Primary Pulmonary Lymphatic Flow Disorder Associated Plastic Bronchitis

Lymphatic plastic bronchitis (PB) most commonly occurs in children with congenital heart disease as a result of secondary pulmonary lymphatic flow disorder (PLFD). However, PB caused by primary PLFD is rare. The clinical symptoms of lymphocytic PB are nonspecific; thus, the diagnosis may be long delayed, especially in the absence of chylothorax. We made a retrospective analysis of two children diagnosed with PB due to primary PLFD, in order to contribute to further understanding of these disorders. Patient 1, an eight-year-old boy, presented with chronic productive cough and expectorated milky-white mucous plugs accompanied by intermitted wheezing for one year. Patient 2, a nine-month-old girl, presented with episodes of acute respiratory distress with expectoration of milky-white bronchial casts for four months. There was no obvious evidence of infection in either child. Bilateral thickening of bronchovascular bundles and interlobular septal, as well as multiple patchy ground-glass opacities were seen on chest computed tomography (CT) in both patients. Lymphangioscintigraphy demonstrated pulmonary lymph reflux in both patients and slowed lymphatic drainage of the lower limbs in patient 1. Primary PLFD was considered for both patients, and a diagnosis of yellow nail syndrome was made in patient 1. Both patients received lymphatic interventional treatment, but all experienced recurrence following the procedure.

Plastic Bronchitis—A Serious Rare Complication Affecting Children Only after Fontan Procedure?

Background: Plastic bronchitis (PB) may occur not only in children following palliative Fontan procedure but also in those without underlying heart disease. We aim to assess the clinical course, therapeutic measures, outcome, and follow-up of PB in children with congenital heart disease (CHD) and children without cardiac problems. Methods: This retrospective case series assessed children with PB admitted to hospital between 2015 and 2019. Parents or guardians of patients were contacted by e-mail or telephone between September 2017 and June 2019 to enquiry about recurrence of PB and strategy of treatment. The diagnosis of PB was based on the expectoration (spontaneous or during bronchoscopy) of endobronchial plugs. Results: This study delineated the clinical, histological, and laboratory features of plastic bronchitis in children following Fontan procedure (Group A) and in those without heart defects (Group B, non-CHD children). The main symptoms were cough accompanied by dyspnea, and hypoxemia with a decrease in oxygen saturation, often leading to acute respiratory failure. In children with CHD, the first episode of PB occurred at a relatively young age. Although chronic, i.e., lasting more than 3 weeks, inhaled therapy was implemented in both groups of patients, the recurrences of PB were observed. The mean time to PB recurrence after the first episode in Group A was longer than that in Group B (1.47 vs. 0.265 years, p = 0.2035). There was no re-episode with recurrence of PB in 3 cases out of 10 in total in Group A (30%) and 1 case out of 4 in total in Group B (25%). While the majority of children in Group A usually developed bronchial casts on the right side, the patients in Group B (without CHD) suffered from bronchial casts located only on the left side. Conclusions: Despite many similarities, clinical, histological, and laboratory studies in the children with plastic bronchitis after Fontan’s surgery and in children without heart defects suggest that there are differences in the course of the disease in patients without CHD, such as a more advanced age of the first episode of PB, the location of plastic casts on the left side, and a stronger role of inflammatory factors and mechanisms. Further research is needed to understand the pathophysiology of PB and choose the most appropriate therapy.

Plastic bronchitis associated with influenza A virus in children with asthma

Plastic bronchitis (PB) is a rare and potentially fatal disease characterized by acute progressive dyspnea caused by bronchial casts in the bronchial tree. We analyzed two children with asthma and PB who presented with high fever, cough and dyspnea. Both cases showed acute onset and rapid disease progression. Laboratory examination revealed that both children were infected with influenza A virus. Emergency fiberoptic bronchoscopy was performed within 20 hours of admission. Immediately after removing the bronchial casts, their dyspnea symptoms improved significantly, and they recovered after comprehensive treatment with antiviral drugs, antibiotics and glucocorticoids. When children with asthma have acute progressive and difficult-to-relieve dyspnea after infection with influenza A virus, clinicians should be aware of the possibility of PB and perform bronchoscopy as soon as possible to facilitate early diagnosis and treatment and improve patient prognosis.

