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European Archives of Paediatric Dentistry

, Volume 16, Issue 1, pp 63–66 | Cite as

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

  • M. BayramEmail author
  • M. Yildirim
  • F. Seymen
Case Report

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.

Keywords

Simpson–Golabi–Behmel syndrome Overgrowth syndrome Oral findings 

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Copyright information

© European Academy of Paediatric Dentistry 2014

Authors and Affiliations

  1. 1.Department of Pedodontics, School of DentistryIstanbul Medipol UniversityIstanbulTurkey
  2. 2.Department of Pedodontics, Faculty of DentistryIstanbul UniversityIstanbulTurkey

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