Hemangiomas y malformaciones vasculares cutáneas y patología asociada (síndrome de Pascual-Castroviejo tipo II). Presentación de 41 pacientes

Cutaneous, Mediastinal and Hepatic Hemangiomas in a Girl Followed During 12 Years

“…Despite the existence of a progressive decrease of the hemangioma size in the 3 locations (skin, liver and mediastinum) in all the MRI controls performed every year, the decrease in size has been very slow and, at 12 ½ years old, some residual hemangioma can be observed in all the 3 locations (skin, liver and mediastinum). The patient also showed psychomotor delay with low-normal or borderline levels, as has been commonly described in most patients with Pascual-Castroviejo II syndrome (P-CIIS) [6,8,9] and in 40 -60 % of individuals with NF1 (most of them also have ADHD).…”

Association of Cutaneous Red-to-Purple Hemangiomas with Leptomeningeal Hemangiomas. A Clinical Study of Two Patients

“…The association of cutaneous hemangiomas with other extracranial and intracranial vascular and non-vascular anomalies has been known since 1978 [6] . In P-CIIS, vascular abnormalitites can be localized not only in the central nervous system (CNS), but also anywhere in the body [7,11] . The P-CIIS is the most frequent neurocutaneous syndrome, even more frequent than the NF1.…”

“…Both vascular anomalies are associated with similar pathologies but they also have some diff erences. The similarities include an association with persistent embryonic arteries involving mostly the trigeminal territory, absence or hypoplasia of some of the main cerebral arteries (carotids and / or vertebrals), mostly ipsilateral to the facial vascular anomaly and malformations of the CNS, located mainly in the cerebral hemispheres [11] . The main diff erences are that hemangiomas frequently are associated with visceral hemangiomas and / or intracorporeal arteries with hemangiomatous and / or aneurysmal propensities that undergo the same process of increasing-regressing as the cutaneous hemangiomas, whereas the vascular malformations are not associated with these types of tumors.…”

“…The association of facial hemangioma with abnormalities of the intra-and extracerebral vessels was fi rst described in 1978 [6] . The disorder, known as " cutaneous hemangiomas: vascular anomaly complex " [7,11] or PHACE (this acronym was used by Frieden et al, in 1996 [3] , to denote the major features reported by Pascual-Castroviejo in 1978 [6] in the original descriptive paper; P derives from posterior fossa malformations, from hemangioma, A from arterial anomalies, C from coarctation of the aorta and other cardiac defects; and they added E from eye abnormalities, but this is and acquired features, mostly secondary to eye closure by the palpebral hemangioma) [3] , and Pascual-Castroviejo type II syndrome (PCIIS) [1,13] (OMIM# 606510) is one of the most common neurocutaneous disorders [3,7,11] . Cutaneous and internal hemangiomas Cutaneous hemangioma is a benign vascular tumor of infancy with an initial proliferating period that appears between 1 to 2 weeks of life, extends during 18 months to 2 years of life, and then slowly regresses during several years until it disappears completely.…”

Segmental Neurofibromatosis Type 1 (NF1) Associated with Cobb Syndrome: Case Report

“…Cobb described the association of skin naevi in the midposterior thoracic area and venous or arteriovenous malformations and possible compression of the spinal cord [3] . This disorder may correspond to a localized type of the Pascual-Castroviejo type II syndrome (P-CII-S) [10,11] (also known as PHACE). We are not aware of any report in which the association of both neurocutaneous disorders has been previously described.…”