Brain gliomas,hydrocephalus and idiopathic aqueduct stenosis in children with neurofibromatosis type 1

PurposeTo evaluate the incidence and clinical importance of brain gliomas – optic pathway gliomas (OPGs) and especially gliomas outside the optic pathway (GOOP) for children with neurofibromatosis type 1 (NF1), additionally, to assess the causes of obstructive hydrocephalus in NF1 children with an emphasis on cases caused by idiopathic aqueduct stenosis.Subjects and methodsWe analysed data from 285 NF1 children followed up on our department from 1990 to 2010 by the same examination battery.ResultsWe have found OPGs in 77/285 (27%) children and GOOPs in 29/285 (10,2%) of NF1 children, of who 19 had OPG and GOOP together, so the total number of brain glioma was 87/285 (30,5%). GOOPs were significantly more often treated than OPGs (p > 0.01). OPGs contain clinically important subgroup of 14/285 (4.9%) spreading to hypothalamus. Spontaneous regression was documented in 4/285 (1.4%) gliomas and the same number of NF1 children died due to gliomas.Obstructive hydrocephalus was found in 22/285 (7.7%) patients and 14/22 cases were due to glioma. Idiopathic aqueduct stenosis caused hydrocephalus in 6/22 cases and was found in 2.1% of NF1 children. Two had other cause.ConclusionsThe total brain glioma number (OPGs and only GOOPs together) better reflected the overall brain tumour risk for NF1 children. However, GOOPs occur less frequently than OPGs, they are more clinically relevant. The obstructive hydrocephalus was severe and featuring frequent complication, especially those with GOOP. Idiopathic aqueduct stenosis shows an unpredictable cause of hydrocephalus in comparison with glioma and is another reason for careful neurologic follow up.     

Single-source dual energy CT to assess myocardial extracellular volume fraction in aortic stenosis before transcatheter aortic valve implantation (TAVI)

PurposeTo assess myocardial extracellular volume fraction (ECV) measurement provided by a single-source dual-energy computed tomography (SSDE-CT) acquisition added at the end of a routine CT examination before transcatether aortic valve implantation (TAVI) compared to cardiac magnetic resonance imaging (MRI).Materials and methodsTwenty-one patients (10 men, 11 women; mean age, 86 ± 4.9 years [SD]; age range: 71–92 years) with severe aortic stenosis underwent standard pre-TAVI CT with additional cardiac SSDE-CT acquisition 7 minutes after intravenous administration of iodinated contrast material and myocardial MRI including pre- and post-contrast T1-maps. Myocardial ECV and standard deviation (σECV) were calculated in the 16-segments model. ECV provided by SSDE-CT was compared to ECV provided by MRI, which served as the reference. Analyses were performed on a per-segment basis and on a per-patient involving the mean value of the 16-segments.ResultsECV was slightly overestimated by SSDE-CT (29.9 ± 4.6 [SD] %; range: 20.9%–48.3%) compared to MRI (29.1 ± 3.9 [SD] %; range: 22.0%–50.7%) (P < 0.0001) with a bias and limits of agreement of +2.3% (95%CI: −16.1%– + 20.6%) and +2.5% (95%CI: −2.1%– + 7.1%) for per-segment and per-patient-analyses, respectively. Good (r = 0.81 for per-segment-analysis) to excellent (r = 0.97 for per-patient-analysis) linear relationships (both P < 0.0001) were obtained. The σECV was significantly higher at SSDE-CT (P < 0.0001). Additional radiation dose from CT was 1.89 ± 0.38 (SD) mSv (range: 1.48–2.47 mSv).ConclusionA single additional SSDE-CT acquisition added at the end of a standard pre-TAVI CT protocol can provide ECV measurement with good to excellent linear relationship with MRI.     

Is T9-11 the true thoracolumbar transition zone?

ObjectiveDegenerative thoracic stenosis has been shown to most frequently involve the lower thoracic segments (T9-T12) where there is greater mobility and vulnerability due to flexion, extension and rotation of the spine. The thoracolumbar junction is considered anatomically to be T12-L1; the anatomical transition between the relatively immobile thoracic spine and relatively mobile lumbar spine. From anecdotal experience at our institution, we hypothesise that the true thoracolumbar junction is higher, at T10-11; the point of transition from floating to false ribs resulting in increased mobility at T10-11.Methods and materialsA retrospective review was performed of MRI lumbar and whole spine performed on patients aged 10–40 years in our institution over a 5-year period. Patients with previous surgery, chronic spinal disorders and congenital abnormalities were excluded from the study. Intervertebral discs from T8-9 to L1-2 were assessed for evidence of degeneration using the Pfirrmann grading system. Data obtained from a study using computer-based models to assess mean resultant loads in flexion, sitting and standing from T8-9 to L1-2 on patients aged 18–35 years was also analysed. The mean load gradients between two consecutive discs from T8 to L2 were analysed. Statistical analysis was performed with SPSS (p < 0.05 was considered statistically significant).ResultsThree-hundred and twenty-two MRI studies were reviewed. Mean Pfirrmann grade was highest at T8-9 and T9-10 (1.35 ± 0.99 and 1.32 ± 0.93 respectively).Pfirrmann grade differed significantly at each level (χ² = 45.137 p = 0.001). Difference in mean load gradient from T9 to T11 was significantly higher than mean load gradient across T11 to L1 in both sitting and standing (0.095 ± 0.062 vs 0.050 ± 0.044 kN; p = 0.007, and 0.101 ± 0.061 kN vs 0.040 ± 0.054 kN; p = 0.007).ConclusionThe changes in segmental loads and more severe disc degeneration at T9-11 compared to T11-L1 support our hypothesis that the true thoracolumbar transition is higher than expected, at T10-11; where the rib cage transitions from floating to false ribs.     

Endovascular Treatment Outcome and CT Angiography Findings in Acute Basilar Artery Occlusion with and without Underlying Intracranial Atherosclerotic Stenosis

PurposeTo compare clinical characteristics and treatment outcomes of intra-arterial thrombectomy (IAT) in acute basilar artery occlusion (BAO) with and without underlying intracranial atherosclerotic stenosis (ICAS) and to investigate the usefulness of preprocedural CT angiography findings in the diagnosis of ICAS.Materials and MethodsTwenty patients who received IAT for acute BAO between September 2014 and March 2019 were included. Additional therapies such as angioplasty, stent placement, and tirofiban infusion were provided while treating ICAS. Clinical and angiographic results of treatment were recorded. Preprocedural CT angiography findings in ICAS and non-ICAS groups were compared to assess (i) basilar tip opacification, (ii) partial occlusion, (iii) presence of convex border, (iv) occlusion segment longer than two thirds of the basilar artery or 20 mm, (v) dense basilar artery, and (vi) wall calcification in the occluded segment.ResultsAmong the 20 patients (mean age, 71.3 y; mean stroke score, 24.8), optimal recanalization was achieved in 19 (95%). Three patients had good clinical outcomes. There were 6 patients with underlying ICAS. No difference was observed between ICAS and non-ICAS groups in terms of optimal angiographic recanalization and good outcome. On CT angiography, basilar tip occlusion (100% vs 29%), partial occlusion (100% vs 83%), and long occlusion length (100% vs 14%) significantly differed between the groups (P ≤ .01).ConclusionsIn acute BAO, underlying ICAS does not affect optimal recanalization rate or clinical outcome. Preprocedural CT angiography is a potentially useful tool to detect it.