lung torsion is a very rare event that has been reported in only 9 cases in the pediatric literature1 - 8 ) ; it has not yet been reported in korean infants . its etiology appeared to be threefold : secondary to trauma , spontaneous , and after thoracic surgery . we experienced a case of lung torsion following esophageal atresia with tracheoesophageal fistula ( tef ) repair that required repeated detorsion and lobectomy surgeries . we describe the clinical features and methods for management with a brief review of the literature . a 30-year - old pregnant woman was transferred to our institution at 32 weeks ' gestation due to polyhydramnios . at 41 weeks of gestation an attempt to pass a nasogastric tube failed , and chest radiographs showed a pouch in front of the first thoracic vertebra . the baby was diagnosed with esophageal atresia with distal tef and underwent esophagoesophagostomy and fistula ligation on the first day of birth . the pleura dissection was carried superiorly to the apex of the chest and inferiorly for a couple of intercostal spaces . pulmonary lobes were retracted to expose the esophagus as usual manner . during the operation , a chest tube was placed in the thoracic cavity . on the first postoperative day , the patient had aggravated respiratory distress with tachypnea and bloody secretions from the endotracheal tube . 1a ) . despite repeated efforts of endotracheal suctioning and physiotherapy , serial chest radiographs on the second postoperative day revealed rapidly progressed consolidation with volume expansion of the right hemithorax ( fig . 1b ) . a computed tomographic ( ct ) scan was performed and showed abrupt narrowing of the right upper lobar bronchus and no visible right middle lobar bronchus , with extensive consolidation ( fig . 2a ) . immediately , the baby underwent an emergency operation on day 3 from birth . intraoperative findings revealed 180 torsion of the rul and right middle lobe ( rml ) in a clockwise direction . after detorsion of both lobes , there was some immediate improvement in the color of the rul , but improvement was uncertain in the rml ( fig . 3 ) . although viability of the rml could not be proven , pexy of both lobes was performed . in spite of emergency rethoracotomy , respiratory distress signs and symptoms chest radiographs showed persistent opacification of the right hemithorax . a ct scan on the seventh day after reoperation showed consolidation in the rml ( fig . 2b ) . bronchoscopy was performed and revealed a patent airway for the rul but not for the rml . on the eighth day of second operation , the third operation was performed . intraoperative findings revealed that the pexy of both lobes were secure , and the rul had improved with regards to color , congestion and edema . on the other hand , rml appeared dark red and was still congested without any improvements from before . the rml was surgically removed . after the third surgery , respiratory distress signs and symptoms and radiologic examinations were improved . the baby was discharged on the 42nd day after birth . on follow - up at 6 months of age , the baby 's growth and development were normal , and radiologic findings were improved , except for evidence of resection of the rml ( fig . in adults , the overall incidence of lung torsion is reported to be 0.089% to 0.3% and is equally rare after thoracic surgery9,10 ) . its etiology appears to be threefold : secondary to trauma , after thoracic surgery , and spontaneously11 ) . predisposing factors of lung torsion have been known to include an airless lobe , a long and free lobar pedicle , the absence of a parenchymal bridge between contiguous lobes , pneumothorax or pleural effusion , and the transection of the inferior pulmonary ligament12 ) . since rotation of the bronchovascular pedicle results in airway obstruction and vascular compromise , adults diagnosed with lung torsion have a high mortality rate of 12 - 16%9 ) . in children , lung torsion is an extremely rare event that has been reported in only 9 cases in the pediatric literature ( table 1)1 - 8 ) . lung torsion usually occurs in children who have undergone thoracic surgery or have experienced blunt trauma . a patient with fever , tachycardia , tachypnea , elevated white blood count , dullness to percussion and reduced breath sounds on examination , malaise , or reduced movements should raise suspicion for lung torsion2,4,9 ) . however , there are no unique symptoms of lung torsion . the arterial blood gas analysis and oxygen saturation are usually normal , secondary to complete shunting of blood from the twisted lobe or segments13 ) . more specifically , radiological signs on chest radiographs may provide further confirmation of the presence of a rapidly progressing consolidated lobe , displacement of hilar structures , positional alteration of the pulmonary vasculature , bronchial cutoff or distortion , and lobar air trapping12 ) . a chest ct scan may show the interruption of the bronchus at the level of torsion and abnormal vascular relationships in the torsive lung or lobe12 ) . bronchoscopy may reveal a compressed or occluded lobar bronchus that resembles a " fish - mouth"11 ) . contrast pulmonary angiography has the advantage of direct evidence of stenosis or complete obstruction of kinking vessels9 ) . in our case , chest radiographs on the first postoperative day showed consolidation in the rul . at this time a ct scan showed abrupt narrowing of the right upper lobar bronchus and no visible right middle lobar bronchus , with extensive consolidation . the conventional treatment of lung torsion is to remove the affected lobes , especially if the affected lobes clearly appear necrotic7,9 ) . resection vs. detorsion of a lobe or lung with doubtful viability is controversial12 ) . among the reported 9 cases , one lobe ( rul ) was saved , and the other lobe ( rml ) was surgically removed on the eighth postoperative day . during the emergency exploration , the rml appeared to be less viable compared to the rul . after detorsion of both lobes , there was some immediate improvement in the color of the rul , but it was uncertain whether the color of the rml was improved . although it is difficult to determine the tissue viability , it could be helpful to discern whether the color of affected lobes is improved after detorsion . during the third operation on the other hand , rml appeared dark red and was still congested without any improvements from before . as the necrosis of lung parenchyma gradually progressed , parenchymal architecture might be destroyed and filled with inflammatory fluid . if consolidation of the affected lobe persists on ct scans , despite detorsion , it suggests a loss of tissue viability . another argument against detorsion is the risk that necrotic tissue and inflammatory mediators flow into the systemic circulation and could potentially induce a systemic inflammatory response , resulting in multiorgan failure and death15 ) . however , detorsion of the affected lobes did not result in sudden embolic events , septic condition , or death in the pediatric literature . it is critical to closely evaluate the ct for evidence of perfusion , because this would favor a more conservative intraoperative approach8 ) . in summary , a high index of suspicion is important for an early diagnosis in children who have undergone thoracic surgery or have experienced blunt trauma . we suggest that detorsion is recommended if affected lobes have high viability during the exploration , as evidenced by this report .
lung torsion is a very rare event that has been reported in only 9 cases in the pediatric literature but has not yet been reported in korean infants . 
 we present a case of lung torsion after tracheoesophageal fistula repair in an infant . 
 bloody secretion from the endotracheal tube and chest radiographs and computed tomographic scan results indicated lung torsion . 
 emergency exploration indicated 180 torsion of the right upper lobe ( rul ) and right middle lobe ( rml ) . 
 after detorsion of both lobes , some improvement in the rul color was observed , but the color change in the rml could not be determined . 
 although viability of the rml could not be proven , pexy was performed for both the lobes . despite reoperation , clinical signs and symptoms 
 did not improve . 
 the bronchoscopy revealed a patent airway in the rul but not in the rml . 
 finally , the rml was surgically removed . 
 the patient was discharged on the 42nd day after birth .