June 22, 2020

Jan ; Hemangiomas and Vascular Malformations; pp [object Object]. Maria Cordisco. The incidence of hemangiomas of infancy or infantile. Bayer ML, Frommelt PC, Blei F, Breur JM, Cordisco MR, Frieden IJ, Goddard DS, Propranolol treatment of infantile hemangiomas: anticipatory guidance for. Background: Haemangioma of infancy (HOI) on the face may be Cordisco MR: Re: propranolol treatment for hemangioma of infancy: risks.

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Cardiac and cerebrovascular anomalies hemangooma the most common extracutaneous features of PHACE, and they also constitute the greatest source of potential morbidity. Congenital heart disease in PHACE cordisc incompletely described, and hemangima study was conducted to better characterize its features. Cardiac and aortic arch imaging with detailed assessment of arch patency and brachiocephalic origins is essential for any patient suspected of having PHACE.

Longitudinal investigation is needed to determine the long-term outcomes of cardiovascular anomalies in PHACE. The PHACE acronym was coined in to describe the association among malformations of the P osterior fossa, H emangiomas of the head and neck, A rterial, C ardiovascular, and E ye anomalies, and ventral developmental defects. In addition, the co-occurrence of cardiac anomalies with cervical and cerebral arterial anomalies significantly increases the risk of acute ischemic stroke in PHACE.

Our aim is to better define the incidence, characterize the clinical and radiologic features of, and provide follow-up data for cardiovascular anomalies in PHACE.

Hemanggioma available clinical, radiology, and pathology data for anatomic abnormalities of the heart, aortic arch, brachiocephalic vessels, and cerebrovascular bed was reviewed by a team of pediatric dermatologists and a pediatric cardiologist from the Medical College of Wisconsin.

Five subjects were excluded due to insufficient records Figure 1. Patent foramen ovale PFO was considered a normal variant and excluded.

A patent ductus arteriosus PDA was considered pathologic if it persisted past the neonatal period and was not associated with significant aortic arch obstruction or complex heart disease.

Pearson chi-square and Fisher’s exact tests were used to analyze data for hemangioma location and associated cardiovascular anomalies. Flowchart demonstrating subject inclusion process and cardiovascular anomaly frequencies.

Individual subjects may have more than 1 anomaly. Aortic coarctation histologic specimens from surgical resection were available for 7 of the 17 PHACE patients who had aorta removed during aortic arch reconstruction. These samples were reviewed centrally at the Medical College of Wisconsin and compared to control specimens from 52 consecutive surgical coarctation repairs non-PHACE performed at Children’s Hospital of Wisconsin.

Agminated Pyogenic Granuloma–Like Growth Arising in a Congenital Hemangioma

Figure 1 summarizes the cardiovascular anomalies: This ratio was similar to that of the entire registry 4. None of the PHACE patients’ aortic arch defects were associated with cordisvo aortic valve, mitral valve anomalies, or left ventricular hypoplasia. Three individuals required interposition grafts as infants because of the long-segment nature of the obstruction. Three dimensional MRI aortic reconstruction in this PHACE patient with severe coarctation with multiple areas of narrowing and aneurysms in the transverse arch as outlined by arrows in 2A.

Both subclavian arteries arise distal to areas of cprdisco arrows in 2Band the right subclavian artery has an aberrant origin. Of the 10 individuals with coarctation and follow-up data available, 7 have undergone surgical repair and 3 have been followed without intervention because of a mild gradient at presentation. Mean age at follow-up was 8 years range years. All of the patients without intervention have continued to demonstrate only mild arch gradients without progression, and none have required late intervention.


The 2 children who required interposition graft placement as infants have developed expected progressive narrowing with growth, and 1 graft has been replaced at 11 years of age.

Pathogenesis of infantile haemangioma

A vascular ring was present in 5 subjects all with a dominant right aortic archand 3 required surgical division. Finally, 7 subjects had ascending aortic dilatation without evidence of aortic valve pathology. Histologic examination of surgically excised aortic segments demonstrated strikingly similar abnormalities in the 7 PHACE specimens. The remaining 2 PHACE coarctation segments had smaller areas of decreased arterial smooth muscle cells in cordiscco tunica media and evidence of increased adventitial collagen deposition.

Inset shows elastin stain. In addition, there was intimal fibrosis and tunica media showing multiple fascicles of muscular elastic tissue occasionally extending from the ductus onto the adjacent aortic wall.

Although the adventitial layer appeared thicker in the PHACE specimens compared to the non-PHACE specimens, the majority of excised segments in both cohorts lacked extra-adventitial soft tissues so that analysis of adventitial thickness was difficult.

