Problems in the orofacial region associated with Ehlers-Danlos and Marfan syndromes: a case series
Abstract
Ehlers-Danlos syndrome (EDS) and Marfan syndrome (MFS) are characterised by hypermobility of joints and cardiovascular morbidity, and typical orofacial signs and symptoms are associated with both. Basic knowledge of these should prevent late-stage diagnosis and enable adequate management. This case series comprises all EDS and MFS patients who consulted the Department of Oral and Maxillofacial Surgery at University Hospitals Leuven between 2005 and 2017. Thirty patients had EDS or MFS, and in seven the diagnosis was made based on temporomandibular dysfunction or craniofacial dysmorphism. Non-facial symptoms led to diagnosis in the remaining patients. Twenty-five interventions were followed by two infections and one haemorrhage. One patient developed trismus. No major complications were reported. The complications we observed did not seem to differ from those often seen in patients who did not have EDS or MFS, possibly as a result of our strictly applied precautions and postoperative instructions. However, our study of relevant publications shows that patients with EDS and MFS are a vulnerable group.
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Evidence Hub reads this as a case series, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
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