Explanation:
The most important x-ray finding is the presence of free subdiaphragmatic air and air in the portal system indicating perforated bowel. Portal venous gas is an ominous finding and often occurs with ischemic bowel.

Schwachman Diamond Syndrome (SDS) is a rare congenital disorder characterized by exocrine pancreatic insufficiency, bone marrow dysfunction, and skeletal abnormalities. It is the third most common cause of inherited bone marrow failure.

For more information on SDS, please go to: http://emedicine.medscape.com/article/958476-overview

** Thank you Dr. Jacqueline Lee for the material for this case.**