Marfan Syndrome—An Orthodontic Perspective

Abstract

Marfan syndrome is a heritable disorder of connective tissue that can affect the heart, blood vessels, lungs, eyes, bones, and ligaments. It is characterized by tall stature, elongated extremities, scoliosis, and a protruded or caved-in breastbone. Patients typically have a long, narrow face. A high-arched palate produced by a narrow maxilla and skeletal Class II malocclusion due to mandibular retrognathia are other common features. For a patient with no family history of the disorder, at least three body systems must be affected before a diagnosis can be made. Individuals affected by the syndrome routinely seek orthodontic treatment to correct the orofacial manifestations. In this report, the authors present the records of three patients with Marfan syndrome who were treated at a dental school. Two patients had severe periodontal disease in the absence of significant contributing local factors. The presentation of systemic symptoms and typical physical characteristics varied. The syndrome thus went unnoticed in one patient for many years. We discuss here the observed intraoral findings and the progress of orthodontic treatment to provide a brief overview of the challenges involved in treating such patients.

Periodontal conditions in patients with Marfan syndrome – a multicenter case control study

“…However, to date there is no data available in the dental/medical literature demonstrating a higher prevalence of periodontitis or more severe types of periodontitis in Marfan patients. Overall, various authors documented a higher prevalence of periodontitis in Marfan patients [12,21,22]. These studies always refer to reports published by Straub et al [11] or De Coster et al [10].…”

Association between Oro-Facial Defects and Systemic Alterations in Children Affected by Marfan Syndrome

“…A maxillary contraction and an ogival palate are also important factors from an orthodontic point of view, as they may determine a posterior cross-bite and a dental crowding. A dolichofacial aspect and a Class II skeletal malocclusion are often observed in patients with MFS [17]. Westling and colleagues [18] studied 76 patients with the MFS syndrome and detected a collapse of the dental arches with crowding and augmented over-jets in 70% of them.…”

Concomitant occurrence of hypohyperdontia in a patient with Marfan syndrome: a review of the literature and report of a case

“…They include constriction of the maxilla, a high-arched palate, crowding, and a posterior cross-bite. 3,[21][22][23][24][25][26][27][28][29][30][31] In the southern Chinese population, the reported prevalence of supernumerary teeth is 2.6%, and these supernumerary teeth are most commonly found in the anterior region of the maxilla. 18 To date, the dental literature on MFS has largely been confined to the reporting of the presence of a high palatal vault as a major clinical manifestation of the syndrome.…”