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

Abstract：

OBJECTIVE: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. PATIENT: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.

journal_name

Cleft Palate Craniofac J

journal_title

The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association

authors

Taniyama T,Kitai N,Iguchi Y,Murakami S,Yanagi M,Takada K

doi

10.1597/1545-1569_2003_040_0550_cmiapw_2.0.co_2

keywords:

subject

Has Abstract

pub_date

2003-09-01 00:00:00

pages

550-5

issue

5

eissn

1055-6656

issn

1545-1569

journal_volume

40

pub_type

杂志文章

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