BACKGROUND: The authors present a rare clinical case of stenosis of the main, intermediate and upper lobe bronchi of the right lung complicated by total lung atelectasis in an infant. They discuss issues of complex disease diagnostics, analyze X-ray and bronchological images, assess outcomes after multidisciplinary approach in the treatment of a premature baby as well as analyze data of catamnestic observation. CLINICAL CASE DESCRIPTION: Girl T., one of monochorionic diamniotic twins, was born at 27 week gestation in 2021 with weight at birth 825 grams, Apgar score 4/5. Since her birth, she had artificial lung ventilation due to her deep immaturity and developed severe respiratory distress syndrome. At the age of 19 days, total atelectasis of the right lung was diagnosed in the child as a result of stenosis formation in the main, intermediate and upper lobe bronchi which was confirmed by clinical and radiological findings. A probable etiology of acquired stenosis is consequences of prolonged intubation under insufficiently formed cartilaginous framework. Chlamydial infection, detected in the child, could also support inflammatory process in the bronchi. The child was examined with the following instruments: radiography, multispiral computed tomography-angiography of the thoracic cavity, bronchofibroscopy, echocardiography. Laboratory tests: polymerase chain reaction of blood and sputum for herpes viruses type 1–6, mycoplasma, ureaplasma, Chlamydia pneumonia; blood enzyme immunoassays — antibodies to viruses of Herpesviridae type 1–6, Chlamydia trachomatis. Conservative therapy provided only temporal improvement; atelectasis was relapsing. At the age of 5 months and 20 days, the child underwent bronchoplasty with resection of damaged areas of the bronchi. Further, age-related diameter of the bronchial lumen was maintained by the staged bougienage. Catamnestic observation lasted for 3 years. Late timing of surgical intervention is explained by severe problems in a deeply premature and immature child that required immediate treatment (intraventricular hemorrhages, hydrocephalus, retinopathy, etc.). Effective surgical treatment (bronchoplasty), active growth of lung tissue at the first year of life as well as reasonable rehabilitation allowed to obtain good long-term outcomes. By the age of 1 year 2 months, normalization of right lung airiness was achieved. Currently, there is a slight reduction in right lung volume. Multispiral computed tomography-angiography revealed a few cylindrical bronchoectases in the segmental and subsegmental bronchi of the upper and middle lobes of the right lung which do not have clinical equivalents yet. The child is under pulmonologist's observation and management. CONCLUSION: A multidisciplinary approach to the treatment of complicated patient's state and high compensatory capabilities of neonatal and infantile periods have promoted the restoration of normal lung aeration and functioning after long-lasting lung atelectasis
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