Introduction
WHOLE LUNG LAVAGE (WLL) remains the gold standard therapy for Pulmonary Alveolar Proteinosis(PAP) which is a rare disease characterized by alveolar accumulation of surfactant material with resulting hypoxemia and reduced lung function.In WLL it removes proteinaceous material from affected lung and it provides a long lasting benefits in majority of the patients. Here we have come up with the case of 32 year old male presented with chief complaints of Dyspnea on exertion since 3 months,progressed from mmrc 2-3 in 1month,dry cough 3months which was evaluated from outside hospital.There HRCT was done which showed B/L lung crazy pavement appearance,S/o PAP? Infective? PFT-FEV1/FVC=0.811(97%),FEV=2.4(57%),FVC=2.96(59%).There diagnostic BAL(Broncho Alveolar Lavage) was performed which was labelled as Non Proteinosis fluid and PAP stain negative and referred to Narayana Hrudayalaya for further evaluation.
Methods
In NH patient underwent VATS Biopsy(Vedio Thoracoscopic Biopsy) and was reported as PULMONARY ALVEOLAR PROTEINOSIS [HDE:Amorphus Easinophilic PAS positive material within alveoli].Pateint was admitted for WLL ,he was found to have left sided Pneumothorax .Managed conservatively in MICU for one day with O2 support.Since left lung was already compromised due to pneumothorax WLL was postponed for four days.
Results
Patient underwent WLL for left lung which was performed under General anaesthesis with lung separation obtained by Double Lumen Endotracheal tube.While Mechanical Ventilation was maintained in Right lung,the contralateral lung was repeatedly filled with 10200ml warm saline and then drained by gravity[retrieved 10100ml of protenosis fluid].The lavage was accompanied by chest percussion to emulsify the surfactant sediment and was continued until the lavage fluid became clear,judged by visual inspection. After five days patient underwent WLL for right lung with 10500ml warm saline and retrieved 10300ml proteinosis fluid.
Conclusion
WLL remains First line therapy for PAP.Patient showed improvement in dyspnea,PaO2,vital capacity and DLCO.WLL is currently a safe procedure in an experienced setting and yields durable benefit in majority of patients.
References
Khan A, Agarwal R:Pulmonary alveolar proteinosis. Respir
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Acknowledgements
Thanks Dr Murali Mohan B. V., Senior Consultant, Department of
Pulmonary Medicine, Mazumdar Shaw Medical Center
Thanks Dr Kedar Hibare, Department of Pulmonary Medicine,
Mazumdar Shaw Medical Center
Thanks Ms Moumitha
Chakraborty, Co-ordinator Respiratory Department, Narayana
Hrudayalaya Foundations
Thanks Dr Pramatha, Department of
Pulmonary Medicine, Mazumdar Shaw Medical Center