MyPACS.net: Radiology Teaching Files > Case 13907505

previously visited 070828 CLEIDOCRANIAL DYSPLASIA
Contributed by: pedrad CSH, Radiologist, Chung Shan Medical University Hospital, Taiwan.
Patient: 14 year old male
History: Short status and abnormal thoracic cage.
Images:[small]larger

Fig. 1: Pelvis X-ray

Fig. 2: Pelvis X-ray (normal case and patient)

Fig. 3: Chest X-ray

Fig. 4: CT scan of facial bones_3D volume rendering (VR)

Fig. 5: CT scan of facial bones_axi

Fig. 6: CT scan of facial bones_axi

Fig. 7: CT scan of facial bones_axi

Fig. 8: CT scan of facial bones_axi

Fig. 9: CT scan of facial bones_axi

Fig. 10: CT scan of facial bones_sagittal reconstruction

Fig. 11: CT scan of facial bones_thick curved multiplanar reconstruction (cMPR)(normal case)

Fig. 12: CT scan of facial bones_thick curved multiplanar reconstruction (cMPR)(patient)

Fig. 13: CT scan of facial bones_axi

Fig. 14: CT scan of facial bones_average intensity projection (AIP)

Fig. 15: CT scan of facial bones_maximum intensity projection (MIP)

Fig. 16: CT scan of facial bones_3D volume rendering (VR)

Fig. 17: CT scan of facial bones_3D volume rendering (VR)

Fig. 18: CT scan of facial bones_3D volume rendering (VR)

Fig. 19: CT scan of facial bones_3D volume rendering (VR)

Fig. 20: CT scan of facial bones_3D volume rendering (VR)

Fig. 21: CT scan of facial bones_3D volume rendering (VR)

Fig. 22: CT scan of facial bones_3D volume rendering (VR)

Fig. 23: CT scan of facial bones_3D volume rendering (VR)

Fig. 24: CT scan of facial bones_3D volume rendering (VR)

Fig. 25: CT scan of facial bones_coronal reconstruction

Fig. 26: CT scan of facial bones_coronal reconstruction
Findings:

Pelvis X-ray (fig.1,2):
1. Coxa vara of both hips.
2. Delayed ossification of pubic bones.
3. Wide symphysis pubis.
4. Narrow bilateral iliac wings.

Chest X-ray (fig.3):
1. Hypoplasia of bilateral clavicles with symmetric absence of
   bilateral distal clavicles.
2. Somewhat bell-shaped thorcic cavity.

CT scan of facial bones with multiplanar reconstruction, thick
 curved multiplanar reconstruction (cMPR), maximum-intensity
 projection (MIP), 3D volume rendering (VR), and without
 intravenous contrast (fig.4-26) :

1. Multiple mosaic accessory bone plates along sagittal and
   bilateral lambdoid sutures with somewhat symmetric distribution,
   in favor of multiple wormian (sutural) bones, possibly
   associated with some kinds of skeletal abnormality (fig.4).
   (Differential diagnosis: osteogenesis imperfecta, cleidocranial
   dysplasia, hypothyroidism, etc).
2. Persistent synchondrosis in skull (fig.4), facial bones, and
   cervical vertebrae (fig.5-9) with paired frontal bones due to
   obvious metopic suture from closed anterior fontanel to frontonasal
   suture (fig.4).
3. Small size of bilateral maxillary sinuses and undevelopment
   of frontal and sphenoid sinuses (fig.10), suggesting some degree
   facial bone hypoplasia.
4. Complete nondevelopment of bilateral mastoid air cells (fig.9).
5. Delay detachment of right lower deciduous canine and fist and
   second deciduous premolars with uneruption of corresponding
   permanent teeth (fig.12).
6. Delay detachment of left lower deciduous canine and fist
   deciduous premolar with uneruption of corresponding permanent
   teeth (fig.12).
7. Supernumerary teeth within maxilla with irregular arrangement
   (fig.13-24).
8. Decreased ossification of hyoid (fig.5-6,13,26);
   Steep vertical clivus (fig.10);
   High anterior arched hard palate (fig.25-26).

Impression:
1. Multiple abnormalities in skull, facial bones, and vertebrae.
2. All of the findings could be compatible with cleidocranial
   dysplasia.

Diagnosis: Cleidocranial dysplasia
Comments:
No comments posted.
Additional Details:

Case Number: 13907505Last Updated: 03-20-2008
Anatomy: Skeletal System   Pathology: Normal/Variants
Modality: CT, Conventional Radiograph, 3D ReconstructionAccess Level: Readable by all users
Keywords: cleidocranial dysplasia, panoramic, coxa vara, wormian, suture, supernumeraryACR: 48.159

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