Clinical and oral findings of a patient with Simpson–Golabi–Behmel syndrome
- 289 Downloads
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.
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.
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.
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.
KeywordsSimpson–Golabi–Behmel syndrome Overgrowth syndrome Oral findings
- Gorlin RJ, Cohen MM, Levin LS. Syndromes of the Head and Neck. Oxford: Oxford University Press; 1990. p. 666–73.Google Scholar
- Gruelich WW, Pyle SI. Radiographic atlas of skeletal development of the hand and wrist. In: Palo A, editor. Human Growth. Stanford: Stanford University Press; 1959. p. 54–5.Google Scholar
- Marinho VCC, Higgins JPT, Logan S, Sheiham A. Fluoride varnishes for preventing dental caries in children and adolescents. Cochrane Database Syst Rev. 2002;(3) Art No: CD002279.Google Scholar
- Marinho VC, Higgins JP, Logan S, Sheiham A. Fluoride mouth rinses for preventing dental caries in children and adolescents. Cochrane Database Syst Rev. 2003;(3) CD002284.Google Scholar