Specific pathology of the infant that corresponds to 2 histological pictures (see these terms):

• neuroendocrine cell hyperplasia or NEHI: 70 % of bronchioles with NE cells and at least one bronchiole with ≥ 10 % of NE cells
• Interstitial pulmonary glycogenosis or PIG: interstitial infiltrate of glycogen-rich mesenchymal cells

The clinical presentation is non-specific: tachypnea, poor weight gain, hypoxia, crackling appearing in the 1st months of life (median: 4 months).

Imaging: on CT, the suggestive but non-pathognomonic lesions are:

-        hourglass glass opacities, mainly located in the middle lobe, lingula and peri-hilar and para-mediastinal regions

Diagnosis of certainty: lung biopsy and bombesin, chromogranin or synaptophysin markings, PAS  and Sudan black staining.

Most often spontaneously favorable evolution in the first years of life with symptomatic management (O2,nutritional support), without specific treatment.

Anesthetic implications:

SpO2 at room air; lung biopsy; gentle ventilation

References :

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Updated: October 2021