Clinical and oral findings of a patient with Simpson-Golabi-Behmel syndrome.

Abstract

BACKGROUND:

The Simpson-Golabi-Behmel syndrome (SGBS) is an overgrowth condition characterised by macrosomia, mental deficiency, large head, prominent skull sutures, midface deficiency, hypertelorism, broad nose, wide mouth, macroglossia, malocclusion, highly arched palate, and musculoskeletal and limb abnormalities. The aim of this case report is to present clinical and oral findings of an 8-year-old boy who had been diagnosed with SGBS.

CASE REPORT:

This patient had supernumerary nipples on the right side, cubitus valgus webbed fingers, scoliosis, umbilical hernia, a coarse face, macrocephaly, hypertelorism, a short broad nose, a wide mouth, a straight facial profile and hearing loss. The patient also had macroglossia, diastemas, over-retained primary tooth, absent mandibular permanent central incisors, and highly arched palate. Lateral cephalometric analysis revealed a large anterior cranial base, a large maxilla and mandible, a large inferior face height, and skeletal Class III jaw relationship.

FOLLOW-UP:

After extraction of the over-retained primary central tooth, a partial prosthesis was fabricated in order to maintain function. The patient has been recalled regularly at 6-month intervals for 2 years. Over the following years the prosthesis was replaced due to facial growth.

CONCLUSION:

Long term follow-up is essential for the patient with SGBS. Preventive dental care, including oral hygiene instructions, diet counselling and the use of fluoride has been implemented.

• Authors: M Bayram, M Yildirim, F Seymen
• Journal: European archives of paediatric dentistry : official journal of the European Academy of Paediatric Dentistry (Eur Arch Paediatr Dent) Vol. 16 Issue 1 Pg. 63-6 (Feb 2015) ISSN: 1818-6300 [Print] England
• PMID: 25245233 (Publication Type: Case Reports, Journal Article)

Topics

• Anodontia (diagnosis)
• Arrhythmias, Cardiac (diagnosis)
• Cephalometry (methods)
• Child
• Denture, Partial, Removable
• Diastema (pathology)
• Follow-Up Studies
• Genetic Diseases, X-Linked (diagnosis)
• Gigantism (diagnosis)
• Heart Defects, Congenital (diagnosis)
• Humans
• Hypertelorism (diagnosis)
• Incisor (abnormalities)
• Intellectual Disability (diagnosis)
• Macroglossia (diagnosis)
• Macrostomia (diagnosis)
• Male
• Malocclusion, Angle Class III (diagnosis)
• Nose (abnormalities)
• Palate (abnormalities)
• Tooth, Deciduous (pathology)