Thoracic Surgery in Children
Congenital lung lesions
Know the family: congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration (systemic arterial supply), congenital lobar emphysema (hyperlucent lobe with mediastinal shift needing urgent lobectomy), bronchogenic cyst and pleuropulmonary blastoma, a rare malignancy whose type I lesion looks cystic and benign. Many CPAMs are asymptomatic and the timing of resection is debated; large lesions causing respiratory distress or hydrops need intervention.
Congenital diaphragmatic hernia is a physiological emergency: gentle ventilation to avoid barotrauma, Replogle nasogastric decompression, avoidance of chest drains placed blindly, delayed repair after stabilisation and ECMO for severe cases.
Mediastinal compartments
Anterior masses include thymoma, teratoma, lymphoma and thyroid; middle compartment masses include bronchogenic cyst and lymphadenopathy; posterior masses are neurogenic - neuroblastoma, ganglioneuroma, schwannoma. The key trap is the child with an anterior mediastinal mass and airway compression who arrests on induction of anaesthesia: obtain tissue under local anaesthesia, or treat presumed lymphoma with steroids or radiotherapy first.
Chest wall deformity is assessed by the Haller index on CT; Poland syndrome combines absence of the sternal head of pectoralis major with symbrachydactyly.
Traps
Never place a chest drain blindly for suspected diaphragmatic hernia, and never assume a cystic lung lesion is benign - type I pleuropulmonary blastoma is cystic.
Worked example
A newborn has respiratory distress, a scaphoid abdomen and bowel gas in the left chest. Rule of thumb: CDH means Replogle tube, gentle ventilation, no chest drain and delayed repair after stabilisation.