Pediatr Radiol DOI 10.1007/s00247-014-3182-y
CASE REPORT
Idiopathic chylopericardium treated by percutaneous thoracic duct embolization after failed surgical thoracic duct ligation Malachi Courtney & Raj R. Ayyagari
Received: 6 February 2014 / Revised: 6 April 2014 / Accepted: 3 September 2014 # Springer-Verlag Berlin Heidelberg 2014
Abstract Chylopericardium rarely occurs in pediatric patients, but when it does it is most often a result of lymphatic injury during cardiothoracic surgery. Primary idiopathic chylopericardium is especially rare, with few cases in the pediatric literature. We report a 10-year-old boy who presented with primary idiopathic chylopericardium after unsuccessful initial treatment with surgical lymphatic ligation and creation of a pericardial window. Following readmission to the hospital for a right-side chylothorax resulting from the effluent from the pericardial window, he had successful treatment by interventional radiology with percutaneous thoracic duct embolization. This case illustrates the utility of thoracic duct embolization as a less-invasive alternative to surgical thoracic duct ligation, or as a salvage procedure when surgical ligation fails. Keywords Chylopericardium . Chylothorax . Pericardial effusion . Thoracic duct embolization . Child . Interventional radiology
Introduction Chylopericardium is a rare diagnosis in pediatric patients. Most often, it is caused by thoracic duct injury sustained during cardiac surgery. We report a 10-year-old boy who presented with primary idiopathic chylopericardium that was successfully treated with M. Courtney : R. R. Ayyagari Yale New Haven Hospital, Yale School of Medicine, New Haven, CT, USA M. Courtney : R. R. Ayyagari (*) Department of Radiology, Division of Interventional Radiology, 789 Howard Avenue, P.O. Box 208042, New Haven, CT 06520-8042, USA e-mail:
[email protected] percutaneous thoracic duct embolization after failed surgical duct ligation.
Case report A 10-year-old boy presented to an outside emergency department with a day of severe right upper quadrant pain, headache and dizziness. During the preceding 2 months, he had intermittent episodes that were similar but less severe. He denied any shortness of breath, chest pain, fatigue, emesis, fever, weight loss or exercise intolerance. Because he had significant abdominal pain and tenderness, a CT of the abdomen and pelvis was obtained. This revealed a large pericardial effusion with concern for impending tamponade, as well as pulmonary edema (Fig. 1). He was immediately transferred to the Pediatric Intensive Care Unit. Vital signs were within normal limits. He was wellappearing and in no acute distress. Jugular venous distension was present. Pulsus paradoxus was not present. He had distant heart sounds without a friction rub. His lungs had scattered rales. His abdomen was soft but tender to palpation in the right upper quadrant. His extremities were warm, and distal perfusion was excellent. Laboratory analysis revealed that his electrolytes, complete blood count, albumin level, and coagulation studies were all within normal limits, as were his lactate dehydrogenase and uric acid levels and his erythrocyte sedimentation level. Rheumatologic and tuberculosis workups were negative. He underwent emergent pericardiocentesis, with 1.3 liters of brown-white fluid removed. Analysis of this fluid revealed 1,930 mg/dL triglycerides, 100,500/uL red blood cells, 4,133/uL white blood cells with 90% lymphocytes, and 257 U/L lactate dehydrogenase. Flow cytometry from the fluid was negative for malignant cells. Cultures from the blood and fluid were negative for bacteria, fungus or acid-fast bacilli.
Pediatr Radiol
Fig. 1 CT. Axial CT image through the lower chest of the 10-year-old boy demonstrates a large pericardial effusion (arrow) surrounding the contrast-enhanced heart
Because of subsequent reaccumulation of the pericardial effusion, the boy had surgical thoracic duct ligation with creation of a right-side pericardial window. A chest tube placed during surgery had minimal output and was removed 3 days later. An echocardiogram prior to discharge showed no recurrent pericardial effusion. The boy was then discharged home without further evidence of a pericardial or pleural effusion. Three months after discharge, however, the boy presented with mild fatigue, shortness of breath with exercise, and intermittent left-side chest pain. He was found on ultrasonography to have a moderate pericardial effusion and a large right pleural effusion. He was admitted for management of his effusions. A right-side chest tube was placed (Fig. 2), and pleural fluid studies were consistent with chylothorax. Noninvasive, conservative management was initially pursued, with a diet restrictive of free fatty acids. However, drainage from the chest tube persisted at more than a liter per day for more than a week, and his nutritional status worsened on the restrictive diet.
