@article{raphael2014obstructive,
  title = {Obstructive Aortic Arch Pathology and Infantile Hemangioma: Coincidence or PHACES Syndrome?},
  author = {Martine F. Raphael and Corstiaan C. Breugem and Anne M. den Boer and Moshe Kon and Suzanne G. M. A. Pasmans and Johannes M. P. J. Breur},
  year = 2014,
  url = {https://ibimapublishing.com/articles/PRIJ/2014/836615/},
  journal = {Pediatrics Research International Journal},
  volume = 2014 (2014),
  pages = 13,
  doi = 10.5171/2014.836615,
  abstract = {The objective of this article is to determine the prevalence of PHACES syndrome (Posterior fossa malformations, Hemangiomas, Arterial anomalies, Cardiac defects, Eye anomalies, Supra-umbilical raphe and/or Sternal pit) in patients with obstructive aortic arch pathology (OAAP) in order to achieve more insight in the possible association between infantile hemangiomas (IH) and cardiovascular anomalies. Pediatric patients diagnosed with OAAP between 1999 and 2013 in our tertiary referral center were included. A questionnaire on the presence of an IH and other symptoms fitting the diagnostic criteria of PHACES syndrome was created. Deceased patients were analyzed separately.  The questionnaire was sent to 266 patients with OAAP, of which 175 (66%) were returned. In 9 cases an IH was diagnosed. One child met the criteria of PHACES syndrome. This child demonstrated a segmental hemangioma and an atypical interrupted aortic arch with atresia of the left common carotid artery, which fits the complex vasculopathy seen in PHACES syndrome. The remainder (n=8) did not meet the PHACES criteria due to characteristics of IH or aortic arch. None of the deceased study objects fulfilled the PHACES syndrome criteria. In this retrospective cohort, one child met the PHACES criteria, indicating that PHACES syndrome in OAAP patients is rare. Due to limited cohort size this study was underpowered to provide evidence of an association between IH and obstructive aortic arch pathology.    },
  keywords = {Obstructive arch pathology, infantile hemangioma, PHACES, aortic coarctation.},
  note = Article ID: 836615
}