The Harlequin phenomenon after thoracoscopic repair of esophageal atresia and tracheoesophageal fistula: Is there any coincidence?

Abstract

The experience and number of endoscopic surgery procedures applied in newborns are constantly growing, showing efficacy in many thoracic and abdominal conditions. However, there is an ongoing debate concerning the safety of this technique and the influence on the developing organism. Due to the relatively recent introduction of these methods in the therapy of congenital anomalies, we still have quite little knowledge about their long-term sequelae. The Harlequin phenomenon is a complex, little known and probably heterogeneous anomaly. It involves episodes of sharply demarcated erythema with the dividing line running exactly along the midline. We observe the appearance of episodes of unilateral flushing and sweating on the left half of the body in children who have been operated on using the thoracoscopic approach in the first days of life, due to esophageal atresia and distal tracheooesophageal fistula (EA/TEF), which are very similar to the Harlequin phenomenon. During long term follow-up visits, parents of 2 out of 55 children, who had been operated on during the period 2005–2011, turned our attention to these unusual symptoms. To our knowledge, this is the first report of the Harlequin phenomenon following esophageal surgery. The aim of the study is to analyze the possible pathogenesis of the observed anomaly, in the context of the existing congenital defect, and the operative technique used.

Keywords

• Harlequin phenomenon
• Thoracoscopy
• Esophageal atresia