title: Successful treatment of plastic bronchitis by selective lymphatic embolization in a Fontan patient.
abstract: Plastic bronchitis is a rare and often fatal complication of single-ventricle surgical palliation after total cavopulmonary connection. Although lymphatic abnormalities have been postulated to play a role in the disease process, the etiology and pathophysiology of this complication remain incompletely understood. Here we report on the etiology of plastic bronchitis in a child with total cavopulmonary connection as demonstrated by magnetic resonance (MR) lymphangiography. We also report on a new treatment of this disease. The patient underwent noncontrast T2-weighted MR lymphatic mapping and dynamic contrast MR lymphangiography with bi-inguinal intranodal contrast injection to determine the anatomy and flow pattern of lymph in his central lymphatic system. The MRI scan demonstrated the presence of a dilated right-sided peribronchial lymphatic network supplied by retrograde lymphatic flow through a large collateral lymphatic vessel originating from the thoracic duct. After careful analysis of the MRI scans we performed selective lymphatic embolization of the pathologic lymphatic network and supplying vessel. This provided resolution of plastic bronchitis for this patient. Five months after the procedure, the patient remains asymptomatic off respiratory medications.
authors.affiliation: Division of Cardiology and doriy@email.chop.edu.
authors.forename: Yoav
authors.initials: Y
authors.lastname: Dori
authors.affiliation: Department of Radiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; and.
authors.forename: Marc S
authors.initials: MS
authors.lastname: Keller
authors.affiliation: Division of Cardiology and.
authors.forename: Jack
authors.initials: J
authors.lastname: Rychik
authors.affiliation: Division of Interventional Radiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Maxim
authors.initials: M
authors.lastname: Itkin
mesh_terms: D001991:Bronchitis / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy*; D002648:Child; D004108:Dilatation, Pathologic; D004621:Embolization, Therapeutic / Q000379:methods*; D018729:Fontan Procedure / Q000009:adverse effects; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000601:surgery; D008208:Lymphatic System / Q000473:pathology; D042601:Lymphatic Vessels / Q000503:physiopathology; D008220:Lymphography; D008279:Magnetic Resonance Imaging; D008297:Male; D011183:Postoperative Complications / Q000628:therapy; D013897:Thoracic Duct / Q000503:physiopathology*
keywords: Fontan; lymphatics; plastic bronchitis; single ventricle