Dados do Trabalho

Título

Dynamic Contrast Magnetic Resonance Lymphangiography and Selective Lymphatic Embolization in a Patient with Plastic Bronchitis

Introdução e/ou Fundamentos

Introduction: Plastic Bronchitis (PB) is a rare and potentially fatal condition, where lymphatic fluid leaks into the bronchi and solidifies. The symptoms include wheezing, cough, expectoration of bronchial casts, and potential airway obstruction and acute respiratory distress.

Métodos

Case Presentation: A 30-year-old male presented with a history of cough and expectoration of bloody bronchial casts for the past 8 years. He had recurrent pneumonias as a child and was previously diagnosed with non-allergic asthma. Despite extensive testing at local hospital including bronchoscopy, echocardiography and CT-scan the diagnosis was not revealed. Symptoms persisted after three times embolization of tortuous, dilated bronchial arteries. Pathological examination of cast expectoration showed proteinaceous material with lymphocytes, indicating lymphatic origin. Subsequently, an Intra-Nodal Dynamic Contrast-enhanced Magnetic Resonance Lymphangiography (IN-DCMRL) identified abnormal lymphatic vessels in the thorax. Contrast agent was injected via bilateral inguinal lymph node access followed by heavily T2 weighed imaging and dynamic time-resolved imaging with interleaved stochastic trajectories (TWIST). Immediately after, the confirmed abnormal lymphatic vessels were catheterized and intra lymphatic methylene blue was injected under simultaneous bronchoscopy to confirm leakage into the bronchi. Subsequently, the selected abnormal lymphatic vessel causing leakage of lymphatic fluid into the bronchi was embolized using intra lymphatic glue (histoacryl). The patient was discharged 4 days after the procedure. Two months later he was doing well, had no episodes with cast expectoration and reports better breathing.

Resultados e Conclusões

Discussion: PB is a rare and potentially underdiagnosed disease. While most PB cases are described in patients with a Fontan circulation it is also seen following pulmonary infections and among but not limited to asthma and sickle cell patients. It is a potentially life-threatening disease requiring frequent hospitalizations. We describe a case of an otherwise healthy, young man, with a delayed diagnosis of PB and subsequently successfully diagnosed and treated using the novel techniques of IN-DCMRL and percutaneous, selective intralymphatic embolization – the first of its kind in Denmark.

Palavras Chave

Keywords: lymphatics, plastic bronchitis