ABSTRACT
Pulmonary Alveolar Proteinosis [PAP] is a rare disease of uncertain etiology and variable natural course. This study aimed to evaluate the clinical features and natural history following our management protocol for PAP which consisted of multiple sessions of modified bronchoalveolar lavage under general anaesthesia. This prospective study was carried in Kasr El Aini's University Hospital and new Kasr El Aini hospital From October 2003 till May 2006. Nine patients received 24 sessions of whole bronchoalveolar lavage for management of pulmonary alveolar proteinosis [PAP]. Patients age ranged from 7 and 42 years [mean of 23 years] and were categorized into two groups: [A] the pediatric group [included 3 patients aged 7,8 and 11 years] and [B] the adult group which included 6 patients. Whole lung lavage [one lung at a time] was carried out under general anesthesia with selective one lung ventilation using a double-lumen endotracheal tube in adult patients. In the pediatric group as a double-lumen endotracheal tubes suitable for their size were not available, a small-for-weight endotracheal tube was introduced through the vocal cords to ventilate one lung while a rigid bronchoscope was passed beside it to lavage the other lung. In each adult patient around 20 liters of warm normal saline [to which 1 ampoule of heparin was added to each 500 mls] were instilled in increments of 5 ml/kg inside the main bronchus of a single lung followed by a few minutes of postural changes while multiple gentle chest percussions were applied. The process was stopped when the lavage fluid suctioned from the endo-bronchial tree became clear. None of our patients died in the follow up period. No morbidity occurred in our patients apart from an episode of mild retention of saline inside the alveolar tree which was manifested by marked increase in the CVP, low arterial oxygen saturation and low [PaO[2]] in the ABG which was managed promptly using combination of Oxygen inhalation by mask, bronchodilators and intravenous diuretics. All patients stated a remarkable improvement in their tolerance to physical exertion. All 9 patients [100%] expressed total disappearance of SOB during the follow-up period. One patient [11.1%] complained of intermittent cough for 4 days before being ameliorated. A single patient [11.1%] complained of transient low-grade fever. Few scattered rales were present in 6 patients [66.6%] due to residual retention of the lavage fluid inside the alveoli from which they were treated by diuretics until it totally disappeared within the first 24 hours. Plain chest radiographs and CT chest showed fainting and near-total disappearance of the ground glass and the reticulation patterns. Spirometry and lung volumes [percentage predicted] revealed a marked improvement with FVC and mean total lung capacity values. The mean value of Forced Expiratory Volume [FEV1] during the first second became 94.9 +/- 13.6, the total lung capacity [TLC] became 83.1 +/- 15.7 and the ratio of FEV1 to the Forced vital capacity became 101 +/- 8.4. Arterial blood gases measurements, showed that the mean arterial oxygen tension [PaO[2]] was 79.5 +/- 6.2 mmHg, while the mean PCO[2] tension was 36.3 +/- 3.5 mmHg. The mean pH value of the blood was 7.43 +/- 0.02. The mean arterial oxygen saturation became 84 +/- 8.8%, [mean of 79-96%]. In our experience whole lung PAL was simple, safe and could be applied in patients with pulmonary alveolar proteinosis with a sound degree of safety with no grave complications. Careful attention to all preoperative and intraoperative patient parameters is mandatory in order to perform whole-lung lavage under general anesthesia in adults or pediatrics