Anaesthetic management of a neonate with congenital pulmonary airway malformation for pneumonectomy – A case report

Congenital pulmonary airway malformation (CPAM) is a rare entity.[1],[2] It results from embryologic insult in early gestation usually before 50th day, due to failure of bronchiolar structure during pseudoglandular stage of lung development. They are usually detected prenatally during ultrasound scan in pregnancy [3]. The incidence of these cases is around 1: 2500 to 8000 live births and is more common in males [3]. Bronchiolar structures fail to mature causing excessive growth of terminal bronchioles without any alveoli [3]. This hampers alveolar growth, the arterial supply to the lung, and the lung's venous drainage [4]. They can have varied presentation at birth varying from complete resolution to mediastinal shift to fetal hydrops. They present as cystic and adenomatous elements which arise from tracheal, bronchial, bronchiolar, and alveolar tissues. Usually, one entire lobe is replaced by cysts which are non-functioning (see Fig. 1, Fig. 2).

In this case, induction was managed by an experienced anaesthesiologist. The paediatric surgeon was standby during anaesthetic induction anticipating risk of cyst rupture causing complications like pneumothorax, hemodynamic instability. The key for successful management of our case was hand ventilating the neonate using Jackson Rees (JR) circuit by gentle intermittent positive pressure (IPPV) avoiding increase in peak pressure which also helped us judge the lung compliance.

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