Case presentation
A 34-year-old Caucasian man presented to a hospital in the UK with a 1-month history of progressive exertional dyspnoea and non-productive cough. He was a current cigarette smoker but had no other medical problems. He was found to be severely hypoxaemic while breathing room air at rest (arterial haemoglobin oxygen saturation, SaO2 87%; arterial partial pressure of oxygen, PaO2 5.4 kPa) and chest X-ray showed bilateral patchy air-space infiltration. Pulmonary function testing demonstrated a restrictive ventilatory defect (forced expiratory volume in 1 s, FEV1 2.4 L; forced vital capacity, FVC 2.5 L; FEV1/FVC 43%) and impaired diffusion capacity (carbon monoxide diffusion capacity 45% of predicted value). Thoracic computed tomography indicated that the right lung was more severely diseased than the left. Broncho-alveolar lavage (BAL) fluid cytological examination was suggestive of PAP. He was admitted to a specialist cardiothoracic unit for urgent lung lavage.

On arrival in the operating room, the patient was dyspnoeic, cyanosed and severely hypoxaemic despite breathing high-flow oxygen via a facemask (SaO2 < 85%). Electrocardiographic and invasive arterial pressure monitoring were established. During pre-oxygenation, SaO2 improved to >90%. Anaesthesia was induced with propofol and fentanyl and subsequently maintained with propofol and remifentanil infusions. A non-depolarising neuromuscular blocking agent was administered to facilitate tracheal intubation. Oxygenation saturation remained stable following induction of anaesthesia. A 39 mm left-sided double-lumen endotracheal tube was inserted and its correct position confirmed by fibreoptic bronchoscopy. Airway pressure, tidal volume and end-tidal carbon dioxide concentration were continuously monitored as well as regular arterial blood gas analysis. WLL was performed with the patient in the supine position on the operating table. One-lung ventilation of the left lung was commenced just before initiation of lavage of the right lung. Under fibreoptic bronchoscopic control, a respiratory physician carried out repeated cycles of instillation of 1 L of 0.9% saline solution at body temperature followed by passive drainage under gravity. In order to achieve maximal filling and drainage of all lung segments, an experienced physiotherapist performed manual chest vibration and percussion. Various positional manoeuvres were also used to facilitate run-in and run-out of fluid. During fluid inflow and outflow, airway pressure and tidal volume were closely monitored to assess for leakage of fluid from the non-ventilated lung into the ventilated lung. Initially, milky fluid effluent was obtained and a total lavage volume of 10–15 L was necessary to obtain clear fluid effluent. The procedure lasted approximately 2 hours. At the end of the procedure, two-lung ventilation was commenced and recruitment manoeuvres were applied to restore expansion of both lungs. Satisfactory oxygenation was maintained throughout the procedure during both two-lung and one lung ventilation (SaO2 > 90% and PaO2 > 8 kPa). Left-sided WLL was planned within the next 24 hours. Hence, the double-lumen endotracheal tube was exchanged for a 9.0 mm single-lumen tube and the patient was transferred to the intensive care unit (ICU) for ventilatory support. For left-sided WLL, the single-lumen endotracheal tube was exchanged for a 39 mm left-sided double-lumen tube and an identical technique for WLL was employed. The procedure was better tolerated with improved oxygenation compared to the previous WLL. At the end of the procedure, following an endotracheal tube exchange, the patient was transferred to ICU where he was extubated within 8 hours. Manual chest physiotherapy techniques and positioning manoeuvres were continued postoperatively.

Bilateral sequential WLL in the same session was performed on two occasions in the subsequent few weeks. Three further unilateral WLL procedures (one right sided and two left-sided) were carried out in the following 6 months. A similar anaesthetic technique was used for each WLL procedure. Serial WLL resulted in clinical, physiological and radiological improvement for the patient and eventual remission of the disease.