Alveolar Proteinosis: Lobar Lavage by Fiberoptic Bronchoscopic Technique

doi:10.1378/chest.69.2.224

Chest

Volume 69, Issue 2, February 1976, Pages 224-227

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Lavage limited to an isolated lobe was performed on multiple occasions using a cuffed fiberoptic bronchoscope in a patient with alveolar proteinosis. Sequential ventilation-perfusion scintiphotoscans were used to preselect and follow the functional behavior of the lavaged lobe. Lavage led to functional improvement. The technique of fiberoptic bronchoscopic lobar lavage is simple and may find application in patients in whom lavage of an entire lung may be hazardous.

Section snippets

CASE REPORT

This 30-year-old white man was first admitted to the University Hospital, San Diego, Calif, on Sept 4, 1973 for chronic alcoholism and alcohol withdrawal. During that admission, a chest x-ray film showed patchy upper lobe infiltrates (Fig 1). With the exception of a dull discomfort in the anterior portion of the chest, the patient denied any respiratory symptoms. The patient had smoked two packs of cigarettes per day for ten years and had worked, on occasion, as a sandblaster. The findings from

RESULTS

Each procedure was well tolerated, and no complication occurred. Considerable proteinaceous material was removed during each lavage. Unrecoverable lavage fluid varied from 200 to 400 ml.

The sequence after each lavage was similar. A widening of the alveolar-arterial oxygen pressure difference [P(A-a)O₂] occurred during the procedure and persisted for approximately five hours after lavage (Table 2). This was associated with immediate postlavage scintiphotographs which demonstrated a marked

DISCUSSION

Bronchopulmonary lavage has been used in therapy for alveolar proteinosis,⁶ asthma,⁷ and cystic fibrosis⁸ since Ramirez-R² offered it as an alternative to segmental irrigation in 1966. The potential hazard of severe hypoxemia⁹ and the difficulty of the technique (which involves tracheal division via a Carlens bronchospirometric tube, degassing the lung, 100-percent oxygen breathing, and general anesthesia) has limited its useful application to a few medical centers and the more advanced cases.

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Cited by (47)