Fig. 2 Radiography. Anteroposterior chest radiograph in the 10-year-old boy shows the right-side pleural effusion with a pigtail drainage catheter in place. Metal clips from the prior attempt at surgical thoracic duct ligation are seen anterior to the T9 and T10 vertebral bodies (arrow)
Fig. 3 MRI. Coronal T2-weighted fat-saturated MRI through the chest of the 10-year-old boy demonstrates a large right pleural effusion occupying the entire right hemithorax. The tortuous T2-bright thoracic duct is seen along the lower midline (arrow), although the site of leakage is not apparent
Because of the lack of improvement in his clinical state, Interventional Radiology was consulted for lymphangiography and thoracic duct embolization. Initially a pre-procedure thoracic duct protocol MRI was obtained, with T2-weighted fatsaturation images demonstrating the ductal anatomy but not showing the location of the leak (Fig. 3). Lymphangiography was then performed by accessing bilateral inguinal lymph nodes with 25-gauge spinal needles, followed by the injection of approximately 6 cc of lipiodol oil on each side. The cisterna chyli was visualized in turn (Fig. 4) and successfully accessed
Fig. 4 Lymphangiography. Lymphangiographic image in the 10-yearold boy shows lipiodol oil filling abdominal lymphatic ducts and nodes, with opacification of the larger central cisterna chyli seen overlying the midline of the L1 an L2 vertebral bodies (arrow)
Pediatr Radiol
via a right upper quadrant approach using a 21-g Chiba needle (Cook Medical, Bloomington, IN), which was then exchanged over a wire for a 2.4-French microcatheter (Boston Scientific, Natick, MA). Contrast injection in the cisterna chyli and thoracic duct revealed an intact and widely patent lower thoracic duct with tortuous curves around the previously placed surgical clips at the level of the diaphragm, indicating that the duct had likely never actually been ligated during the original surgery, or less likely that a collateral channel had formed (Fig. 5). The duct continued into the upper chest, where vague lymphatic extravasation was seen bilaterally, outlining the upper mediastinal vascular structures. The thoracic duct was then successfully embolized using cyanoacrylate glue (DePuy Synthes, Raynham, MA) and numerous coils (Cook Medical, Bloomington, IN) that served as scaffolding for the glue, to prevent nontarget glue embolization into the pulmonary vasculature via the left subclavian vein (Fig. 6). Post-procedure lymphangiogram confirmed complete closure of thoracic duct.
Fig. 6 Successful coil- and glue-embolization of the thoracic duct and cisterna chyli in the 10-year-old boy. Coils extend along the entire thoracic duct, and at the level of T5 a coil extends out of a hole in the duct on the right, illustrating the site of leakage into the right chest (white arrow). The radiopaque cast of glue is seen filling the lower thoracic duct and cisterna chyli at T10–T12 (black arrow)
The remainder of the boy’s hospitalization was unremarkable, and his chest tube drainage decreased daily. His chest tube was removed 7 days after the thoracic duct embolization, when drainage had become negligible. His diet was liberalized, and with no evidence of further leakage he was discharged to home. Upon follow-up 1 month after thoracic duct embolization, he was asymptomatic, without significant physical examination findings, and his functional and nutritional status were improving. His chest radiograph was negative for an enlarged cardiomediastinal silhouette or pleural effusion. At repeat follow-up 7 months after thoracic duct embolization, the boy’s chest radiograph again showed no reaccumulation of fluid.
