Isolated atlas-duplication as a manifestation of persistent proatlas: a case report.

PURPOSE: Atlas-duplication is an exceedingly rare dysplasia of the craniocervical junction. To the best of our knowledge, only two cases of atlas-duplication have been reported and these were associated with complete anterior rachischisis and os odontoideum. We aimed to report a case of isolated atlas-duplication of incidental finding and without attributable symptoms which makes it unique. METHODS: Following a normal coronarography for a suspected myocardial infarction, a 60-year-old-man with no significant medical history developed a transient ischemic attack that justified brain computed-tomography angiography. RESULTS: There was no evidence for cerebral ischemic lesion, intracranial occlusion or significant artery disease. Bone analysis revealed eight cervical vertebral segments with an additional vertebral level located between the occiput and the atlas. This vertebra presented all the morphological characteristics of an atlas vertebra except for hypoplasia of the left transverse process. An incomplete anterior rachischisis was associated, and there was no other abnormality of craniocervical junction. The clinical examination revealed no neck pain, no limitation of joint amplitude and no neurological deficit. Apart from preventive treatment of ischemic stroke, no orthopedic or surgical treatment was undertaken. After 1.5 years of radiological monitoring, the patient remains symptom-free. CONCLUSIONS: Atlas-duplication is an exceedingly rare dysplasia of the craniocervical junction that may be found isolated and incidentally. If this variation does not necessarily warrant specific treatment, brain CT angiography is recommended to detect anatomical variations of the vertebral arteries.

Lung Dual-Energy CT Perfusion Blood Volume as a Marker of Severity in Chronic Thromboembolic Pulmonary Hypertension.

In chronic thromboembolic pulmonary hypertension (CTEPH), assessment of severity requires right heart catheterization (RHC) through cardiac index (CI). Previous studies have shown that dual-energy CT allows a quantitative assessment of the lung perfusion blood volume (PBV). Therefore, the objective was to evaluate the quantitative PBV as a marker of severity in CTEPH. In the present study, thirty-three patients with CTEPH (22 women, 68.2 ± 14.8 years) were included from May 2017 to September 2021. Mean quantitative PBV was 7.6% ± 3.1 and correlated with CI (r = 0.519, p = 0.002). Mean qualitative PBV was 41.1 ± 13.4 and did not correlate with CI. Quantitative PBV AUC values were 0.795 (95% CI: 0.637-0.953, p = 0.013) for a CI ≥ 2 L/min/m2 and 0.752 (95% CI: 0.575-0.929, p = 0.020) for a CI ≥ 2.5 L/min/m2. In conclusion, quantitative lung PBV outperformed qualitative PBV for its correlation with the cardiac index and may be used as a non-invasive marker of severity in CTPEH patients.