Craniofacial morphology in a patient with Simpson-Golabi-Behmel syndrome

We present the case of a 6-year-old boy with a coarse face, cleft palate, and malocclusion with anterior open bite who had been diagnosed with Simpson-Golabi-Behmel syndrome. Morphology of the craniofacial structures was examined on the basis of conventional radiographs, three-dimensional (3D) compu...

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Bibliographic Details
Published in:The Cleft palate-craniofacial journal 2003-09, Vol.40 (5), p.550-555
Main Authors: TANIYAMA, Tomohide, KITAI, Noriyuki, IGUCHI, Yoshitaka, MURAKAMI, Shumei, YANAGI, Miho, TAKADA, Kenji
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - diagnosis
Biological and medical sciences
Cephalometry
Child
Cleft Palate - diagnostic imaging
Craniofacial Abnormalities - diagnostic imaging
Dentistry
Face - abnormalities
Face - diagnostic imaging
Facial bones, jaws, teeth, parodontium: diseases, semeiology
Humans
Investigative techniques, diagnostic techniques (general aspects)
Male
Malocclusion - diagnostic imaging
Malocclusion - therapy
Medical sciences
Non tumoral diseases
Otorhinolaryngology. Stomatology
Otorhinolaryngology. Stomatology. Orbit
Radiodiagnosis. Nmr imagery. Nmr spectrometry
Radiography, Panoramic
Syndrome
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Summary:We present the case of a 6-year-old boy with a coarse face, cleft palate, and malocclusion with anterior open bite who had been diagnosed with Simpson-Golabi-Behmel syndrome. Morphology of the craniofacial structures was examined on the basis of conventional radiographs, three-dimensional (3D) computed tomography (CT) and magnetic resonance (MR) scanning. This patient had 13 ribs on the right side, slight scoliosis, supernumerary nipples, a coarse face, hypertelorism, a short broad upturned nose, a wide mouth, a straight facial profile with incompetence of the lips, midline groove of tongue, and cleft palate. The patient also had severe anterior open bite, a distal step-type molar relationship, five congenitally missing teeth, and a supernumerary tooth. Lateral cephalometric analysis revealed a large anterior cranial base, a large maxilla and mandible, a large inferior face height, and skeletal Class I jaw relationship with a high mandibular plane angle and large gonial angle. The 3D CT image showed a large cranium, a long face height, and prominent skull sutures. The MR image showed a large tongue, midline groove of the tongue, and a small space between tongue and palate.
ISSN:1055-6656
1545-1569
DOI:10.1597/1545-1569(2003)040<0550:CMIAPW>2.0.CO;2