Mittelgesichtshypoplasie
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Aberrant
growth of the anterior cranial base relevant to severe midface hypoplasia of Apert syndrome. Panoramic view. a An Apert syndrome patient, a 9-year-old male, with an upward convex palatal plate (arrows) and a short and thin nasal septum (arrow heads). Noted the severe anterior open bite (*). b A 9-year-old normal male with normal horizontal palatal plate (arrows) and a long and thick nasal septum (arrow heads)
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Aberrant
growth of the anterior cranial base relevant to severe midface hypoplasia of Apert syndrome. Water’s view: An Apert syndrome patient (a), a 9-year-old male, showed a shorter zygomatico-maxillary width and a shorter and thinner nasal septum than a 9-year-old normal male (b)
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Aberrant
growth of the anterior cranial base relevant to severe midface hypoplasia of Apert syndrome. Cephalogram: a An Apert syndrome patient, a 9-year-old male, noted with retained suture wires for distraction osteotomy performed at 6 months after birth. b A 9-year-old normal male. c overlapping cephalogram panels a and b by adjusting to the SN line. The Apert syndrome patient showed an increased posterior-anterior cranial base angle (angle PC-S-AC), a retruded maxilla, and a counter-clockwise growth of the mandible compared to the control
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Aberrant
growth of the anterior cranial base relevant to severe midface hypoplasia of Apert syndrome. Posterior to anterior (PA) view. a An Apert syndrome patient, a 9-year-old male. b A 9-year-old normal male. c Overlapping panels a and b by adjusting the line between the bilateral jugular points. The Apert syndrome patient showed orbital hypertelorism, less descended nasal floor, and downward growth of the mandible compared to the control
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Aberrant
growth of the anterior cranial base relevant to severe midface hypoplasia of Apert syndrome. Basal cranial view. a An Apert syndrome patient, a 9-year-old male. b A 9-year-old normal male. c Overlapping panels a and b. The Apert syndrome patient showed increased zygomatic axis angle of the cranial base (angle Z1-S-Z2) and decreased otic axis angle of the cranial base (angle O1-Oc-O2) compared to the control
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Aberrant
growth of the anterior cranial base relevant to severe midface hypoplasia of Apert syndrome. Coronal sutures in PA X-ray views. a A 9-year-old normal male with still immature coronal suture (arrows). b An Apert syndrome patient, a 9-year-old male, with tightly interdigitated coronal suture (arrows) similar to that of a 30-year-old normal male (c)
Midfacial hypoplasia is a type of congenital facial anomaly and can be feature of a number of congenital syndromic anomalies. These include:
- Antley-Bixler syndrome
- chromosome 1p36 deletion syndrome
- chromosome 18q deletion syndrome
- Crouzon syndrome
- fetal alcohol syndrome
- fetal valproate syndrome
- fetal Warfarin syndrome
- holoprosencephaly spectrum
- Stickler syndrome group
Assocations
Associated features that may be seen with midfacial hypoplasia include:
Siehe auch:
- Holoprosencephalie
- Fetales Valproat-Syndrom
- Crouzon-Syndrom
- De-Grouchy-Syndrom Typ 2
- Fetales Alkoholsyndrom
- Alport syndrome
- Stickler-Syndrom
- Fetales Warfarinsyndrom
- Antley-Bixler-Syndrom
- short maxilla
und weiter:
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