Alvarado GTJ, González EG, Soto CTA, Pérez CG

Language: Spanish
References: 7
Page: 1382-1385
PDF size: 217.12 Kb.

ABSTRACT

Introduction. Marfan syndrome is a hereditary connective tissue disorder that mainly affects the cardiovascular, ocular and musculoskeletal system, also known as "congenital mesodermal dystrophy" or "dolicostenomelia." It is characterized by a disproportionate growth and an enlargement of the fingers and toes, presenting class II skeletal patterns, dolicofacial biotype, micrognasia, maxillary atresia, deep palate, posterior crossbite, crowding, ATM subluxation with anterior displacement of the articular disc. The manifestation of the physical and systemic characteristics of this syndrome can vary from one patient to another. Usually these patients seek with orthodontic treatment the correction of the orofacial problems they present. Case report. We present the case of a patient with Marfan syndrome, treated in the orthodontics department of the Autonomous University of Baja California, whose characteristics are: skeletal class II, ligamentous hyperlaxity, convex profile, interlabial separation, neutral growth, severe dolicofacial biotype, Increased third third, collapse of both arches and severe crowding. Conclusions. Marfan syndrome develops abnormalities in the facial skeleton so it is common for patients to require orthodontic and/or surgical treatment. This must be done through a multidisciplinary team, always taking into account the special considerations that we must have in patients with this syndrome.

REFERENCES

1. Nemet AY, Assia EI, Apple DJ, Barequet, IS. Current Concepts of Ocular Manifestations in Marfan Syndrome. Sur Ophth 2006; 51(6): 561-75.

2. Achint Utreja and Carla A. Evans. (2009) Marfan Syndrome An Orthodontic Perspective. The Angle Orthodontist 79: 2: 394-400.

3. Disabella E, Grasso M, Marziliano N, Ansaldi S, Lucchelli C, et al. (2006). Two novel and one known mutation of the TGFBR2 gene in Marfan syndrome not associated with FBN1 gene defects. Eur. J. Hum. Genet. 14: 34-38.

4. Barriales-Villa R, García DA, Monserrat L. Genética del síndrome de Marfan. Cardiocore. 2011; 46: 1014.

5. Fortea-Sanchis C, Ángel Yepes V, Priego Jiménez P, Martínez- Ramos D, Escrig Sos J. Neumotórax y síndrome de Marfan. Cir Esp. 2015; 93: e87-e88.

6. Bertrand Moura, Florence Tubach, Moana Sulpice, Catherine Boileau, et al. Multidisciplinary Marfan Syndrome Clinic Group. Bone mineral density in Marfan syndrome. A large case-control study. Joint Bone Spine 73 (2006) 733-35.

7. Ana Lebreiro, Elisabete Martins, Cristina Cruz, Jorge Almeida, et al. Genotypic characterization of a Portuguese population of Marfan syndrome patients. Rev Portuguesa Cardiol 2011; 649-54.