>Year 2014, Issue 11
Rubio GT, Álvarez VH, Hechavarría ED, Cuadras BY
Association of sternal malformation and vascular dysplasia in an infant
MediSan 2014; 18 (11)
PDF: 92.18 Kb.
The clinical case of a 3 months first-born infant is described who had been evaluated by a
specialist of Clinical Genetics in "Dr. Juan de la Cruz Martínez Maceira" Northern Pediatric
Hospital in Santiago de Cuba, for presenting a fissure in the inferior third of the sternum.
Later on hemangiomas emerged in the inferior lip, the lobe of the right ear and the
lateral right part of the neck, as well as a mid abdominal raphe from the sternal fissure to
the navel. The required complementary tests were carried out and she was scheduled
for several specialties, with which it was concluded that the patient presented an
association of sternal malformation and vascular dysplasia. It is the first case notified in
||sternal malformation, vascular dysplasia, hemangiomas, abdominal raphe, infant, pediatric hospitals.
Lyons Jones K, Smith DW. Espectro de malformación esternal-displasia vascular. En: Smith´s Recognizable patterns of human malformation. 6 ed. Philadelphia: Elsevier-Saunders; 2005. p. 746.
Hersh JH, Waterfill D, Rutledge J, Harrod MJ, O'Sheal SF, Verdi G, et al. Sternal malformation- vascular displasia association. Am J Med Genet. 1985; 21(1): 177-86.
Durusoy C, Mihci E, Tacoy S, Ozaydin E, Alpsoy E. PHACES syndrome presenting as hemangiomas, sternal clefting and congenital ulcerations on the helices. J Dermatol. 2006; 33(3): 219- 22.
Vermeer S, van Oostrom CG, Boetes C, Verrips A, Knoers NV. A unique case of PHACES syndrome confirming the assumption that PHACES syndrome and the sternal malformation- vascular dysplasia association are part of the same spectrum of malformations. Clin Dysmorphol. 2005; 14(4): 203- 6.
Chakkarapani E, Barnard I, Couriel J. Superior sternal cleft, cutaneous, and airway haemangiomas. Arch Dis Child Fetal Neonatal. 2007; 92(1): 3.
Mazereeuw-Hautier J, Syed S, Harper JI. Sternal malformation/vascular dysplasia syndrome with linear hypopigmentation. Br J Dermatol. 2006; 155(1): 192-4.
Roganovic J, Adams D. PHACES syndrome- case report and literature review. Coll Antropol. 2009; 33(1): 311-4.
Bronzetti G, Giardani A, Patrizi A, Prandstraller D, Donti A, Formigari R, et al. Ipsilateral hemangioma and aortic arch anomalies in posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, and cardiac defects and eye abnormalities (PHACE) anomaly: report and review. Pediatrics. 2004; 113(2): 412-5.
Kronenberg A, Blei F, Ceisler E, Steele M, Furlan L, Kodsi S. Ocular and systemic manifestations of PHACES (Posterior fossa malformations, Hemangiomas, Arterial anomalies, Cardiac defects and coarctation of the Aorta, Eye abnormalities, and Sternal abnormalities or ventral developmental defects) syndrome. J AAPOS. 2005; 9(2): 169- 73.
Heyer GL. Millar WS, Ghatan S, Garzon MC. The neurologic aspects of PHACE: case report and review of the literature. Pediatric Neurol. 2006; 35(6): 419-24.
>Year 2014, Issue 11