Thoracoscopic surgery for congenital pulmonary airway malformation

Abstract

In just 30 years, operation in the thorax of children has changed drastically from an open to purely thoracoscopic approach. As a budding sub-specialty, a lot of questions remained to be answered, in particular those regarding its safety and efficacy. Congenital pulmonary airway malformation (CPAM) is one of the most common thoracic conditions that require surgical intervention. However, there were only a handful of studies about thoracoscopic surgery of CPAM in the literature. The objective of this thesis is therefore to shed light on some of these puzzles. While in the past CPAM patients presented when they were symptomatic, most patients can be detected antenatally nowadays. In contrast to the traditional belief of it being an extremely rare disease with incidence at 1 in 35000 births, the finding in this thesis suggested that it is at least 1 in 7200 births in our locality. The incidence in the past 2 decades also increased significantly from 1 in 27400 births (p = 0.024). Conventionally CPAM was resected via a thoracotomy. Arrival of thoracoscopic techniques has challenged this concept. Patients with thoracoscopic lobectomy had significantly shorter duration of chest tube drainage (4.3 days vs 6.9 days, p = 0.004), shorter intensive care unit stay (2.5 days vs 5.9 days, p = 0.003) and shorter hospital stay (6.9 days vs 12.0 days, p < 0.001) when compared to the thoracotomy group. Post-operative complication rate was similar between the two groups. Thoracoscopic lobectomy of CPAM has been proven to be safe in terms of physiological parameters. Long term follow-up on pulmonary function has showed no difference between thoracoscopic group and healthy individuals, while the result after open lobectomy was demonstrated to be inferior (forced vital capacity, 98.9% vs 84.3% predicted, p = 0.03). In addition, musculoskeletal studies have found a significantly less occurrence of scapular winging in thoracoscopic group patients (13% vs 58%, p = 0.01) In the uncommon event of CPAM with absence of lung fissure, thoracoscopic lobectomy can be technically demanding if not dangerous. Wedge resection may result in residual disease if the lesion is deep-seated and cannot be visualized on the pleural surface. In this thesis we described our innovative method to solve the problem by adopting hookwire guidance during thoracoscopic resection. In conclusion, this thesis has highlighted some essential concepts in thoracoscopic surgery for CPAM in children. This minimally invasive approach can be performed safely for CPAM patients with improved outcomes.