Case Report of International Journal of Case Reports
Individualized approach for Coarctation of the Aorta in a Patient with PHACE syndrome and Extensive Hemangiomas
Wejdan Ba-Atiyah, Riad Abou Zahr, Amjad Kouatli
Department of Pediatrics, Pediatric Cardiology section, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia.
PHACE syndrome is a neurocutaneous disorder with large facial segmental hemangiomas associated with anomalies of the brain, eye and heart.
We report a case of a 3 years old girl who presented at the age of 6 weeks with severe coarctation of the aorta and aortic arch aneurysm. In addition, she had extensive strawberry hemangiomas over the scalp, neck and chest which, after extensive investigation, led to the diagnosis of PHACE syndrome.
After complete evaluation we successfully initiated treatment with propranolol that was pursued without complications over three years with striking success and remarkable regression of her hemangiomas. Meanwhile the coarctation was managed conservatively.
This case highlights that propranolol treatment, while effective against hemangiomas, may contribute to slow the progression of aortic arch aneurysm and delay the need for high risk surgical intervention.
Keywords: PHACE syndrome, Propranolol, Coarctation of the aorta
How to cite this article:
Wejdan Ba-Atiyah, Riad Abou Zahr, Amjad Kouatli. Individualized approach for Coarctation of the Aorta in a Patient with PHACE syndrome and Extensive Hemangiomas. International Journal of Case Reports, 2020 4:128. DOI: 10.28933/ijcr-2020-05-0105
1. Frieden IJ, Reese V, Cohen D. PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol. 1996;132(3):307-11.
2. Garzon MC, Epstein LG, Heyer GL, Frommelt PC, Orbach DB, Baylis AL, et al. PHACE Syn-drome: Consensus-Derived Diagnosis and Care Recommendations. The Journal of pediatrics. 2016;178:24-33 e2.
3. Raas-Rothschild A, Nir A, Gillis R, Rein AJ. Giant congenital aortic aneurysm with cleft sternum, supraumbilical raphe, and hemangiomatosis: re-port and review. Am J Med Genet. 2000;90(3):243-5.
4. Haggstrom AN, Lammer EJ, Schneider RA, Mar-cucio R, Frieden IJ. Patterns of infantile heman-giomas: new clues to hemangioma pathogenesis and embryonic facial development. Pediatrics. 2006;117(3):698-703.
5. Pascual-Castroviejo I. Vascular and nonvascular intracranial malformation associated with external capillary hemangiomas. Neuroradiology. 1978;16:82-4.
6. Rotter A, Samorano LP, Rivitti-Machado MC, Oliveira ZNP, Gontijo B. PHACE syndrome: clinical manifestations, diagnostic criteria, and management. An Bras Dermatol. 2018;93(3):405-11.
7. Haggstrom AN, Garzon MC, Baselga E, Chamlin SL, Frieden IJ, Holland K, et al. Risk for PHACE syndrome in infants with large facial hemangio-mas. Pediatrics. 2010;126(2):e418-26.
8. Metry D, Heyer G, Hess C, Garzon M, Haggstrom A, Frommelt P, et al. Consensus Statement on Diagnostic Criteria for PHACE Syndrome. Pediatrics. 2009;124(5):1447-56.
9. Castellano-Martinez A, Rodriguez-Gonzalez M, Benavente-Fernandez I, Zuazo-Ojeda A. Hemangioma, aortic coarctation and intracranial dolichoectasia: PHACE syndrome. Pediatr Int. 2017;59(2):230-3.
10. Drolet BA, Frommelt PC, Chamlin SL, Haggstrom A, Bauman NM, Chiu YE, et al. Initia-tion and use of propranolol for infantile heman-gioma: report of a consensus conference. Pedi-atrics. 2013;131(1):128-40.
11. Leaute-Labreze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taieb A. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008;358(24):2649-51.
12. Caragher SP, Scott JP, Siegel DH, Mitchell ME, Frommelt PC, Drolet BA. Aortic arch repair in children with PHACE syndrome. J Thorac Cardi-ovasc Surg. 2016;152(3):709-17.
13. Prada F, Mortera C, Bartrons J, Rissech M, Jimenez L, Carretero J, et al. Complex aortic co-arctation and PHACE syndrome. Rev Esp Cardi-ol. 2010;63(11):1367-70.
This work and its PDF file(s) are licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.