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.