Background and Purpose-PHACE is an acronym for posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Several case reports of arterial ischemic stroke (AIS) in individuals with PHACE have been published, but risk factors for AIS in PHACE have not been clearly defined. The objective of this article is to review all cases of stroke in PHACE in children and describe clinical characteristics that may be associated with an increased risk of AIS. Methods-A literature and registry search was conducted to identify patients with PHACE who had experienced AIS. Data were analyzed to determine age of onset, presenting signs and symptoms, and clinical features among this cohort compared with PHACE without AIS. Results-Twenty-two individuals with PHACE and AIS were identified. Imaging of the arteries of the head and neck was reported in 20 of 22. Narrowing or nonvisualization of at least 1 great cerebral vessel was present in 19 of 20 and of those, 15 had Ն2 vessels involved. Aortic arch anomalies were reported in 13 of 22 individuals. Conclusions-Aplasia, hypoplasia, or occlusion of a major cerebral artery appears to be a significant risk factor for AIS in children with PHACE, especially when Ͼ1 vessel is involved or if there is coarctation of the aorta. (Stroke. 2012;43:1672-1674.)Key Words: arterial ischemic syndrome Ⅲ hemangioma Ⅲ Pascual-Castroviejo Type II syndrome Ⅲ PHACE syndrome Ⅲ PHACES association Ⅲ propranolol F rieden coined the term PHACE syndrome to describe the association of infantile hemangiomas of the head and neck with developmental anomalies. 1 PHACE is an acronym for Posterior fossa brain malformations, Hemangiomas, Arterial anomalies, Coarctation of the aorta and cardiac defects, and Eye abnormalities. Approximately 30% of infants with large, facial hemangiomas meet diagnostic criteria for definite PHACE with the most common extracutaneous finding being abnormalities of the craniocervical arteries. The relationship between arteriopathy and arterial ischemic stroke (AIS), a rare but devastating complication affecting a subset of individuals with PHACE, is poorly understood.In this study we review all known cases of PHACE-related stroke to determine age of onset, presenting symptoms, clinical characteristics, and associated comorbidities to further understand potential risk factors. Methods Data Extraction and Methodologic QualityAbstracts were reviewed to identify cases of stroke, and the relevant articles were reviewed in full by 3 authors. A pediatric neurologist (H.J.F.) independently reviewed the selected articles to confirm the stroke diagnosis and data regarding cerebrovascular imaging. For inclusion in this analysis, stroke was defined by (1) clinical symptoms such as sudden onset of focal neurological deficit, headaches, loss of consciousness, or seizure; and (2) CT or MRI documenting a large-territory infarct for arterial ischemic stroke or intracerebral, subarachnoid, and/or intraventricular hemorrhage in a lo...

Celiac axis compression syndrome has generated much controversy since its original description in 1963. The main symptoms are postprandial epigastric abdominal pain, regurgitation of undigested food, and weight loss, all of which are caused by gastric ischemia from impingement of the celiac axis by the median arcuate ligament of the diaphragm. These symptoms are seen in other common disorders such as chronic mesenteric ischemia and gastroparesis. This makes the diagnosis of celiac axis compression syndrome a true challenge for the clinician. We present data on three patients successfully treated. The pre- and postoperative studies clearly demonstrate a resolution of the condition. The duplex ultrasound images clearly show variable compression on the celiac axis. The angiogram presented shows a classic image of the disease. A review of the data has enabled us to develop an algorithm for the diagnosis of this disease.

We describe the isolation of the melanized meristematic fungus Pseudotaeniolina globosa from the aortic wall of a patient who died while undergoing surgery for aortic aneurysm and aortic valve regurgitation as a result of dilated cardiomyopathy. Meristematic fungi related to P. globosa have until now been considered as environmental saprobes found predominantly in ecological niches with low water activity. The isolate was identified by phenotypic analyses and by sequencing of the rDNA internal-transcribed spacer domain. The clinical significance of this isolation remains unclear but isolation of meristematic fungi from clinical specimen should be thoroughly evaluated in terms of their significance in future.