EURORAD ESR

Case 10851

Morquio type B syndrome

Author(s)
Augsburg L1, Vanhoenacker FM1, 2, 3, Spaepen M1, Parizel PM1, Mortier G4

1University Hospital Antwerp, Department of Radiology, Wilrijkstraat 10, 2650 Antwerp (Edegem), Belgium; Email: filip.vanhoenacker@telenet.be
2University Hospital Gent, Department of Radiology, Gent, Belgium
3General Hospital Sint-Maarten, Department of Radiology, Duffel-Mechelen, Belgium
4University Hospital Antwerp, Department of Medical Genetics, Antwerp, Belgium
 
Patient
female, 14 year(s)
 
 
  • Figure 1
    Lateral plain radiograph of the cervical spine in flexion

    There is flattening of the vertebral bodies and hypoplasia of the dens (letter D). Note broad atlantodental interval (blue arrow).

     
    Area of Interest: Musculoskeletal bone; Musculoskeletal joint; Musculoskeletal spine; Imaging Technique: MR; Plain radiographic studies; Procedure: Diagnostic procedure; Education; eLearning; Special Focus: Congenital; Dysplasias; Genetic defects;
     
     
  • Figure 2
    Plain radiographs

    Plain radiographs of the thoracic (a) and lumbosacral (b) spine. Note platyspondyly and central vertebral „beaking” with tongue-like appearance (arrows).

     
    Area of Interest: Musculoskeletal bone; Musculoskeletal joint; Musculoskeletal spine; Imaging Technique: MR; Plain radiographic studies; Procedure: Diagnostic procedure; Education; eLearning; Special Focus: Congenital; Dysplasias; Genetic defects;
     
     
  • Figure 3
    Full-leg radiograph

    Genu valga.

     
    Area of Interest: Musculoskeletal bone; Musculoskeletal joint; Musculoskeletal spine; Imaging Technique: MR; Plain radiographic studies; Procedure: Diagnostic procedure; Education; eLearning; Special Focus: Congenital; Dysplasias; Genetic defects;
     
     
  • Figure 4
    Plain radiograph of the hand

    There is a distal ulnar and radial hypoplasia and delayed ossification of the carpals. Note also „squaring” of the metacarpal heads (arrows).

     
    Area of Interest: Musculoskeletal bone; Musculoskeletal joint; Musculoskeletal spine; Imaging Technique: MR; Plain radiographic studies; Procedure: Diagnostic procedure; Education; eLearning; Special Focus: Congenital; Dysplasias; Genetic defects;
     
     
  • Figure 5
    Plain radiograph of the pelvis

    Note flattening and fragmentation of the femoral heads (arrows) and shallow acetabuli.

     
    Area of Interest: Computer applications; Musculoskeletal bone; Musculoskeletal joint; Imaging Technique: MR; Plain radiographic studies; Procedure: Diagnostic procedure; Education; eLearning; Special Focus: Congenital; Dysplasias; Genetic defects;
     
     
  • Figure 6
    Coronal images

    Coronal fatsuppressed T2-WI (a) and coronal T1-WI (b) of the pelvis. There is marked destruction and subluxation of both femoral heads (arrows). Note also bilateral joint effusion.

     
    Area of Interest: Musculoskeletal bone; Musculoskeletal joint; Musculoskeletal spine; Imaging Technique: MR; Plain radiographic studies; Procedure: Diagnostic procedure; Education; eLearning; Special Focus: Congenital; Dysplasias; Genetic defects;
     
     
There is flattening of the vertebral bodies and hypoplasia of the dens (letter D). Note broad atlantodental interval (blue arrow).
 
Plain radiographs of the thoracic (a) and lumbosacral (b) spine. Note platyspondyly and central vertebral „beaking” with tongue-like appearance (arrows).
 
Genu valga.
 
There is a distal ulnar and radial hypoplasia and delayed ossification of the carpals. Note also „squaring” of the metacarpal heads (arrows).
 
Note flattening and fragmentation of the femoral heads (arrows) and shallow acetabuli.
 
Coronal fatsuppressed T2-WI (a) and coronal T1-WI (b) of the pelvis. There is marked destruction and subluxation of both femoral heads (arrows). Note also bilateral joint effusion.
 
 
 
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