Thoracic Duct Embolization for Noonan Syndrome: A Case Report

Background: Noonan syndrome (NS) consists of a variety of congenital anomalies, and less than 20% of NS cases are associated with lymphatic malformations. Chylothorax is a critical complication of NS lymphatic abnormalities, where thoracic duct embolization can be used as an effective conservative management.

Case: A 35-year-old female patient, known case of Noonan syndrome, was admitted with recurrent episodes of spontaneous chylothorax. Lymphangiography revealed congenital anomalies of the thoracic duct, including left bronchitis plastica and lymphangiectasia in the upper abdominal and thoracic lymphatic channels with lymphatic malformation in the right paratracheal space. During the admission, patient underwent several ineffective conservative and surgical treatment of effusion drainage, thoracic duct embolization, octreotide and video-assisted thoracoscopic thoracic duct ligation. Then, the patient underwent a second unsuccessful trial of direct cannulation and embolization, except that the injected lipiodol achieved adequate embolization of the abnormal duct and improved the patient's symptoms.

Discussion: We highlighted the process of treating patients with Noonan syndrome presenting with recurrent chylothorax due to lymphatic malformations. Complex cases may need multiple trials of embolization to achieve the anticipated results, especially when conservative and surgical management are not effective.

Publication History

Article published online:
09 February 2023