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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Yeungnam Univ J Med. 2011;28(1):70-76. Published online June 30, 2011
Rhabdomyosarcomas are soft tissue sarcomas; while extremely rare in adults, they are one of the most common neoplasms in children and adolescents. Histologically, they can be classified into embryonal(ERMS), alveolar(ARMS), pleomorphic, and undifferentiated types. The ARMS type is very rare, and is associated with a poor prognosis. Common primary sites of ARMS are the trunk and extremities. We report on a case of paraaortic, supraclavicular, and axillary lymph node metastasis from paratesticular ARMS treated with VAC(vincristine, dactinomycin, cyclophosphamide)/ IE(ifosfamide, etoposide) chemotherapy in a young adult. Administration of six cycles of chemotherapy with VAC/ IE resulted in complete remission. The patient has maintained complete remission over the past 27 months.
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
Diffuse alveolar hemorrhage is a rare but serious and frequently life-threatening complication of a variety of conditions. The first goal in the management of patients with diffuse alveolar hemorrhage is to achieve or preserve stability of the respiratory status. Subsequently, the differential diagnosis is aimed at the identification of a remediable cause of the alveolar hemorrhage. The most common causes of diffuse alveolar hemorrhage with glomerulonephritis are microscopic polyangiitis and Wegener's granulomatosis, followed by Goodpasture syndrome and systemic lupus erythematosus. Microscopic polyangiitis (MPA) is a distinct systemic small vessle vasculitis affecting small sized vessels with few or no immune deposits and with no granulomatosus inflammation. The disease may involve multiple organs such as kidney, lung, skin, joint, muscle, gastrointestinal tract, eye, and nervous system. MPA is strongly associated with antineutrophil cytoplasmic autoantibody (ANCA) that is a useful serological diagnostic marker for the most common form of necrotizing vasculitis. Our report concerns a case of microscopic polyangiitis with diffuse alveolar hemorrhage in a 54-year-old man. He was admitted to our hospital due to dyspnea upon exertion and recurrent hemoptysis. Laboratory findings showed hematuria, proteinuria and deterioration of renal function. In the chest CT scan, diffuse ground glass appearance was seen in both lower lungs. A lung biopsy revealed small vessel vasculitis with intraalveolar hemorrhage and showed a positive reaction to against perinuclear ANCA. The patient was treated with prednisolone and cyclophosphamide. Chest infiltration decreased and hemoptysis and hypoxia improved. He is still being followed up in our hospital with a low dose of prednisolone.
Pre-surgical and post-surgical change in adult clef lip and palate patient following Le Fort I advancement osteotomy combined with bone graft was evaluated clinically and cephalometically. We obtained a successful function and esthetic improvement. The bone graft of alveolo-palatal clefts provides a stable bone support to the adjacent teeth of the cleft area, and well union of adjacent bone tissue, the closure of oronasal fistula and improvement of speech problem. Le Fort I osteotomy following the ostectomy of nasal septum for advancement of the maxilla was obtained relative improvement of esthetics and functional occlusion. 1. The orthodontic correction was required before and after surgery. 2. In this case, there was a limited range of anterior advancement of the Premaxillary-segment due to the scar tissue. 3. After 8 months of operation, we could show the new bone deposition on the cleft sites in dental radiograph and then the prosthetic treatment to the missing teeth was done.
We obtained successful functional and esthetic results by grafting of iliac marrow-cancellous bone in 2 cases of alveolar-palatal cleft patients. Bone graft of alveolar-palatal clefts provide bony support to adjacent teeth of cleft area, prevented from relapse of orthodontic arch expansion, closure of oroantral fistula and improvement of speech problem. 1. In one case, extraction of upper right central incisor that was little bone support, alignment of rotated teeth and expansion of collapsed arch segment were done with pre-orthodontic treatment. The other case. Bone grafting was done after removal of prosthesis with no pre-orthodontic treatment. 2. After mucoperiosteal incision in cleft area, the mucosal flap of labial area, palate and nose were separation and the raised nasal mucosa was sutured for closure of oroantral fistula. Then, the iliac marrow-cancellous bones were grafted to cleft site. 3. After 6 months of operation, we had seen the new bone deposition to cleft site in dental radiograph and prosthetic treatment of missing teeth were done.