All PHACE patients with cardiovascular anomalies had a segmental infantile hemangioma of the head and neck region. Additionally, there was no ipsilateral association between the aortic arch direction and cervicofacial hemangioma location.

This study is the largest comprehensive investigation to date addressing cardiovascular anomalies in PHACE. In addition, these studies lacked detailed assessment of the brachiocephalic arterial and venous vessels and had ccordisco subjects than our cohort.

The incidence of cardiovascular anomalies in PHACE is significantly higher than the general population and many other syndromes well-known to be associated with congenital heart disease. Brachiocephalic vessel and aortic arch sidedness abnormalities are also much more common in PHACE than the general population. A review of CT angiograms of adults found aberrant subclavian artery in 1.

Coarctation or interrupted aortic arch in PHACE is unique and complex, both in its location and character, compared to typical coarctation anatomy. The coarctation is most often in the transverse aortic arch, rather than the aortic isthmus, and can be characterized by multiple areas of long segment arch narrowing with adjacent segments of bizarre aneurysmal dilatation Figure 2. Follow-up data fike a small hemangiomz of PHACE patients with coarctation cared for at the Medical Hemajgioma of Wisconsin did not show evidence of aortic arch narrowing; however, longer-term data from larger numbers of patients is needed to determine risk for progression.

Histopathologic examination of affected aortic tissue from 7 of our PHACE subjects demonstrated markedly similar zonal bands of smooth muscle loss, elastin loss, and fibrosis of the inner tunica media. These findings parallel other histologic descriptions of affected aortic and arterial tissue in PHACE. Although our series identified a few patients with structural venous anomalies, the clinical, radiologic, and histopathologic evidence suggests that the primary vascular defect in PHACE is developmental dysplasia of large and medium sized arteries.

The identification of ascending aortic dilatation without aortic valve disease also suggests a primary vasculopathy and emphasizes the need for long-term major artery surveillance of all PHACE patients.

Previous studies have suggested that IH location could predict the risk for associated anomalies. Hemangioma location should not dictate screening exams; all children with large segmental hemangiomas of the head and neck need a careful cardiac and aortic arch assessment. Limitations in this study included the retrospective study design and lack of longitudinal data on some patients. Some of the subjects enrolled in our registry have been previously reported in the literature.


Although it is possible that patients with more complex anomalies may be referred to our tertiary institution or to the registry, reviewing the entire registry population provided a large sample cordisck and allowed us to approximate the incidence of cardiovascular anomalies in PHACE. Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the NIH.

This is a PDF file of an unedited manuscript that has been accepted for publication. Gemangioma a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

National Center for Biotechnology InformationU. Author manuscript; available in PMC Dec BayerMD, a Peter C. CordiscoMD, d Ilona J. FriedenMD, e Deborah S. GoddardMD, f Kristen E.

HollandMD, a Alfons L. MetryMD, h Kimberly D.

Intense Pulsed-Light Therapy for Proliferative Haemangiomas of Infancy

MorelMD, i Paula E. SiegelMD, a and Beth A. DroletHemangjoma a. Cordisco d Hospital de Pediatria Prof Dr. Author information Copyright and License information Disclaimer. Garrahan, Buenos Aires, Argentina.

Cordisci publisher’s final edited version of this article is available at Am J Cardiol. See other articles in PMC that cite the published article. PHACE syndrome, congenital heart disease, cardiovascular anomalies, coarctation of the aorta.

Open in a separate window. Results Figure 1 summarizes the cardiovascular anomalies: Discussion This study is the largest comprehensive investigation to date addressing cardiovascular anomalies in PHACE. Vascular and nonvascular intracranial malformation associated with external capillary hemangiomas.

Case Reports in Dermatological Medicine

The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Coarctation of the aorta with congenital hemangioma of the face and neck and aneurysm or dilatation of a subclavian or innominate artery.

Stroke in children with posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities PHACE syndrome: Cerebral vasculopathy and neurologic sequelae in infants with cervicofacial hemangioma: Ipsilateral hemangioma fie aortic arch anomalies in posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, and cardiac defects and eye abnormalities PHACE anomaly: Interact Cardiovasc Thoracic Surg.

Haemangioma of the head and neck with corcisco involvement and atypical coarctation. A new syndrome of multiple hemangiomas, right dominant double aortic arch, and coarctation. J Thorac Cardiovasc Surg. Repair of a complex aortic arch anomaly associated with cutaneous hemangioma. Patterns of infantile hemangiomas: Am J Med Genet A. Cardiovascular anomalies in Turner syndrome: Mazzanti L, Cacciari E.

Congenital heart disease in patients with Turner’s syndrome. Variants and anomalies of thoracic vasculature on computed tomographic angiography in adults. J Comput Assist Tomogr. Support Center Support Center. Please review our privacy policy.