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.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.bjoms.2019.11.018 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Quality of life in people with syndromic heritable thoracic aortic disease and their relatives: a qualitative interview based study.
Orphanet J Rare Dis
January 2025
Department of Social Work, Child Welfare and Social Policy, Faculty of Social Science, Oslo Metropolitan University, Oslo, Norway.
Introduction: The purpose of this study was to investigate perceptions and opinions on what constitutes determinants for quality of life (QoL) in individuals with syndromic Heritable Aortic Disease (sHTAD), utilizing a qualitative study approach. Further to discuss clinical implications and direction for research.
Method: A qualitative focus group interview study was conducted of 47 adults (Marfan syndrome (MFS) = 14, Loeys-Dietz syndrome (LDS) = 11, vascular Ehlers Danlos syndrome (EDS) = 11, relatives = 11).
The impact of Marfan syndrome and Ehlers-Danlos syndrome on the risk of penile fracture in patients between 18 and 45 years.
J Sex Med
October 2024
Department of Urology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, 19107, United States.
Article Synopsis
- A study aimed to explore the prevalence of penile fractures (PF) in men with Ehlers-Danlos Syndrome (EDS) and Marfan Syndrome (MFS), revealing that these patients have significantly higher rates of PF compared to disease-free controls.
- The research analyzed a large database over 30 years, finding that men with EDS had a prevalence ratio of 30.18 and those with MFS had a ratio of 23.4 for PF.
- While the study highlights an important link between connective tissue diseases and sexual health issues, it is limited by its reliance on claims data, which lacks finer details on disease progression and complications.
Temporal Pattern Analysis of Ultrasound Surveillance Data in Vascular Connective Tissue Disorders.
Diagnostics (Basel)
August 2024
Department of Vascular and Endovascular Surgery, Klinik Ottakring, 1160 Vienna, Austria.
Article Synopsis
- - The study analyzes imaging and clinical data from 19 patients with vascular connective tissue disorders (vCTDs) to investigate the effectiveness of various imaging modalities and their impact on monitoring arterial diameter changes.
- - Findings show that nearly half of the patients experienced vascular events, and consistent ultrasound monitoring was more effective for tracking diameter progression than using multiple imaging methods.
- - The research emphasizes the need for standardized imaging protocols tailored to individual patient needs, suggesting further studies are necessary to improve surveillance strategies for vCTDs.
Fatigue in patients with syndromic heritable thoracic aortic disease: a systematic review of the literature and a qualitative study of patients' experiences and perceptions.
Orphanet J Rare Dis
May 2023
TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital, 1450, Nesoddtangen, Norway.
Introduction: The purpose of this study was to explore the literature on fatigue in patients with syndromic heritable thoracic aortic disease (sHTAD), including Marfan syndrome (MFS), Loeys-Dietz syndrome (LDS), vascular Ehlers Danlos syndrome (vEDS) and other sHTADs, critically appraise and synthesize the relevant literature. We also aimed to investigate how adults with sHTAD experience and perceive fatigue, and to discuss clinical implications and direction for further research.
Methods: First, a systematic review was performed by searching the published literature in all relevant databases and other sources until 20th October 2022.
Somatic symptoms, pain, catastrophizing and the association with disability among children with heritable connective tissue disorders.
Am J Med Genet A
July 2023
Center for Medical Genetics, Ghent University Hospital/Ghent University, Ghent, Belgium.
The aim of the present study was to investigate the nature and prevalence of nonspecific somatic symptoms, pain and catastrophizing in children with Heritable Connective Tissue Disorders (HCTD), and to determine their association with disability. This observational, multicenter study included 127 children, aged 4-18 years, with Marfan syndrome (MFS) (59%), Loeys-Dietz syndrome (LDS) (8%), Ehlers-Danlos syndromes (EDS) (12%) and hypermobile Ehlers-Danlos syndrome (hEDS) (23%). The assessments included the Children's Somatization Inventory or parent proxy (CSI, PCSI), pain visual-analogue scale (VAS), SUPERKIDZ body diagram, Pain Catastrophizing Scale Child or parent proxy (PCS-C, PCS-P) and Childhood Health Assessment Questionnaire (CHAQ-30).
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!