Thoracoscopic lung biopsy in a patient with pulmonary lymphangiomyomatosis

We describe the anesthetic management of a patient with pulmonary lymphangiomyomatosis (LAM) during thoracoscopic lung biopsy (TSLB).

LAM is a rare idiopathic disease characterised by progressive deterioration in respiratory function, occurring almost exclusively in women. In establishing the diagnosis, an open lung biopsy (OLB) has been employed in patients with relatively normal lung function. However, TSLB rather than OLB is less invasive. A 38 yr old woman developed a clinical course of cough, shortness of breath and sputum production, Chest X-ray findings, 99mTc-MAA scintigraphy and thin-sliced high resolution CT, typical of LAM, TSLB was scheduled to establish the diagnosis. General anesthesia, employing differential lung ventilation and high frequency jet ventilation combined with epidural anesthesia and continuous intravenous propofol was performed successfully. High frequency ventilation was applied to the non-dependent lung and intermittent positive pressure ventilation (IPPV) to the dependent lung with lower tidal volume and respiratory rate, allowing permissive hypercapnia. In the postoperative period, although synchronized intermittent mandatory ventilation was applied, pressure support ventilation or continuous positive airway pressure (CPAP) would have been a better selection. Postoperative sedation was performed satisfactorily using propofol.

We recommend general anesthesia using differential lung ventilation combined with epidural anesthesia and intravenous propofol during TSLB for LAM. Postoperative ventilation should be pressure support ventilation or CPAP to keep peak inspiratory pressure low and allow permissive hypercapnia.