Asphyxiating Thoracic Dystrophy

Etiology: Congenital
Imaging Radiograph: Short ribs, bowed extremities, trident pelvis, normal spine
DDX:
Complications: Death
Treatment: None
Clinical: Lethal skeletal dysplasia

Radiology Cases of Asphyxiating Thoracic Dystrophy

CXR of asphyxiating thoracic dystrophy — CXR AP shows short ribs resulting in a small thoracic cavity. The spine was normal in appearance.

CXR of asphyxiating thoracic dystrophy (Jeune syndrome) — CXR AP shows shortened ribs resulting in a small thorax and a normal spine.

CXR of asphyxiating thoracic dystrophy — CXR AP and lateral shows a small thorax due to severely shortened ribs. The spine is normal.

Radiology Cases of Asphyxiating Thoracic Dystrophy Causing Trident Pelvis

Radiograph of trident pelvis of asphyxiating thoracic dystrophy — AP radiograph of the lower extremities shows a trident pelvis and bowing of the lower extremities.

Gross Pathology Cases of Asphyxiating Thoracic Dystrophy

Gross pathological image of asphyxiating thoracic dystrophy — Post-mortem gross pathological image shows an extremely small thorax due to shortened ribs, particularly when compared to the size of the abdomen.

Pathology image of asphyxiating thoracic dystropy — Post-mortem gross pathological image (above) shows a small volume thorax and hypoplastic lungs caused by ribs that are very short in length (below).