LAUSR

Dear Editor: We read with great interest the article by Dr. Foster and colleagues titled BPHACE syndrome is associated with intracranial cavernous malformations^ [1]. The authors state that four of the six patients who met criteria of PHACE syndrome had cerebral cavernous malformations (CCMs) identified on MR brain imaging. From these data, the authors state that there is an association between PHACE syndrome and CCMs, a finding not previously described in the literature. We would like to raise multiple important considerations to this possible association. First, the radiologic appearance of many of these foci of susceptibility artifact in the provided images may simply be microhemorrhages, not CCMs. Typically, CCMs are larger and exhibit significant blooming artifact. CCMs are also routinely observed on T1and T2weighted imaging, classically demonstrating a Bpopcorn^ appearance. The authors do not mention if these lesions were present on additional pulse sequences, but the reader assumes that they were not. Second, two of the four patients (cases 1 and 3) with reported CCMs had undergone prior cardiac surgery, which is known to result in cerebral microhemorrhages [2]. Case 1 shows several foci of susceptibility artifact involving typical distributions of the anterior and posterior circulation bilaterally, including the internal carotid artery watershed territory, which can often be seen with embolic phenomenon. The authors mention that these abnormalities were detected on the most recent MRI, suggesting that they were not present on the original examination at 2 months of age prior to surgery. Similarly, case 3 is also reported to have numerous supratentorial CCMs. We believe that these numerous foci of susceptibility artifact in both of these cases likely represent microhemorrhages from the prior cardiac surgery and not CCMs. Moreover, it is rare to have several CCMs at these young ages in the absence of a familial CCM syndrome. Third, we also question if all four of these patients truly meet criteria for PHACE syndrome. The authors report that all four patients met major criteria for the diagnosis of PHACE syndrome based on the cerebrovascular abnormalities; however, based on the information and images provided, we question the validity in cases 3 and 4. The authors state there is hypoplasia of the right A1 anterior cerebral artery segment in both of these cases as well as the right A2 segment in case 3. Figure 3, which corresponds to case 3, shows an arrow at the right A2 segment (called A1 segment in the legend) and does not appear hypoplastic. With regard to the A1 segment hypoplasia, this is a frequent normal variant in the general population and not a criterion for PHACE syndrome. Furthermore, these arterial abnormalities are contralateral to the facial hemangioma in both patients, which is quite uncommon in PHACE syndrome. We previously reported that in 54 patients with PHACE syndrome and unilateral arteriopathy that 98% * Mark D. Mamlouk [email protected]; [email protected]