Ina Sorge (Leipzig)  

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10 Years  

Male  

Syndromes  

congenital  

10 year old hungarian boy, presentation because of growth hormone deficiency. The images (outside institution) should be also evaluated.

There was obvious waddle gait, increased mobility in the shoulder girdle and dental dysplasia.  

N/A  

<- view X-Ray 1

X-Ray 1: (foreign institution): Widend skull. Noticeable are many wormian bones between the skull sutures of the sagittal suture. Hypopneumatisation of the maxillary sinuses.






<- view X-Ray 2

X-Ray 2: (foreign institution): Many wormian bones between the skull sutures. There is still a complete calcification of the fontanelles seen.






<- view X-Ray 3

X-Ray 3: (foreign institution): Hypoplastic claviculae, where both lateral portions are not developed, or rather at least not calcified.






<- view X-Ray 4

X-Ray 4: (foreign institution): Pelvis: Wings of ilium seem steep, no mineral salt deposition in the ischial bones, wide pubic bone joints.

Of note is the femoral head. Here one finds the physial disk not directly under the epiphysis, but rather in the area of the femoral neck. The femoral neck-diaphysal shaft angle is therefore not obtuse, but rather is pathologically a right angle (ca. 90°).

 

Expert's opinion  

None  

In late adulthood, the calcification anomolies partially regress. The changes in the femoral neck are not reversible, and should be seen as a pre-arthrosis due to the pathological stress of the femoral head.  

N/A  

N/A  

1. Medline:
R.Schumacher, I.Greinacher:
Segmental betonte Osteochondrodysplasien, Seite 356-358; in W.Schuster, D.Färber: Kinderradiologie, 2. Auflage, Springer Verlag (1996)  

Dysostosis, cleidocranialis, cleidocranial, Dysplasia, Clavicula, Wormian Bone, child, childhood, pediatric radiology  

Ina Sorge. Cleidocranial dysostosis. PedRad [serial online] vol 3, no. 12.
URL: www.PedRad.info/?search=20031208164107