Results and Case Presentation
patient 1 An eight-year-old boy presented to our department with a complian of chronic productive cough, expectorated milky-white mucous plugs, resembling bronchial casts every 4-5 days (Figure. 1A), accompanied by intermitted wheezing for 1 year. Treatment of antibiotics, inhaled corticosteroids and brinchoidilaters fail to control the symptoms. His medical history revealed recurrent pneumonia as well as intermitted wheezing from two years of age, and he was diagnosed with asthma. His mother noticed the slow yellowing and thickening of the first toenail on his right foot about three years ago. The nail changes had been interpreted as onychomycosis. He had no history of trauma or surgery. On physical examination, he was normally developed; slight shortness of breath was noticed with a respiratory rate of 28 breaths/min and oxygen saturation of 97% on room air. Chest auscultation showed decreased air entry at the left lung base. The first toenail on his right foot had been removed before presentation to the hospital and a small and yellowish new toenail was observed (Figure. 1B). There was no peripheral edema, and the remainder of his examination was unremarkable.
The patient’s white blood cell (WBC) count, eosinophil count, and C-reactive protein (CRP) were normal. The serum levels of aspergillus specific IgE, 1,3-β-glucan, and galactomannan were normal. Antineutrophil cytoplasmic antibodies, antinuclear antibodies, and rheumatoid factor were all negative. Sputum cultures were positive forCandida albicans , while bacterial and mycobacterium cultures were negative. Fungal microscopic examination and culture of the first toenail were negative. Serial chest computed tomography (CT) revealed migratory patchy ground-glass opacities and focal mild bronchiectasis with mucus impactions. Bronchoscopy showed that the airway mucosa was rough and swollen, with thin white secretions attached; however, no casts were observed. The bronchial lavage fluid (BALF) was slightly milky-white and turbid; fluid cultures showed no pathogens. BALF cell composition analysis was as follows: small lymphocytes 60%, macrophages 20%, exfoliated epithelial cells 20%. The pulmonary function test showed mild obstructive ventilatory disturbance and the bronchodilation test was negative. An echocardiogram showed no abnormalities.
Suspicion of allergic bronchopulmonary mycosis (ABPM) was arised after the patient’s admission. He was treated with fluconazole (8 mg/kg.d) and prednisone (1 mg/kg.d, gradually decreased to 0.5 mg/kg.d) for three weeks. However, his cough and expectoration of mucous plugs worsened. Repeated chest CT showed the progression of lung lesions with bilateral thickening of bronchovascular bundles, multiple patchy ground-glass opacities, and interlobular septal thickening, mainly in the middle-inferior lung, indicating pulmonary lymphatic stasis (Figure. 1C). Evaluation of the lymphatic system by lymphangioscintigray was performed subsequently and the results demonstrated slow lymphatic drainage in both lower limbs, abnormal distribution of the radiotracer in the bilateral hilar and lungs, suggesting pulmonary chylous reflux. Moreover, continuous widening of the left venous angle and the radiotracer was retained in the proximal end of the thoracic duct were observed, suggesting thoracic duct obstruction (Fig. 1D).
Based on the patient’s clinical picture, medical history, chest CT and lymphangioscintigraphy findings, in addition to the yellow toenail on his right foot, the patient was finally diagnosed with yellow nail syndrome (YNS) and lymphatic PB. He was treated with a low-fat diet and percutaneous embolization of the thoracic duct was performed three weeks later. His respiratory symptoms were relieved. However, three months after the surgery, he experienced recurrence of bronchial cast expectoration. His parents refused further evaluation and treatment as he had developed no other symptoms.
Patient 2 A nine-month-old girl was referred to our department for episodes of acute respiratory distress with cough and expectoration of milky-white bronchial casts requiring bronchoscopy treatment for 4 months. During the course of the disease, there was no obvious fever. She had been admitted to the local intensive care unit and required mechanical ventilation two times because of severe bouts of dyspnea. Bronchoscopy demonstrated many milky-white casts blocking the airway (Figure. 2A); these were extracted by aspiration and her dyspnea and hypoxemia can be temporarily relieved. Chest CT demonstrated diffuse thickening of the interlobular septa and bronchovascular bundles, left partial atelectasis and consolidations, as well as enlargement of the mediastinum and hilar lymph nodes (Figure. 2B). Diagnoses of severe pneumonia and PB were made. However, despite vigorous therapy with antimicrobials (including antifungal drugs), intermittent systemic and inhaled glucocorticoids, and intravenous immunoglobulin, she continued to exhibit chronic expectoration of bronchial casts and episodes of acute respiratory distress requiring bronchoscopy to clear the airways about every two weeks. Her birth history was normal. She had normal development and no history of trauma or surgery. Physical examination revealed tachypnea, a respiratory rate of 42 breaths/min, and oxygen saturation of 90% on room air. Chest auscultation showed bilateral moist rales and wheezing. Heart auscultation and the remainder of her examination was unremarkable.
The patient’s WBC count, eosinophil count, hemoglobin, and CRP were normal. Sputum and BALF cultures showed a small amount ofKlebsiella pneumoniae. Serum and nasopharyngeal aspiration for influenza virus, adenovirus, and mycoplasma detection were all negative. The echocardiogram showed no abnormalities. Abdominal ultrasound showed multiple cysts of the spleen. Lymphangioscintigraphy was performed subsequently and revealed thoracic duct obstruction, abnormal distribution of the radiotracer in the bilateral hilar and lungs, suggesting pulmonary chylous reflux (Figure. 2C).
Given her early age of onset, congenital lymphatic dysplasia was considered. Splenic cystic lesions were observed on abdominal ultrasound, suggesting extrathoracic involvement. The child was treated with high medium-chain triglycerides (MCT) milk powder, and oral rapamycin was administered (0.8-1 mg/m2.d). Her symptoms had gradually improved and there were no further episodes of acute dyspnea for one month. Serum sirolimus levels were maintained at 8-13 ng/mL without any adverse reactions. Because the patient was still coughing up casts intermittently, percutaneous lymphatic intervention was performed (thoracic duct and right lymph duct outlet compression band loosening and stenotic adventitia stripping). Unfortunately, on postoperative day ten, she was readmitted with acute respiratory distress requiring emergent intubation. Bronchoscopy demonstrated that tenacious bronchial casts were obstructing the airways. After a series of bronchoscopies, her acute respiratory distress could not be alleviated, and thus, surgical ligation of the thoracic duct was performed. Her respiratory symptoms gradually improved. Currently, four months post-surgery, she is asymptomatic.