Fatal Pulmonary Lymphangiectasia Manifesting After Repeated Surgeries for Intractable Chylopericardium and Chylothorax in a 20-Month-Old Girl
A 20-month-old girl was admitted to our hospital because of progressive dyspnea on exertion and a 10-month history of failure to thrive. She had bilateral hearing impairment. The family history was unremarkable. The patient had a hemangioma on the temporal portion of the head and no edema on the legs or face. A chest radiograph (Figure 1A) showed enlargement of the cardiac silhouette with pulmonary congestion. Cardiac ultrasound and computed tomographic scanning of the chest (Figure 1B) revealed copious pericardial effusion with no structural heart diseases. Diagnostic pericardiocentesis produced 150 mL of milky fluid (Figure 1C), which confirmed a diagnosis of chylopericardium because of positive Sudan III staining, a high triglyceride concentration, and a predominance of lymphocytes (>95%). Despite a medium-chain triglyceride-enriched diet and total parenteral nutrition for more than 1 month after pericardiocentesis, the massive pericardial effusion persisted, which prompted surgical management including the clipping of the thoracic duct and creation of a left pericardial window. A chest radiograph obtained 2 months after the operation (Figure 2A) showed a large left-sided pleural effusion, including 480 mL of straw-colored chyle with predominance of lymphocytes. A prominent pleural effusion, persistent under conservative management with total parenteral nutrition and pleural drainage for another 7 weeks, prompted the second surgical intervention, which included multiple ligations and clipping of the thoracic duct. Because the thoracic surgeons observed a pinkish, turbid fluid resembling chyle oozing from the left parietal pleura, dibekacin (Meiji Seika Kaisha Ltd, Tokyo, Japan) was infused into the left-sided pleural cavity for sclerotherapy. A chest radiograph obtained 1 month after the second operation (Figure 2B) showed a right-sided pleural effusion, necessitating placement of a chest tube. Despite conservative treatment for another 10 weeks, including intravenous administration of octreotide (Novartis, Basel, Switzerland), chyle loss from the right chest tube persisted, fluctuating between 200 and 700 mL daily. Such intractable chylopericardium and chylothorax prompted a third surgery, which included adhering the right parietal pleura to the pericardium with argon laser. On gross examination, the lung had an irregular surface (Figure 3A and Data Supplement Movie), with scattered nodular changes that contained cystic spaces on the cut surface of the lung (Figure 3B and Data Supplement Movie). Microscopic findings of tissues obtained during the third operation demonstrated lymphatic dilatation in the lung (Figure 4A), the pericardium (Figure 4B), and the parietal pleura (Figure 4C), confirming pulmonary and pericardial lymphangiectasia. A chest radiograph obtained after the third operation (Figure 2C) showed bilateral interstitial infiltrate of the lung fields. The patient subsequently experienced severe respiratory distress and marked lymphedema in the lower limbs, neck, and face. Despite maximum support, the patient developed progressive respiratory failure and died 6 months after the third operation.
In children, pulmonary lymphangiectasia, characterized by dilatation of the pulmonary lymphatics,1,2 is a rare disorder that is of unknown origin unless accompanied by congenital heart diseases with severe pulmonary venous hypertension. The lymphatic abnormality can be localized to the lung or be part of a more widespread abnormality of lymphatic drainage.1,2 The prognosis of the former category is considered to be poor.3,4 The patient in the present case was diagnosed with the latter disease because of the multiple sources of lymphatic leakage in the vicinity, the typical histology in such tissues, and the occurrence of fatal pulmonary lymphangiectasia and systemic lymphedema after the repeated surgeries for such lymphatic sources. A series of images from the present case illustrated the clinical significance of otherwise masked pulmonary lymphangiectasia in the surgical treatment for intractable lymphatic leakage in the thorax and gives us insights into the close interaction between intra- and extrapulmonary lymphatic systems in this setting.
The online-only Data Supplement, which contains a movie, is available with this article at http://circ.ahajournals.org/cgi/content/full/117/14/1894/DC1.