3",

}

TY - JOUR

T1 - PHACE without Face? Infantile hemangiomas of the upper body region with minimal or absent facial hemangiomas and associated structural malformations

AU - Nabatian, Adam S.

AU - Milgraum, Sandy S.

AU - Hess, Christopher P.

AU - Mancini, Anthony J.

AU - Krol, Alfons

AU - Frieden, Ilona J.

PY - 2011/5

Y1 - 2011/5

N2 - Infantile hemangiomas can be associated with congenital anomalies such as PHACE syndrome with facial hemangiomas and genitourinary and spinal anomalies in the setting of lower body hemangiomas. We describe five infants in whom segmental hemangiomas involving the upper torso and extremities with absent or small facial hemangiomas were associated with structural anomalies similar to those reported with PHACE syndrome, including three with structural arterial anomalies of the subclavian arteries, three with aortic arch anomalies (right sided or narrowed arch), two with congenital heart disease (atrial septal defect and ventricular septal defect; tetralogy of Fallot), one with a retinal scar, and one with a sternal defect (scar). Two of five had small facial hemangiomas of the lower lip, but none had large segmental hemangiomas of the face. Three of five would have met diagnostic criteria for PHACE but lacked a facial hemangioma of 5 cm in diameter or greater. Patients with segmental arm and thorax hemangiomas may have associated structural abnormalities with overlapping features of PHACE, suggesting that a similar syndrome can occur in this clinical setting.

AB - Infantile hemangiomas can be associated with congenital anomalies such as PHACE syndrome with facial hemangiomas and genitourinary and spinal anomalies in the setting of lower body hemangiomas. We describe five infants in whom segmental hemangiomas involving the upper torso and extremities with absent or small facial hemangiomas were associated with structural anomalies similar to those reported with PHACE syndrome, including three with structural arterial anomalies of the subclavian arteries, three with aortic arch anomalies (right sided or narrowed arch), two with congenital heart disease (atrial septal defect and ventricular septal defect; tetralogy of Fallot), one with a retinal scar, and one with a sternal defect (scar). Two of five had small facial hemangiomas of the lower lip, but none had large segmental hemangiomas of the face. Three of five would have met diagnostic criteria for PHACE but lacked a facial hemangioma of 5 cm in diameter or greater. Patients with segmental arm and thorax hemangiomas may have associated structural abnormalities with overlapping features of PHACE, suggesting that a similar syndrome can occur in this clinical setting.

UR - http://www.scopus.com/inward/record.url?scp=79958038171&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=79958038171&partnerID=8YFLogxK

U2 - 10.1111/j.1525-1470.2011.01407.x

DO - 10.1111/j.1525-1470.2011.01407.x

M3 - Article

C2 - 21453307

AN - SCOPUS:79958038171

VL - 28

SP - 235

EP - 241

JO - Pediatric Dermatology

JF - Pediatric Dermatology

SN - 0736-8046

IS - 3

ER -