P39 CHYLOPTYSIS AFTER ESOPHAGECTOMY FOR ESOPHAGEAL CANCER; A RARE COMPLICATION

Aim We present a case of chyloptysis after esophageal resection for esophageal squamous cell cancer. Symptoms were caused by chylous casts occluding the airways. This necessitated surgical ligation of the main thoracic duct and sealing the airway fistula with a latissimus dorsi muscle flap. Background & Methods Chyloptysis is rare and usually occurs spontaneously and secondary to disorders of the mediastinal lymphatic system. Chyloptysis manifests with the production of milky-white sputum or the formation and expectoration of bronchial casts. Initially no more than 20 cases have been reported in the literature (1,2). There are two mechanisms for the pathogenesis of chyloptysis: 1) congenital or acquired incompetence of the lymphatic valves and 2) occurrence of bronchopleural fistula in the context of chylous effusion (3,4). However, iatrogenic injury also can be a cause, in which injury to the thoracic duct leading to chylous pleural effusion with coexisting esofagotracheal fistula facilitates chyloptysis. Results A 66-year-old mal underwent a mini-invasive esophagectomy and gastric tube reconstruction for epidermoid cancer of the esophagus. Neoadjuvant chemo-radiation was given before operation. Post-operatively a minor chylous leak was managed by total parenteral nutrition for one week. He was discharged 15 days after operation. Four weeks after surgery he was readmitted because of fever, hypoxemia, wheezing, and expectoration of bronchial casts. He had fever, C-reactive protein level was 353 mg/l and white blood cell count 15.4 E9/l. He was intubated for respiratory failure and taken to ICU for treatment of aspiration pneumonia. Both chest x-ray and i.v. contrasted computed tomography (CT)of the chest confirmed bilateral aspiration pneumonia ( Figure 1). In bronchoscopy, occluding whitish mass was evacuated from both sides of the airways and this was thought to be because of aspiration (Figure 2). Infection responded to antibiotics and he was weaned from mechanic ventilation with tracheostomy until his symptoms recurred. Fiberoptic bronchoscopy revealed again milky-white sputum on both sides of the bronchial three. This time, a hole in the posterior tracheal wall was noticed and tracheal stent was applied to cover possible fistula to the esophagus or gastric conduit. However, in gastroskopy, such fistula could not be confirmed. Patient was under total parenteral nutrition (TPN) and after seven days since readmission, jejunal feeding was started. This caused a third deterioration period. Analysis of the bronchial aspiration revealed an elevated triglyceride level which was consistent with chyle and chylous fistula to the airways was therefore the most likely diagnosis. A right posterolateral thoracotomy was performed, and during surgery, thicker fibrous tissue was removed around the fistulae. Chylous reflux was observed form the fistula. Latissimus dorsi muscle flap was applied to cover the TEF and a main thoracic duct was controlled by ligating all soft tissue between descending aorta and azygous vein at the level of the diaphragm. After surgery, patient had TPN for 10 days. Enteral feeding was started to jejunal tube and we served a fat-free diet gradually increasing the fat content. Patient had tracheostomy and 2 weeks after operation he was noted to have recurrent TEF, and esophageal stent was applied. Postoperative recovery showed no further chyloptysis and he was discharged eventually 96 days after original operation. Conclusion Surgical ligation of thoracic duct resulted in complete resolution of chyloptysis. Surgery is the treatment of choice, although radiation therapy, chemotherapy, laser, and sclerosing agents have been described with varying success. To our knowledge, this is the first reported case of iatrogenic induced chyloptysis after esophageal resection.

Expectoration of bronchial casts in association with Ramipril treatment

We report of a 26-year-old female patient who was referred to our centre with congestive heart failure (CHF). Acute myocarditis with a high Parvovirus B19 virus load was diagnosed by myocardial biopsy. CHF improved after start of ramipril 5 mg/d, metoprolol, diuretics, immunoglobins, and a 24-hour infusion of levosimendan. Soon after initiation of medical therapy, the patient started to expectorate bronchial casts with varying frequencies (three times per week to five times daily). Thorough pneumological workup, including histology of the casts, microbiology, and a CT scan of the lungs, did not reveal any cause for bronchial cast formation. Inhalative corticoids were started without any benefit. Two years later, cardiac catheterisation demonstrated normalised left ventricular function. LV end-diastolic pressure, however, was still elevated at 14 mmHg. Endomyocardial biopsies at this time were negative for virus genome. Finally, we changed afterload reduction therapy from ramipril to candesartan. Within 24 hours, expectoration of bronchial casts terminated. Four weeks later, re-exposition to ramipril prompted immediate re-appearance of cast formation, which again stopped with switching back to candesartan. Finally, we were to prove that treatment with ramipril resulted in bronchial cast formation in this patient.