Whole-Lung Lavage—a Narrative Review of Anesthetic Management
2022, Journal of Cardiothoracic and Vascular Anesthesia
Pulmonary alveolar proteinosis is a rare disease characterized by progressive accumulation of lipoprotein material in the alveoli as a result of a dysfunction in surfactant clearance. The whole-lung lavage procedure is considered the current standard of care and consists of the sequential lavage of both lungs for mechanical removal of residual material in the alveoli. However, a lack of standardization has resulted in different procedural techniques among institutions. Even though whole-lung lavage is considered to be a safe procedure, unforeseen complications might occur, and proper knowledge of physiologic implications may allow clinicians to establish the appropriate therapy. This review provides an insight into the underlying physiology of the disease, the technical details of the procedure from an anesthesiologist's perspective, and discussion of potential intraoperative complications.
Pulmonary alveolar proteinosis
2014, Revue des Maladies Respiratoires
La protéinose alvéolaire pulmonaire (PAP) est une maladie rare caractérisée par l’accumulation alvéolaire de composants du surfactant qui gêne les échanges gazeux. Les PAP sont séparées en trois groupes : les PAP auto-immunes caractérisées par la présence d’anticorps sériques anti-GM-CSF qui sont les plus fréquentes ; les PAP secondaires à une maladie sous-jacente, le plus souvent hématologique, mais aussi à l’inhalation de toxiques et à des infections ; enfin, les PAP d’origine génétique. Le diagnostic de PAP est évoqué sur le scanner thoracique avec une pneumopathie infiltrante diffuse ayant un aspect classique de crazy paving, puis confirmé par l’aspect laiteux du lavage bronchoalvéolaire, et la présence de matériel lipoprotéinacé PAS positif. La confirmation du diagnostic de PAP par biopsie pulmonaire chirurgicale est désormais rarement nécessaire. La positivité des anticorps sériques anti-GM-CSF signe le caractère auto-immun de la maladie. Le traitement de référence des PAP auto-immunes est le « grand lavage pulmonaire thérapeutique ». En cas de maladie réfractaire, d’autres traitements sont disponibles, tels que le GM-CSF en injection sous-cutanée ou en aérosol et le rituximab. L’évolution de la maladie est imprévisible. Une évolution spontanément favorable ou même une guérison spontanée peuvent survenir et la survie actuarielle à 5 ans est de 95 %. Les principales complications sont infectieuses.
Alveolar proteinosis (AP) is a rare disease characterized by alveolar accumulation of surfactant components, which impairs gas exchange. AP is classified into three groups: auto-immune AP defined by the presence of plasma autoantibodies anti-GM-CSF, the most frequent form (90 % of all AP); secondary AP, mainly occurring as a consequence of haematological diseases, or following on from toxic inhalation or infections, and genetic AP, which affects almost exclusively children. AP diagnosis is suspected where chest CT-scan demonstrates interstitial lung disease with a crazy paving aspect; and confirmed by bronchoalveolar lavage, which has a milky appearance and contains periodic acid Schiff positive proteinaceous alveolar deposits. The use of surgical lung biopsy to confirm AP is less frequent nowadays. In this context, positive antibodies against GM-CSF indicates an auto-immune etiology of the AP. Concerning management, whole lung lavage is the gold standard therapy. In refractory AP, new treatments are available such as subcutaneous or inhaled GM-CSF supplementation, or rituximab infusions. The clinical course is unpredictable. Spontaneous improvement or even cure can occur, and the 5-year actuarial survival is 95 %. The most frequent complications are infectious etiology.
Total lung lavage by awake flexible fiberoptic bronchoscope in a 13-year-old girl with pulmonary alveolar proteinosis
2007, Respiratory Medicine
Citation Excerpt :
In children, persistent hypoxemia after therapeutic bronchoscopy under a general anesthesia, has also been described. Successful treatment of PAP with multiple segmental or lobar lavage by fibreoptic bronchoscopy without general anesthesia has also been reported in adults.11 However, the yield by this method is small and the volumes of lavage fluid limited.11
Pulmonary alveolar proteinosis (PAP) is a rare, heterogeneous diffuse lung disease in childhood. We report a case of an asymptomatic 13-year old girl with PAP. She had radiolographic findings suggesting the diagnosis, which was confirmed by the “milky” bronchoalveolar lavage fluid and the histology of transbronchial biopsy. Total lung lavage was performed by flexible fiberoptic bronchoscope under local anesthesia with success. This is the first reported case of a PAP in a child that was treated by being awake during bronchoscopy. After one-year of follow-up, the patient remains free of symptoms.
Pulmonary alveolar phospholipoproteinosis
2005, Revue de Pneumologie Clinique
La lipoprotéinose alvéolaire pulmonaire (LPA) est une maladie pulmonaire rare, d’étiologie inconnue, caractérisée par le comblement alvéolaire par du surfactant anormal. Il est maintenant admis que la biopsie pulmonaire chirurgicale, longtemps considérée comme la référence, n’est pas indispensable au diagnostic de LPA lorsque les anomalies tomodensitométriques et l’analyse du liquide de lavage broncho-alvéolaire sont typiques. Son traitement repose sur la réalisation de grands lavages pulmonaires, mais la mise en évidence d’anticorps anti-GM-CSF, dans les formes primitives, ouvre des perspectives thérapeutiques nouvelles.
Pulmonary alveolar phospholipoproteinosis is a rare lung disease of unknown cause characterized by surfactant plugging of the alveoli. At the present time, surgical lung biopsy, long considered as the gold standard, is not necessary for positive diagnosis of pulmonary alveolar phospholipoproteinosis when computed tomography anomalies and analysis of bronchial lavage fluids present a typical pattern. Treatment requires abundant lavage, but the demonstration of anti-GM-CSF antibodies in primary forms opens new therapeutic perspectives.
Pulmonary alveolar proteinosis: Treatment by bronchofiberscopic lobar lavage
