Percutaneous Lymphatic Embolization of Abnormal Pulmonary Lymphatic Flow as Treatment of Plastic Bronchitis in Patients With Congenital Heart Disease
Background—Plastic bronchitis (PB) is a potentially fatal disorder occurring in children with single ventricle physiology as well as other diseases such as asthma. In this study we report findings of abnormal pulmonary lymphatic flow, demonstrated by MRI lymphatic imaging, in patients with PB and percutaneous lymphatic intervention as a treatment for these patients.
Methods and Results—This is a retrospective case series of 18 patients with surgically corrected congenital heart disease and PB who presented for lymphatic imaging and intervention. Lymphatic imaging included heavy T2 weighted MRI and DCMRL. All patients underwent bilateral intranodal lymphangiogram and most patients underwent percutaneous lymphatic intervention. In 16/18 patients MR imaging and/or lymphangiogram demonstrated retrograde lymphatic flow from the TD toward lung parenchyma. Intranodal lymphangiogram and TD catheterization was successful in all patients. 17/18 patients underwent either lymphatic embolization procedures or TD stenting with covered stent to exclude retrograde flow into the lungs. One of the two patients that didn't have retrograde lymphatic flow did not undergo a lymphatic interventional procedure. A total of 15/17(88%) patients that underwent an intervention had significant symptomatic improvement at a median follow-up of 315 days (range 45 - 770 days). The most common complication observed was non-specific transient abdominal pain and transient hypotension.
Conclusions—In this study we demonstrated abnormal pulmonary lymphatic perfusion in most patients with PB. Interruption of the lymphatic flow resulted in significant improvement of symptoms in these patients and in some cases, at least temporary resolution of cast formation.
- Received September 29, 2015.
- Revision received January 21, 2016.
- Accepted February 1, 2016.