(PIG)

Rare pulmonary interstitial disease, of unknown cause, generally associated with a disorder of pulmonary growth and development. Sometimes associated with a congenital heart disease or a chromosomal abnormalities (trisomy 21). Signs appear early in the neonatal period or in the first months of life: tachypnea, respiratory distress and hypoxemia during effort, sometimes pulmonary arterial hypertension. Cases of spontaneous resolution have been reported. The prognosis is more related  to the cause of the pulmonary disorder or the cardiac malformation than to the interstitial pathology.

RX thorax, or CTScan: image of interstitial pneumonia with zones of atelectasis and hyperinflation, as well as 'watch glass' opacities.

Histology: thickening of cellular origin of the interstitial tissue; glycogen abundance in these pulmonary interstitial cells of mesenchymal origin (which explains its name but there is no connection with the abnormalities in the metabolism of glycogen). The pathology can be diffuse or  unequally distributed in the lung parenchyma.

Treatment: symptomatic: oxygen therapy; the response to corticosteroid therapy is variable.

Anesthetic implications: 

check saturation on room air, preoperative Xray; hemodynamic management adapted to the possibly associated heart disease or pulmonary hypertension.

References : 

-         Deutsch GH, Young LR. 
Pulmonary interstitial glycogenosis: words of caution. 
Pediatr Radiol 2010; 40: 1471-5.

Updated: Octobre 2018