Patterns of Infantile Hemangiomas: New Clues to Hemangioma Pathogenesis and Embryonic Facial Development

Abstract

The patterns provide new clues regarding facial development. The observed patterns resemble previously described facial developmental units on the lower face but are distinctly different on the upper face. The patterns suggest that neural crest derivatives may play a role in the development of facial hemangiomas. Finally, these patterns (Seg1-Seg4) help standardize the nomenclature of facial segmental hemangiomas to analyze more effectively hemangioma risks and behavior.

Rapid Involuting Congenital Hemangioma in the Setting of PHACE Association

“…The maxillary region is analogous with S2, whereas S3 is mandibular and S4 frontonasal. 2,9 IH has been well described in patients with PHACE association, and a prospective study revealed that in infants with large facial hemangiomas, onethird had extracutaneous manifestations consistent with PHACE. 10 The definition of PHACE consists of the characteristic facial IH and at least 1 extracutaneous manifestation.…”

“…2 The majority of IH in PHACE is in the cervicofacial area and is usually of segmental morphology. 8,9 Segmental IH has been hypothesized to conform to a unique developmental unit, and there are 4 segments in which they tend to aggregate. 9 Our patient had an S1 lesion involving the frontotemporal region.…”

“…8,9 Segmental IH has been hypothesized to conform to a unique developmental unit, and there are 4 segments in which they tend to aggregate. 9 Our patient had an S1 lesion involving the frontotemporal region. The maxillary region is analogous with S2, whereas S3 is mandibular and S4 frontonasal.…”

Infantile Hemangioma

“…Miks tipte hem yüzeyel hem de derin doku tutulumu olduğu için her ikisinin de özelliklerini içeren, üzerinde keskin sınırlı kırmızı plak bulunan nodüller dikkati çeker (Şekil 2). Bir diğer sınıflama da İH'leri morfolojik olarak lokalize, segmental, belirsiz ve multifokal olmak üzere sınıflandırır 10,11 . Bu sınıflama prognoz açısından oldukça değerlidir.…”

Infantile haemangioma: harmless ‘strawberry' or life-threatening vascular anomaly?

“…Lesions which do not conform to these patterns are known as indeterminate. 8 This distinction is thought to be important prognostically as segmental haemangiomas are more often associated with extracutaneous anomalies, including spinal dysraphism, genitourinary anomalies and PHACE syndrome (posterior fossa malformations, haemangiomas, arterial anomalies, coarctation of the aorta and other cardiac defects, and eye abnormalities) ( …”