Over the years many amazing cases have been presented during the PEMPix presentation at the American Academy of Pediatrics’ annual national Conference and Exhibition during the Section on Emergency Medicine Sessions. PEMPix Classic is a featured series on PEMBlog that will highlight some of those classic cases.

 

The Case

 

A 9 month old previously healthy infant presents with three days of intermittent “noisy” breathing with crying or exertion. The infant has associated difficulty swallowing and choking with feeds unless they are upright. There is no fever, congestion or cyanosis. the infant’s vaccines are up to date and there are no sick contacts. An X-Ray of the airway was obtained.

 

AP Airway X-Ray

 

Lateral Airway X-Ray

 

What is the diagnosis?

 

A. Croup
B. Party balloon in the airway
C. Tracheolaryngomalacia
D. Cervical lung herniation
E. Retropharyngeal abscess

 

 

D. Cervical lung herniation

This a a rare condition in which there is protrusion of apical lung parenchyma through Sibson’s Fascia – an extension of the endothoracic fascia from the transverse process of the first thoracic vertebra to anterolateral margin of the first rib. Though less than 300 cases gave been reported it is thought to be associated with vitamin E deficiency, children with cleft lip and/or palate and Cri-du-chat. Overall less than 1/5 of these are seen in children (18%). Many resolve on their own, but they can lead to noisy breathing due to adjacent compression on the trachea, and feeding problems due to compression of the esophagus. There are at risk for spontaneous pneumothorax. Surgical repair is indicated for respiratory distress, dysphagia and cosmetic concerns.
 

References