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Trial Lecture – time and place
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Adjudication committee
- First opponent: Meike Rybczynski, University Medical Center Hamburg-Eppendorf,
- Second opponent: Professor Erik Magnus Berntsen, NTNU - Norwegian University of Science and Technology,
- Third member and chair of the evaluation committee: Professor Mona Elisabeth Skjelland, University of Oslo
Chair of the Defence
Professor II Helga Sanner, University of Oslo
Principal Supervisor
Senior consultant Rigmor Lundby, OUS - Oslo University Hospital
Summary
This thesis covers radiological manifestations in adults with Marfan syndrome (MFS) described in the revised Ghent criteria. MFS is a rare genetic connective tissue disorder.
Possible age-related changes of radiological manifestations in MFS patients need to be observed. The role of dural ectasia (DE) and protrusio acetabuli (PA) in the diagnosis of MFS is debated. Longitudinal and explorative studies on DE and PA are scarce.
The aims of the study were to provide new knowledge on radiological manifestations in adults with MFS over time and discuss the ability of DE and PA to discriminate between MFS patients and individuals without hereditary connective tissue disorder (HCTD).
The first part of the study was an observational study of changes in radiological manifestations in 46 adults with verified MFS. The study was designed as a 10-year follow-up with two observation points. We found most changes in the cardiovascular system, but we also observed that DE, scoliosis and pulmonary blebs could develop in adults and may progress over time. PA remained stable.
In the second part we explored our findings on DE and PA. Our observations on DE and PA in 58 participants with diagnosed HCTD were reported. Possible changes in DE and PA were analyzed in the 46 patients with MFS. Data from the ten-year follow-up of the MFS patients were compared to matched controls.
In our study, DE was observed in 45 participants with MFS after ten years, and in four of these, DE had developed during the 10 years. PA remained unchanged and was detected in 32 of the individuals with MFS. Both DE and PA showed good ability to discriminate between patients with MFS and individuals without any HCTD.
Overall MFS can evolve throughout life beyond cardiovascular changes and when MFS is suspected, imaging of the spine and hips can provide valuable information.
Additional information
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