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Journal of Stomatology Oral and Maxillofacial Surgery
Volume 120, n° 2
pages 110-115 (avril 2019)
Doi : 10.1016/j.jormas.2018.10.011
Received : 12 June 2018 ;  accepted : 28 October 2018
Original Articles

Contribution of a better maxillofacial phenotype in Silver–Russell syndrome to define a better orthodontics and surgical management

S. Vo Quang a, E. Galliani a, b, S. Eche a, b, C. Tomat a, b, B. Fauroux c, d, A. Picard a, b, d, N. Kadlub a, b, d,
a AP–HP, Necker–Enfants-Malades, service de chirurgie maxillo-faciale et chirurgie plastique, 75015 Paris, France 
b AP–HP, Necker–Enfants-Malades, Centre de référence des fentes et malformations faciales, 75015 Paris, France 
c AP–HP, Necker–Enfants-Malades, unité de ventilation non invasive et du sommeil de l’enfant, 75015 Paris, France 
d Université Paris Descartes, 75006 Paris, France 

Corresponding author at: Service de chirurgie maxillofaciale et chirurgie plastique, AP–HP, Necker–Enfants-Malades, 149, rue de Sèvres, 75015 Paris, France.Service de chirurgie maxillofaciale et chirurgie plastiqueAP–HPNecker–Enfants-Malades149, rue de SèvresParis75015France

Maxillofacial phenotype for SRS is incompletely described in literature. The aim of this study was to describe a maxillofacial phenotype for SRS, to determine a better treatment.

Materials and methods

A retrospective study was conducted including 37 patients with SRS. 24-control patients had been included and appareled. The subjective clinical examination included analyzes of SRS defined criteria. Frontal and lateral photographs had been reviewed, according to Farkas analysis; dental photographs had been examined for the deep-bite and the crowding severity. Radiologic cephalometric analysis had been reviewed.


Maxillofacial examination showed protruding forehead (55%), anteverted ears (55%) and low-set ears (16%), small triangular face (48%); retrognatia (29%) and micrognathia (13%). SSR patients presented a lower forehead transverse growth, forehead height, and higher sagittal and transverse mandibular growth than control patients. Deep-bite was present in 21 patients of patient, and crowding in 17 patients. Cephalometric analysis showed 18 patients with the skeletal class II. We did not note a correlation between sleep apnea and retrognatia, neither between genetic anomalies and craniofacial phenotype.


In this study, we showed new SRS characteristics: small forehead, small mandible, skeletal class II and a dental phenotype, leading to a specific maxillofacial and orthopedic management.

The full text of this article is available in PDF format.

Keywords : Silver–Russell syndrome, Maxilla-facial phenotype, Retrognatia

© 2018  Published by Elsevier Masson SAS.