Craniofacial Morphology and Dental Findings of Seckel Syndrome: Case Reports of Two Siblings

Seckel Syndrome (SS) is a rare form of primordial autosomal recessive dwarfism involving multiple malformations. The major characteristic features of SS are intrauterine and postnatal growth deficiency, severe microcephaly, craniofacial dysmorphism which includes characteristic ‘Bird-headed’ appearance, prominent nose, sloped forehead, receding jaw, low-set ears with hypoplastic lobules and large eyes with down-slanting palpebral fissures. Characteristic skeletal anomalies include premature closure of the cranial sutures and fifth finger clinodactyly. In addition to the characteristic craniofacial dysmorphism and skeletal defects, abnormalities have been described in the cardiovascular, hematopoietic, endocrine and central nervous systems. Dental abnormalities include enamel hypoplasia, hypodontia, microdontia, taurodontic root morphology and a high-arched palate. Retarded bone age and moderate to severe mental retardation (I.Q. < 50 in 50% of cases) are observed in patients with Seckel Syndrome. The purpose of this paper is to describe craniofacial morphology and dentition in two siblings with Seckel Syndrome and to present the dental treatments provided for these patients. Case report (J Int Dent Med Res 2011; 4: (3), pp. 139-144)