Citation, DOI and case data
This is a 23 month old girl with a history of recurrent lower repsiratory tract infections.
Loading Stack -
0 images remaining
Frontal and lateral radiographs reveal a bell-shaped narrow thorax with short, horizontally oriented ribs. The clavicles are abnormally elevated. The frontal view shows both acetabula to be dysplastic. A gastrostomy catheter and right femoral catheter are noted.
This is an example of Jeune syndrome (asphyxiating thoracic dystrophy). This is a rare type of short limb, skeletal dysplasia. It is characterized by a long, narrow bell-shaped thorax with short, horizontally oriented ribs.
Other radiographic findings can include polydactyly, short distal limbs and dysplastic acetabuli. The condition is also associated with cystic renal disease and hepatic fibrosis. Most morbidity and mortality is related to respiratory compromise 1.
1 article features images from this case
14 public playlist include this case
- Resident Session 11/27/13 by Dr Hector Rivera-Melo
- peds by Dr Sharbell Hashoul
- Viva cases by SG
- AEmira MS/P by Ahmed Emira
- core ped by ahmed alaa al-agamy
- Rad revision by Dr Menyatsoe
- Skeletal Dysplasia by JEANNIE
- Peads by Quratulainali
- FRCR 2B Listing by Dr Sherif Mohsen
- 22/9/17 by Morgan
- sp3 by dr [email protected]
- Izak paediatric cases by Izak Van der Walt
- 2b paediatrics by Prakruthi Venkatappa
- Syndrome by Dr. Kenneth Kwok