Congenital cystic adenomatoid malformation of the lung: about a fatal neonatal presentation

¹Pediatric Intensive Care Unit and Anesthesiology Department, Children´s Hospital, Ibn Rochd University Hospital, Hassan II University of Casablanca, Casablanca, Morocco

^&Corresponding author
Ouissal Aissaoui, Pediatric Intensive Care Unit and Anesthesiology Department, Children´s Hospital, Ibn Rochd University Hospital, Hassan II University of Casablanca, Casablanca, Morocco

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Congenital cystic adenomatoid malformation (CCAM) of the lung, also known as congenital pulmonary airway malformation (CPAM), is a rare congenital developmental abnormality, representing about 25% of all congenital lung lesions. It is often revealed by respiratory distress occurring during the neonatal period which can be severe, even fatal. Antenatal screening allows early recognition and therefore improves prognosis. We report a case of a newborn (21 days of life) admitted to the pediatric ICU for respiratory distress. The chest X-Ray showed a voluminous cystic formation of the right lung. The Chest CT identified an aspect in favor of a cystic adenoid malformation of the right lung classified type 1 according Stocker´s classification (figure 1). As initial management, the patient was intubated ventilated, sedated and antibiotherapy associating Ceftriaxone and Gentamycine was initiated. Refractory hypoxemia persisted making the surgical intervention impossible to perform. He presented later on a hypoxic refractory cardiorespiratory arrest.

Figure 1: chest CT scan showing typical appearance of a congenital cystic adenomatoid malformation of the lung