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Loeys-Dietz syndrome (LDS) is an increasingly recognized autosomal-dominant connective tissue disorder with distinctive radiological manifestations, including arterial tortuosity/aneurysms, craniofacial malformations and skeletal abnormalities. LDS exhibits a more aggressive course than similar disorders, such as Marfan or the vascular subtype of Ehlers-Danlos syndrome, with morbidity and mortality typically resulting from complications of aortic/arterial dissections. Early diagnosis, short-interval follow-up imaging and prophylactic surgical intervention are essential in preventing catastrophic cardiovascular complications. This review focuses on the cardiovascular, neuroradiological and musculoskeletal imaging findings in this disorder and recommendations for follow-up imaging.

Abstract: Objective: To evaluate value of adding non-contrast MR angiographic sequence (In-Flow Inversion Recovery [IFIR]) to standard fat-suppressed T1-weighted postcontrast sequence (3D spoiled gradient echo [3D-GRE]) for evaluating hepatic arterial anatomy. Methods: Retrospective evaluation of 30 consecutive patients undergoing multiphase liver MRI. Individual vessels for IFIR/3D-GRE sequences were evaluated by two blinded readers using a four-point scale. Statistical analysis was performed using the Wilcoxon signed-rank test for vessel conspicuity between IFIR/3D-GRE sequences. Results: IFIR alone diagnostically imaged 8.1% of vessels, 3D-GRE alone 25.8%, 55.8% by both 3D-GRE/IFIR, and 10.3% of vessels by neither. Two patients with variant vascular anatomy were visualized with both sequences. Addition of IFIR to 3D-GRE resulted in statistically significant increase in arterial visualization (p <0.001), 10% relative increase in identified vessels, and 3–5mi increase in acquisition time for total scan time of 30–35min. Conclusions: IFIR may be a useful adjunct to 3D-GRE in hepatic angiography without adding considerably to scan time. 10% more hepatic arteries were seen when combining information from IFIR/3D-GRE vs. 3D-GRE alone. [Copyright &y& Elsevier]

Abstract: Background: Magnetic resonance imaging has the diagnostic advantages of being noninvasive and able to visualize soft tissue. However, conventional recumbent MRI may underestimate a disease because the position of imaging takes stress off the spine. Case Description: A 37-year-old woman presented with complaints of pain in the neck that radiated down her right arm when she turned her head to the right and increased with extension. She complained of paresthesias, numbness/tingling in the index and middle fingers and thumb, in the C6 and C7 nerve root dermatomes. Conventional conservative measures, including anti-inflammatories, muscle relaxants, opiates, and physical therapy, had been tried without positive results. Magnetic resonance imaging was performed in a weight-bearing upright neutral position, in the extended upright position, and in the extended upright position with the head turned to the right. The latter images showed a clear protrusion at C5-C6 and C6-C7. These protrusions were not clearly evident in the upright neutral position. A targeted epidural block at C5-C6 and C6-C7 relieved the patient''s pain, and she has been able to continue work. Conclusions: Magnetic resonance imaging of the cervical spine in the position that causes the patient''s symptoms may increase the sensitivity and accuracy of the diagnostic study and thus provide the spine-care professional with a potentially more accurate diagnosis and a targeted treatment plan. Such MRIs may also decrease the need for myelography. [Copyright &y& Elsevier]