Orofacial Development and Craniofacial Manifestations of Silver-Russell Syndrome: Case Report

Abstract

Introduction: Silver-Russell syndrome is a heterogeneous disease in its clinical and genetic manifestations, feature prenatal and postnatal growth restriction, relative macrocephaly, body asymmetry and some typical facial characteristics that mainly include a small and triangular face, large mouth and inverted lip corners, prominent forehead, normal head circumference and micrognathism. Objective: To detail the craniofacial findings through the case presentation of a patient with the referred syndrome, attended by the Centrinho das Obras Sociais Irmã Dulce, in Salvador, Bahia, Brazil, with the purpose of guiding the dentist in relation to the diagnosis and possible therapeutic conducts. Case presentation: An eight-year-old female patient, diagnosed with Silver-Russell syndrome, who presented low birth weight and difficulty gaining weight since childhood. She presented the classic facial characteristics of the syndrome (triangular face, micrognathia, prominent frontal region, inverted lip commissures), in addition to the presence of cleft palate, a rare characteristic in the syndrome. The patient was scheduled for surgery to rehabilitate the cleft in the palate (after stipulated weight gain) and complementary ortho surgical support. Conclusions: in the scientific dental literature, there are few works that address the topic. We emphasise the need for early monitoring of these patients in order to identify, prevent and correct the manifestations caused by the syndrome.