Discussion Fig. 5 After lymphangiography, the cisterna chyli was accessed with a microcatheter. Here, contrast agent opacifies the thoracic duct in the 10year-old boy. Note contrast extravasation into the upper chest bilaterally (white arrows), as well as the tortuous thoracic duct kinked by the overlying surgical ligation clips (black arrow). A right-side pigtail drainage catheter is in place to drain the right chylous pleural effusion
The most common presenting symptoms of chylopericardium include cough, fatigue and shortness of breath, but the presentation can be variable and include any of the following symptoms: chest pain, dyspnea on exertion, nausea, vomiting,
Pediatr Radiol Table 1 Etiologies of chylopericardium • Cardiothoracic surgery • Chest trauma • Lymphoma • Mediastinal malignancy • Mediastinal radiation therapy • Cystic hygroma • Congenital lymphatic anomalies • Thrombosis of subclavian vein • Tuberculosis
fever, syncope or sudden death; it can also be asymptomatic [ [1] ]. When concern for primary idiopathic chylopericardium is present, the initial workup should include a chest radiograph, echocardiogram, blood testing to rule out more common etiologies of chylopericardium (Table 1), and pericardiocentesis for assessment of triglycerides, cholesterol, cultures, cell counts and flow cytometry. The diagnosis of chylopericardium is characterized by a milky-yellowish appearance of the pericardial fluid, and analysis of the fluid demonstrates a cholesterol-to-triglyceride ratio of 500 mg/dL, lymphocytic cell predominance, and negative fluid culture. Conservative treatment for primary idiopathic chylopericardium includes diet modification and pericardiocentesis with or without octreotide administration. But such treatment has a reported failure rate of nearly 60% [2]. Although difficult to maintain, restricted dietary intake of free fatty acids (except medium-chain triglycerides) is a recommended intervention to decrease lymphatic output. As opposed to long- or short-chain triglycerides, medium-chain triglycerides pass directly into the portal venous circulation and thereby bypass the lymphatic drainage system. Such a restrictive diet was first reported to be efficacious in an adult patient in 1964 [3], and it was subsequently reported to be successful in a pediatric patient in 1968 [4]. Invasive treatment modalities for primary idiopathic chylopericardium offer better treatment success rates but are not without disadvantages. The first reported case of primary chylopericardium, in 1954 [5], was treated with exploratory thoracotomy, during which the lymphatic communications to the pericardial space were identified using a lipophilic dye and ligated, and a pericardial window was created into the right pleural space. Overall, surgical thoracic duct ligation is reported to be efficacious in up to 80% of intrathoracic chyle leaks, although there are complications related to the morbidities of open thoracic surgery [6]. Thoracic duct embolization, first
described by Constantine Cope [7] in 1998, offers a much less invasive and less risky means to treat thoracic chyle leaks. The procedure, which is technically successful in about 70% of cases, carries significantly less morbidity than surgery [8]. Post-procedural complications are rare, with instances of treatable access site infections and self-limited visceral organ injury reported in 2–3% of cases [8]. Long-term complications of thoracic duct embolization are also rare. In a recent case series, Chen and Itkin [8] reported long-term complications in patients who had thoracic duct embolization completed for chylopericardium or chylothorax. Technically successful embolization was achieved in 115 of 169 (68%) patients [8]. Of the 57 patients who had long-term follow-up, 7% had chronic lower extremity edema and 12% had chronic diarrhea [8]. In summary, primary idiopathic chylopericardium is an exceedingly rare diagnosis that requires thorough diagnostic workup and prompt therapeutic intervention. Lymphangiography followed by thoracic duct embolization by a skilled interventional radiologist is an excellent and under-utilized treatment modality that offers a technical success rate close to that of surgery, with significantly lower morbidity and fewer complications. This case illustrates the utility of thoracic duct embolization, in the setting of idiopathic chylopericardium treated unsuccessfully with surgical thoracic duct ligation and pericardial window creation.
Conflicts of interest None
References 1. Mask WK, Penido JR, Printup C (1990) Primary idiopathic chylopericardium. J Thorac Cardiovasc Surg 99:569–571 2. Bhat P, Ananthakrishna R, Panneerselvam A et al (2011) Recurrent chylopericardium. BMJ Case Rep. doi:10.1136/bcr.07.2011.4520 3. Hashim SA, Roholt HB, Babayan VK et al (1964) Treatment of chyluria and chylothorax with medium-chain triglyceride. N Engl J Med 270:756–761 4. Lichter I, Hill GL, Nye ER (1968) The use of medium-chain triglycerides in the treatment of chylothorax in a child. Ann Thorac Surg 5: 352–355 5. Groves LK, Effler DB (1954) Primary chylopericardium. N Engl J Med 250:520–523 6. Nath DS, Savla J, Khemani RG et al (2009) Thoracic duct ligation for persistent chylothorax after pediatric cardiothoracic surgery. Ann Thorac Surg 88:246–252 7. Cope C (1998) Diagnosis and treatment of postoperative chyle leakage via percutaneous transabdominal catheterization of the cisterna chyli: a preliminary study. J Vasc Interv Radiol 9:727–734 8. Chen E, Itkin M (2011) Thoracic duct embolization for chylous leak. Semin Intervent Radiol 28:63–74