Dental manifestations associated with Seckel syndrome type II: a case report.Pediatr Dent. 2010 Sep-Oct; 32(5):445-50.PD
Seckel syndrome is a rare form of primordial dwarfism that is characterized by short stature, skeletal defects, mental retardation, and characteristic facial features such as microcephaly, micrognathia, and a bird-head appearance. Dental findings include hypodontia, enamel hypoplasia, crowding, and Class II malocclusion. The purpose of this paper was to report the case of a female patient with Seckel syndrome type II and describe her orodental manifestations. She presented with interesting dental findings, including gingival hyperplasia, recession and ulceration, significant crowding, and early exfoliation of the primary dentition with accelerated eruption of the permanent dentition. The patient received comprehensive dental care under general anesthesia, and hard and soft tissue samples were collected for histologic analysis. The patient was followed for over 3 years.