IADR Abstract Archives

Seckel syndrome: Dental Manifestations and Management

Introduction:The Seckel syndrome [OMIM 210600] is an uncommon autosomal recessive genetic disorder, which is characterized marked intrauterine and postnatal growth retardation resulting in a proportionate slight stature, microcephaly, mental retardation and unusual facies. A receding forehead and beaked nose are consistent craniofacial features. Oro-dental abnormalities, including tooth crowding, malocclusion and micrognathia are significant syndromic components, which may warrant specialized dental measures. Dental and aneasthetic intervention may be difficult because of intellectual compromise, slight stature and involvement of other systems. Objective: To document and depict the phenotypic manifestations in an affected boy, with emphasis upon his orofacial features. A proposed protocol for dental management is outlined. Methods: The affected boy attended the Dental-Medical Genetics Unit at Red Cross Children's Hospital for routine observation. Craniofacial and oral abnormalities necessitated that he was referred to the Dental Faculty of the University of the Western Cape for management. Standardized radiographs and routine clinical examination and treatment were performed. Results: The affected child was severely mentally handicapped making both radiographic and clinical examination extremely difficult. Caries was the most prominent dental problem at this stage. Routine dental treatment could only be performed under conscious sedation. Conclusion: The maintenance of good oral health is a significant challenge in individuals affected by Seckel syndrome. It is therefore vital to motivate and educate both the parents and affected children about the importance of the prevention of dental caries and periodontal disease.
South African Division
2006 South African Division (Midrand, South Africa)
Midrand, South Africa
2006

Scientific Groups
  • Roberts, T.  ( University of Western Cape, Cape Town, N/A, South Africa )
  • Fakir, Ebrahim  ( University of Western Cape, Cape Town, N/A, South Africa )
  • Oral Session
    Craniofacial Biology