Affiliation:
1. Irkutsk State Medical Academy of Postgraduate Education (ISMAPgE – Branch Campus of the FSBEI FPE RMACPE MOH Russia)
2. Irkutsk State Regional Children’s Clinical Hospital
Abstract
Objective: to present a case of successful treatment of rare severe lung lesions in a child.Methods: the article analyzes the result of the treatment of lung histiocytosis in a child aged 4 years. The clinical manifestation of the disease began with an increase in the parotid lymph nodes, otitis, pneumonia, and rapidly progressive respiratory failure. On the roentgenogram, bulle transformation of both lungs was noted. The severity of the disease was complicated by bilateral pneumothorax, the formation of bronchopleural fistulas. Histiocytosis of the lungs from Langerhans cells (GCR) was determined morphologically when examining tissue taken at thoracoscopy. Endoscopically, the lung was without signs of inflammation, with multiple subpleural air cavities. The child underwent chemotherapy (vinblastine, prednisone), repeated drainage of the pleural cavity with recurrent intense pneumothorax. For health reasons, a longitudinal sternotomy was performed, total decortication of the costal pleura on both sides (mechanical pleurodesis), which contributed to subsequent fixation of the lung to the chest wall and prevention of recurrent total stressed pneumothorax. Twice developed limited pneumothorax was stopped by drainage and chemical pleurodesis.Results: Against the background of ongoing complex therapy (drainage of the pleural cavity, mechanical and chemical pleurodesis, chemo- and antibacterial therapy), the condition improved (clinically and radiographically). The child was discharged on day 152 of treatment. On the control MSCT of the lungs, eight months after discharge, there are minimal signs of bullous transformation, and the symptoms of pneumosclerosis of both lungs are moderate. There are no manifestations of pulmonary hypertension.Conclusion: verification of the diagnosis of pulmonary GLC is very difficult. The complex treatment requires the participation of a wide range of doctors, including a pediatric surgeon.
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