Pediatric Moyamoya Disease and Syndrome in Italy: A Multicenter Cohort.

Background: Moyamoya is a rare progressive cerebral arteriopathy, occurring as an isolated phenomenon (moyamoya disease, MMD) or associated with other conditions (moyamoya syndrome, MMS), responsible for 6-10% of all childhood strokes and transient ischemic attacks (TIAs). Methods: We conducted a retrospective multicenter study on pediatric-onset MMD/MMS in Italy in order to characterize disease presentation, course, management, neuroradiology, and outcome in a European country. Results: A total of 65 patients (34/65 women) with MMD (27/65) or MMS (38/65) were included. About 18% (12/65) of patients were asymptomatic and diagnosed incidentally during investigations performed for an underlying condition (incMMS), whereas 82% (53/65) of patients with MMD or MMS were diagnosed due to the presence of neurological symptoms (symptMMD/MMS). Of these latter, before diagnosis, 66% (43/65) of patients suffered from cerebrovascular events with or without other manifestations (ischemic stroke 42%, 27/65; TIA 32%, 21/65; and no hemorrhagic strokes), 18% (12/65) of them reported headache (in 4/12 headache was not associated with any other manifestation), and 26% (17/65) of them experienced multiple phenotypes (≥2 among: stroke/TIA/seizures/headache/others). Neuroradiology disclosed ≥1 ischemic lesion in 67% (39/58) of patients and posterior circulation involvement in 51% (30/58) of them. About 73% (47/64) of patients underwent surgery, and 69% (45/65) of them received aspirin, but after diagnosis, further stroke events occurred in 20% (12/61) of them, including operated patients (11%, 5/47). Between symptom onset and last follow-up, the overall patient/year incidence of stroke was 10.26% (IC 95% 7.58-13.88%). At last follow-up (median 4 years after diagnosis, range 0.5-15), 43% (26/61) of patients had motor deficits, 31% (19/61) of them had intellectual disability, 13% (8/61) of them had epilepsy, 11% (7/61) of them had behavioral problems, and 25% (13/52) of them had mRS > 2. The proportion of final mRS > 2 was significantly higher in patients with symptMMD/MMS than in patients with incMMS (p = 0.021). Onset age <4 years and stroke before diagnosis were significantly associated with increased risk of intellectual disability (p = 0.0010 and p = 0.0071, respectively) and mRS > 2 at follow-up (p = 0.0106 and p = 0.0009, respectively). Conclusions: Moyamoya is a severe condition that may affect young children and frequently cause cerebrovascular events throughout the disease course, but may also manifest with multiple and non-cerebrovascular clinical phenotypes including headache (isolated or associated with other manifestations), seizures, and movement disorder. Younger onset age and stroke before diagnosis may associate with increased risk of worse outcome (final mRS > 2).

Neuroembolization may expose patients to radiation doses previously linked to tumor induction.

OBJECTIVE: Epidemiological studies indicate a link between low-dose irradiation (<10,000 mGy) to the head and the local occurrence of tumors after decades of delay. Comparable radiation doses can be reached during neuro-endovascular procedures (NEP), but the incidence of similar exposures has not been completely delineated. We compared the levels of radiation to the head measured during NEP to those reported for patients developing radiation-induced cancers. METHODS: In our prospective study we determined the cumulative maximum entrance skin doses (MESD) and the incidence of epilation in 107 consecutive patients submitted to NEP between 2003 and 2007. We also extensively searched the literature and compared our results with the data we found. RESULTS: The cumulative MESD due to NEP was above 3,000 mGy (range 3,101-5,421 mGy) in 18 patients. In 22 we observed partial epilation within 10 weeks from the initial NEP. Sixty cases of epilation after NEP have been previously reported in the literature. The average of the reported MESD was 4,241 mGy (range 2,000-6,640 mGy). CONCLUSION: Physical dosimetry and the incidence of partial epilation indicate that about one fifth of the patients submitted to NEP received radiation doses comparable to those linked to the occurrence of tumors. The potential risks of developing tumors after a long delay, when compared to the immediate benefits of endovascular treatment of aneurysm and arteriovenous malformations (AVM) of the brain, do not counterindicate NEP, but increased awareness of the risk should help physicians and patients to make a fully informed decision when other treatments are available.

Radiochromic films for improved evaluation of patient dose in liver interventions.

PURPOSE: To investigate a method for evaluation of the maximum skin dose (MSD) of radiation in patients undergoing interventional radiology procedures in the liver. MATERIALS AND METHODS: Transarterial chemoembolization, transarterial embolization, portal vein embolization, and biliary interventions were the procedures considered in this study. Ninety procedures in 70 patients were studied. The complexity of the biliary interventions was taken into account during the analysis. The MSD was measured with use of GAF chromic XR type R films, whereas the dose-area product (DAP) was measured by a transmission chamber. RESULTS: The MSD was measured in a group of 19 patients. The coefficient of the interpolation line between the skin dose and the DAP (0.0051 cm(-2)) was determined. An approximated value of MSD from the DAP for the remaining 71 procedures was estimated by means of an interpolation line. The mean MSD in the endovascular procedures was 4.4 Gy; for the three different levels of complexity of the biliary procedures, the mean MSDs were 0.4, 1.2, and 3 Gy, respectively. CONCLUSIONS: Radiochromic films are an easy-to-use and efficient method for measuring skin entrance radiation dose and have the advantage of providing information on the MSD as well as the distribution of radiation to the skin. In light of these results, the authors suggest recording the dose for biliary and endovascular procedures as indicated by the International Commission on Radiological Protection and the United States Food and Drug Administration.