In many tertiary care pediatric centers, pediatric cardiac surgeons are responsible for the care of patients with a vascular compression syndrome. Nevertheless, pediatric surgeons need to be aware of the signs and symptoms of these uncommon anomalies and should be familiar with the basic anatomy and principles of management. It is not uncommon to encounter a vascular anomaly during a thoracotomy or thoracoscopy being performed for esophageal atresia or other mediastinal lesion, in which case it is important to know what to do and, perhaps more importantly, what not to do. Such a finding usually prompts an immediate review of available imaging to see if something was missed on the initial reading. The next step is to carefully dissect and define all structures, including those on the opposite side of the mediastinum, being very careful not to injure important adjacent structures. No vessel should be divided unless it is clear that a true ring exists and that there is brisk flow in the descending aorta after test occlusion of the vessel in question.
For certain vascular anomalies, the thoracoscopic approach is safe in experienced hands. Vessels can be ligated with a variety of techniques and most surgeons prefer to use at least two for added safety. In small children, most vessels can be clipped and then divided with bipolar electrocautery or the harmonic scalpel. Other options include simple suture ligation and oversewing the end with a running permanent monofilament suture. Stapling devices would probably work well but there is rarely enough space to manipulate the device and fire it properly without fear of inadvertently incorporating an important adjacent structure or nerve.
Bleeding is the most feared complication as these are large, high-pressure, high-flow vessels that have a tendency to retract and become extremely difficult to control. Even when the bleeding is stopped, much to the relief of all present, the risk of injury to other structures (recurrent laryngeal nerve) is significant. A contingency plan must be prepared in advance, especially if the procedure is being performed thoracoscopically, with the ability to perform a rapid thoracotomy at a moment’s notice. Proximal and distal control of all vessels can be difficult and time-consuming but it is time well invested.
Finally, chylothorax is a particularly distressing complication and one that is often frustrating to manage. It is best prevented by using meticulous technique and staying close to all vessels being dissected. At the conclusion of the operation, one should take a moment to observe the operative field closely for signs of a chyle leak and place sutures to repair a leak if one is found. Some have suggested that application of a commercially available fibrin sealant works well to help small chyle leaks to seal. If a chylothorax occurs, management includes establishing drainage with a chest tube, initial bowel rest, and a great deal of patience. If the leak persists after 3 or 4 weeks, operative management should be considered, which can be difficult given that this entails a redo operation in the chest.