Predicting Ischemic Risk Using Blood Oxygen Level-Dependent MRI in Children with Moyamoya.

BACKGROUND AND PURPOSE: Moyamoya is a progressive steno-occlusive arteriopathy. MR imaging assessment of cerebrovascular reactivity can be performed by measuring the blood oxygen level-dependent cerebrovascular reactivity response to vasoactive stimuli. Our objective was to determine whether negative blood oxygen level-dependent cerebrovascular reactivity status is predictive of ischemic events in childhood moyamoya. MATERIALS AND METHODS: We conducted a retrospective study of a consecutive cohort of children with moyamoya who underwent assessment of blood oxygen level-dependent cerebrovascular reactivity. The charts of patients with written informed consent were reviewed for the occurrence of arterial ischemic stroke, transient ischemic attack, or silent infarcts. We used logistic regression to calculate the OR and 95% CI for ischemic events based on steal status. Hazard ratios for ischemic events based on age at blood oxygen level-dependent cerebrovascular reactivity imaging, sex, and moyamoya etiology were calculated using Cox hazards models. RESULTS: Thirty-seven children (21 female; median age, 10.7 years; interquartile range, 7.5-14.7 years) were followed for a median of 28.8 months (interquartile range, 13.7-84.1 months). Eleven (30%) had ischemic events, 82% of which were TIA without infarcts. Steal was present in 15 of 16 (93.8%) hemispheres in which ischemic events occurred versus 25 of 58 (43.1%) ischemic-free hemispheres (OR = 19.8; 95% CI, 2.5-160; P = .005). Children with idiopathic moyamoya were at significantly greater risk of ischemic events (hazard ratio, 3.71; 95% CI, 1.1-12.8; P = .037). CONCLUSIONS: Our study demonstrates that idiopathic moyamoya and the presence of steal are independently associated with ischemic events. The use of blood oxygen level-dependent cerebrovascular reactivity could potentially assist in the selection of patients for revascularization surgery and the direction of therapy in children with moyamoya.

Non-hodgkin B-cell lymphoma of the ovary in a child with Ataxia-telangiectasia.

BACKGROUND: Ataxia-telangiectasia is a multisystem, life-limiting, recessively inherited genetic disorder caused by mutations in the Ataxia-telangiectasia mutated gene. It is characterized by the onset of changes in neurological and immunological development, organ maturation in childhood, as well as a high incidence of malignancies. CASE: We describe a case of an 11-year-old girl with a history of progressive ataxia and new finding of bilateral pelvic masses. Given an elevated alpha-fetoprotein, the pre-operative working diagnosis was a malignant germ cell tumor. Final ovarian pathology revealed a non-Hodgkin B-cell lymphoma with Burkitt-like morphology. SUMMARY: We present the first case of a primary ovarian non-Hodgkin B-cell lymphoma in a child with Ataxia-telangiectasia.

Frequency and topographic distribution of brain lesions in pediatric cerebral venous thrombosis.

BACKGROUND AND PURPOSE: Cerebral sinovenous thrombosis (CSVT) is increasingly encountered in children, including neonates. The purpose of this study was to assess the frequency and topographic distribution of parenchymal brain lesions associated with CSVT in children and to compare these with the known anatomic venous drainage pathways. MATERIALS AND METHODS: Brain CT/CT venograms and/or MR imaging/MR venograms of 71 consecutive patients with CSVT were reviewed retrospectively. The patients were grouped into neonates, infants, and older children. The site of CSVT, the location and size of the brain lesions, and the presence of hemorrhage were documented. The frequency of the brain lesions was calculated. RESULTS: There were 34 neonates, 10 infants, and 27 older children with CSVT who were included. The most common sites of CSVT were the transverse sinuses, the superior sagittal sinus, and the straight sinus. Overall, 37 of 71 children with CSVT had parenchymal brain lesions. There were 21 of 34 neonates, 4 of 10 infants, and 12 of 27 older children who had brain lesions. The most common locations were in the frontal and parietal lobes. The topographic distribution of lesions correlated with the corresponding venous drainage territory in 16 of 21 neonates, all infants, and all older children. The neonates had smaller-sized lesions. Brain lesions were hemorrhagic in 76% of neonates, 75% of infants, and 33% of older children. CONCLUSION: The topographic distribution of brain lesions associated with CSVT correlates with the known drainage territories of the dural venous sinus in children.