Pulmonary alveolar proteinosis (PAP) is an uncommon disease characterized by progressive accumulation of lipoprotein material in the lungs due to impaired surfactant clearance. Whole-lung lavage (WLL) is the current standard treatment and consists of sequential lavage of each lung to mechanically remove the residual material from the alveoli. Although WLL is considered safe, unexpected complications can occur. Moreover, due to the rarity of the disease itself, this procedure is unknown to many physicians, and management of intraoperative complications can be challenging for anesthesiologists. Lung ultrasound (LUS) provides reliable and valuable information for detecting perioperative pulmonary complications and, in particular, quantitation of lung water content. There have been reports on monitoring the different stages of controlled deaeration of the non-ventilated lung during WLL using LUS. However, it has been limited to non-ventilated lungs. Therefore, we report the use of LUS in WLL to proactively detect pulmonary edema in the ventilated lung and implement a safe and effective anesthesia strategy. Given the limited diagnostic tools available to anesthesiologists in the operating room, LUS is a reliable, fast, and noninvasive method for identifying perioperative pulmonary complications in patients with PAP undergoing WLL.
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Anesthetic management of broncho- alveolar lavage in pulmonary alveolar proteinosis: A case report Abinav Sarvesh S P S, Sucheta Gaiwal, Nimitha Prasad Indian Journal of Clinical Anaesthesia.2024; 11(2): 251. CrossRef
Whole lung lavage (WLL) is a therapeutic procedure to remove accumulated material by infusing and draining the lungs with lavage fluid. This procedure has been regarded as the current standard of care to treat pulmonary alveolar proteinosis. However, the WLL protocol has not yet been standardized and the technique has been refined and modified a number of times. A rapid infusion system is a device used to infuse blood or other fluids at precise rates and normothermic conditions. This device is not typically used in WLL, which relies on the passive infusion of fluids using the gravitational force. However, in this study we performed WLL using a rapid infusion system, since we aimed to take advantage of its shorter operation time and greater degree of control over fluid volume and temperature. The patient’s symptoms improved without the occurrence of any complications.
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Autoimmune Pulmonary Alveolar Proteinosis: A Review of Pathogenesis and Emerging Therapies Akshay Mathavan, Akash Mathavan, Swaminathan Perinkulam Sathyanarayanan, Cormac McCarthy, Ali Ataya Current Pulmonology Reports.2024; 13(3): 233. CrossRef
Whole-Lung Lavage—a Narrative Review of Anesthetic Management Santiago M. Mata-Suarez, Agustina Castro-Lalín, Santiago Mc Loughlin, Juan De Domini, Juan C. Bianco Journal of Cardiothoracic and Vascular Anesthesia.2022; 36(2): 587. CrossRef
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Anesthetic management during whole-lung lavage using lung ultrasound in a patient with pulmonary alveolar proteinosis: a case report Jae Wan Jung, Hyunho Lee, Jimi Oh Yeungnam University Journal of Medicine.2021; 38(4): 374. CrossRef
Pulmonary alveolar proteinosis (PAP) is a rare disorder that's characterized by accumulation of surfactant components in the alveolar space. Idiopathic PAP is recognized as an autoimmune disease that's due to impaired alveolar macrophage function and this caused by autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). We report here a case of pulmonary alveolar proteinosis that was deemed interstitial lung disease at the initial diagnosis. A 61-year-old man presented with intermittent blood tinged sputum and dyspnea on exertion. The man was a painter for 30 years and he had a 10 pack-years smoking history. Chest computerized tomography (CT) revealed multifocal ground-glass opacity with interstitial thickening at both lungs. His pulmonary function tests and methacholine test revealed non specific results. He was diagnosed with interstitial lung disease on the basis of the chest CT finding and occupational history. However, seven months later, his symptoms progressed. Follow-up chest CT was performed. Wedge resection via video-assisted thoracoscopic surgery (the anterior basal segment of the left lower lobe) was done. Microscopic examination showed large groups of alveoli with excessive amounts of surfactant and a complex mixture of protein and lipid (fat) molecules. Finally, he was diagnosed as having pulmonary alveolar proteinosis.
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Pulmonary alveolar proteinosis in a 15-year-old girl Yechan Kyung, Jihyun Kim, Hong Kwan Kim, Joungho Han, Kangmo Ahn Allergy, Asthma & Respiratory Disease.2015; 3(1): 86. CrossRef