gms | German Medical Science

Background: Thoracoscopic Aortopexy (TA) is an effective treatment for severe tracheomalacia when conservative treatment has failed. In children with esophageal atresia (EA) and tracheoesophageal fistula (TEF) tracheomalacia is a well-known consequence following repair of EA and TEF respectively. We report on a patient with VACTERL-syndrome who previously underwent thoracoscopic surgery for repair of EA and TEF respectively. TA could be successfully carried out at the age of 4 months.

Materials and methods: The patient was born at 35 weeks of gestation with a birthweight of 2080 g and diagnosis of VACTERL-syndrome, including EA and TEF, anal atresia, left persistent superior vena cava, grade 5 bilateral vesical-ureteral reflux and trigonocephalus. Following primary thoracoscopic repair of EA and TEF, the patient developed severe tracheomalacia in the further course.

Results: Thoracoscopic repair for EA and TEF respectively was carried out at the first day of life. In addition the patient received a sigmoid colostomy. Postoperative course was uneventful and the patient could be discharged home at 6 weeks of age. Elective re-admission for follow-up as well as for a PSARP was scheduled and carried out. Further course at home was then complicated by an increasing frequency of recurrent cyanotic and obstructive apnea. Patient was therefore re-admitted for further evaluation and repeated bronchoscopy which revealed a high-grade tracheomalacia. TA was then performed at the age of 4 months. Postoperatively the patient suffered from a transient chylothorax, a repeated bronchoscopy proved a significant improvement of tracheomalacia and the patient could be discharged home. In the further course, the patient underwent surgery for trigonocephalus additionally. With regard to EA and TEF and tracheomalacia further follow-up was uneventful so far.

Conclusion: TA has been proven to be safe and effective in our case reported. Even in small infants and children who have been undergoing previous thoracoscopic surgery and with special regard to those patients suffering from more than just one malformation, the thoracoscopic approach offers less pulmonary impairment, faster recovery and avoids the morbidity and long term sequelae of (repeated) thoracotomy.