Research Day

Pseudo-Horseshoe Lung Herniation Following Tracheoesophageal Fistula Repair In A Child With Situs Inversus Totalis

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INTRODUCTION: Tracheoesophageal fistula with esophageal atresia (TEF-EA) is a common congenital upper respiratory tract malformation requiring surgical repair. Situs inversus complicates the surgical approach of TEF-EA repair. We describe an extremely rare complication of TEF-EA surgery, a pseudo-horseshoe lung herniation in a patient with situs inversus totalis.

CASE HISTORY: A four-year-old girl presented with respiratory distress and severe cough. Past medical history included TEF-EA surgical repair via open thoracotomy, situs inversus totalis, an atrial septal defect, and recurrent pneumonia. Due to the recurrent pneumonia, impaired airway clearance, and situs inversus, primary ciliary dyskinesia was suspected. Bronchoscopy elucidated bronchomalacia in the left subsegmental airway. Subsequent chest x-ray showed an abnormal lucency in the left lower lobe region. Computed tomography revealed the left lung was hyperinflated and herniated across the midline. The herniated lung section had no visible arterial supply, suggesting abnormal pseudo-horseshoe lung, rather than sequestration. Lung herniation occurred through the site of TEF-EA repair.

DISCUSSION: There are no known reports of such a lung herniation following TEF-EA repair. The patient's primary ciliary dyskinesia, atrial septal defect, and passive smoke exposure likely contributed to recurrent pneumonia and severe cough. Increased pleural cavity pressure generated by violent coughing may have provided enough force to cause the lower segment of right lung to herniate through the hole remaining from TEF repair. Bronchoscopy and persistent data collection were crucial in diagnosing such a rare complication causing serious morbidity in a patient with a complex constellation of symptoms.

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