2002, Chest
Citation Excerpt :
Harris and colleagues16 used a cuffed bronchoscopic catheter to perform lobar lavage under fluoroscopic guidance. Brach et al17 used a modified bronchoscope with an inflated tracheostomy cuff and a Venturi mask to perform the lavage procedure. Heymach et al18 used general anesthesia to perform a bronchoscopic lavage.
The current mainstay of treatment for pulmonary alveolar proteinosis (PAP) is whole-lung lavage. Therapy with granulocyte-macrophage colony- stimulating factor is a possibility, although its long-term safety has not been determined. An alternative procedure is selected lobar lavage by fiberoptic bronchoscopy (FOB). We report here our experiences with lobar lavage by FOB in treating three patients with PAP. PAP was diagnosed in three patients (two men, one woman) who had dyspnea and hypoxemia after undergoing open-lung biopsy. The patients underwent lobar lavage by FOB under local anesthesia. The bronchoscope was wedged into a lobar bronchus. Approximately 2,000 mL warm normal saline solution was instilled via syringe in 50-mL aliquots through a fiberoptic bronchoscope. After undergoing multiple lobar lavages, two patients showed clinical, physiologic, and radiologic improvement. The third patient, who had more advanced disease, showed improvement only in oxygenation. The major complications were severe cough and hypoxemia during lavage. Our experience suggests that bronchoscopic lobar lavage is simple and safe, and may find application in patients in whom a whole-lung lavage with generalized anesthesia may be hazardous, and in patients with less advanced disease whose proteinaceous substances can be removed with a small volume of lavage fluid.
Role of bronchoscopy in modern medical intensive care unit
2001, Clinics in Chest Medicine
Citation Excerpt :
Other uses of FOB in the ICU include therapeutic administration of solutions to selected regions of the tracheobronchial tree—for example, surfactant in ARDS and N-acetyl cysteine in mobilization of mucous plugs. Whole lung lavage for pulmonary alveolar proteinosis59 and drainage of lung abscess and bronchogenic cysts also can be attempted using FOB. Flexible bronchoscopy also could be employed to identify the bronchopulmonary segment that is the source of air leakage.
Gustav Killian,⁶⁰ known as the “father of bronchoscopy,” was the first to perform bronchoscopy in 1897 to examine the airways and remove a foreign body. He used a rigid endoscope to remove an aspirated pork bone from the right main bronchus. Chevalier Jackson of Philadelphia later modified the rigid bronchoscope (RB) in 1904 by adding a direct ocular mechanism, suction tube, and tip illumination.¹⁰⁰ The RB was the only available instrument to examine the airways until the late 1960s, when Shigeto Ikeda⁵⁴ introduced the flexible fiberoptic bronchoscope for clinical use. Since its introduction in 1967, the use of the flexible bronchoscope (FB) rapidly has become widespread for various pulmonary disorders, and presently it is considered one of the most useful modalities, primarily for diagnostic and, to a lesser extent, therapeutic purposes.
A critically ill patient can be defined as one whose vital bodily functions are compromised seriously from pathologic injury to one or more organ systems. Critically ill patients therefore require close monitoring by specially trained medical and paramedical personnel. Involvement of the respiratory system is common, whatever the underlying cause of disease that precipitates the critical illness. The common causes of pulmonary disease in the intensive care unit (ICU) include pneumonia (community-acquired and nosocomial), pulmonary edema (cardiogenic and noncardiogenic), pulmonary hemorrhage, thromboembolic disease, primary and metastatic lung cancer, drug- and radiation-induced lung injury, and others, including connective tissue disorders.
Because critically ill patients may be too unstable to move safely, diagnostic procedures that can be performed in the ICU at beside are preferable to those that involve transport to remote sites. The versatility and the easy portability of the FB enable the performance of FOB easily and safely at the bedside. In addition, in contrast to RB, FOB offers enhanced visualization of the distal bronchi and averts the need for general anesthesia and operating room resources. The FB therefore is considered an essential diagnostic and therapeutic instrument in the care of patients admitted to the ICU.28, 71 According to the estimation of Tobin et al,¹²¹ as many as 98% of all bronchoscopic procedures are performed using the FB, and most bronchoscopists have never been trained in the technique of RB.
Despite the many advantages of FBs, the RB sometimes is preferred especially as the primary therapeutic and diagnostic tool in massive hemoptysis and large foreign body aspiration. With the advent of new therapeutic modalities such as the neodymium yttrium-aluminium-garnet (Nd–YAG) laser, endobronchial stenting, cryotherapy, and so forth, the RB increasingly is being used in patients with respiratory failure secondary to endobronchial obstruction or extrinsic compression of the bronchus. Rigid bronchoscopy, when used in the ICU, is limited to situations in which an FB is unavailable or when a mature tracheal stoma is present in a spontaneously breathing patient. High-risk patients and those who require bronchoscopic lung biopsies (BLB) probably still should undergo the procedure in the operating room because of the risk for bleeding and tension pneumothorax (especially with positive-pressure ventilation.)

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: This project was supported by National Heart and Lung Institute grants HL 14169 and HL 00134.

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Chest

Selected RportsAlveolar Proteinosis: Lobar Lavage by Fiberoptic Bronchoscopic Technique

Section snippets

CASE REPORT

RESULTS

DISCUSSION

Chest

J Thorac Surg

Chest

Pulmonary alveolar proteinosis

Am Rev Respir Dis

Pulmonary alveolar proteinosis

Arch Intern Med

Selected Rports
Alveolar Proteinosis: Lobar Lavage by Fiberoptic Bronchoscopic Technique