Chylopericardium and Plastic Bronchitis From Thoracic Duct Dysfunction

Abstract

Background

Chylopericardium is a rare lymphatic disorder and an uncommon cause of large pericardial effusion, often associated with diagnostic uncertainty and potential for recurrent, noncurative interventions when the underlying thoracic duct dysfunction is not recognized.

Case Summary

A 17-year-old woman with prior complex thoracic interventions presented with obstructive shock and large pericardial effusion. Transthoracic echocardiography demonstrated swinging heart with tamponade physiology, and emergent pericardiocentesis yielded chylous fluid confirming chylopericardium. Recurrent hemodynamic compromise required surgical pericardial window. Progressive respiratory failure prompted flexible bronchoscopy, revealing bronchial chylous casts consistent with plastic bronchitis. Dynamic contrast lymphangiography identified bilateral apical lymphatic ectasia without a discrete leak. Percutaneous thoracic duct embolization achieved complete clinical resolution.

Discussion

This case underscores thoracic duct dysfunction as a unifying mechanism and supports early lymphatic evaluation and thoracic duct embolization as definitive therapy in complex chylous disease.

Visual Summary

History of Presentation

A 17-year-old Hispanic woman was admitted following a generalized tonic–clonic seizure associated with nausea and vomiting. On presentation, she was hypotensive (90/60 mm Hg) and tachycardic (113 beats/min). Neuroimaging showed no acute intracranial abnormalities. Physical examination revealed jugular venous distension and globally muffled heart sounds, without chest pain or pericardial rub. Laboratory evaluation showed leukocytosis with neutrophilic predominance and mildly elevated inflammatory markers. Twelve-lead electrocardiogram showed sinus tachycardia with low-voltage QRS complexes, and chest radiography revealed marked cardiomegaly, collectively raising strong suspicion for a large pericardial effusion.

Take-Home Messages

• •Concurrent chylopericardium and plastic bronchitis should prompt early suspicion of thoracic duct dysfunction, particularly in patients with prior thoracic interventions.
• •Recurrent chylous effusions despite drainage should trigger lymphatic evaluation, as thoracic duct embolization can provide definitive treatment.

Past Medical History

The patient had a history of epilepsy with poor adherence to antiepileptic therapy. Two years earlier, she underwent emergency laparotomy for bowel perforation secondary to superior mesenteric artery syndrome. That hospitalization was complicated by hemothorax following central venous catheter placement, ventilator-associated pneumonia, and pleural effusion requiring video-assisted thoracoscopic surgery with decortication and temporary tracheostomy.

Investigations

Transthoracic echocardiography demonstrated a large circumferential pericardial effusion with a swinging heart (Figure 1). Although echocardiographic findings were suggestive of tamponade physiology, the absence of unequivocal acute chamber collapse was consistent with a chronic, hemodynamically adapted process. Chest computed tomography demonstrated no mediastinal masses, lymphadenopathy, or structural lesions that could account for the effusion, raising suspicion of thoracic duct dysfunction.

Figure 1.

Large Pericardial Effusion on Transthoracic Echocardiography

Transthoracic echocardiography demonstrating a large circumferential pericardial effusion with a “swinging heart” appearance. These findings are consistent with tamponade physiology in the setting of a large-volume effusion with partial hemodynamic compensation.

Given the large symptomatic effusion with hemodynamic compromise, urgent ultrasound-guided pericardiocentesis was performed per current guideline recommendations, yielding approximately 400 mL of milky pericardial fluid (Figure 2). Fluid analysis revealed lymphocytic predominance (88%) with triglyceride concentration of 1,354 mg/dL (normal: 35-150 mg/dL) and cholesterol level of 84 mg/dL (normal: 130-200 mg/dL), producing a cholesterol:triglyceride ratio of 0.06 (normal ratio ≥1), consistent with diagnostic criteria for chylopericardium.¹ Microbiological cultures for bacteria, fungi, and mycobacteria were negative. Antinuclear antibodies were positive with a nucleolar pattern (AC-8/9/10) at a titer of 1:640, p24 antigen, hepatitis B surface antigen, hepatitis B and C antibodies, and QuantiFERON-TB were all negative, effectively excluding active infectious etiologies including tuberculosis and viral hepatitis.

Figure 2.

Chylous Pericardial Fluid

Gross appearance of milky, opalescent pericardial fluid obtained during ultrasound-guided pericardiocentesis. The characteristic chylous appearance supports the diagnosis of chylopericardium, later confirmed by biochemical analysis.

Differential Diagnosis

In the setting of a large pericardial effusion with hemodynamic compromise, the primary considerations included infectious (particularly tuberculosis), malignant, and autoimmune etiologies. However, the milky appearance of the fluid and biochemical profile rapidly shifted the diagnostic focus toward an underlying lymphatic disorder, particularly thoracic duct dysfunction.

Management (Medical/Interventions)

Two days after admission, the patient deteriorated abruptly with fever, acute respiratory failure, and hemodynamic instability attributable to obstruction of the pericardial drain. Surgical pericardial window was urgently performed, yielding an additional 300 mL of chylous fluid, and pericardial biopsy demonstrated nonspecific chronic inflammatory changes with no evidence of malignancy, granulomatous disease, or specific infection.

Subsequent imaging revealed right lower lobe atelectasis and a new ipsilateral pleural effusion. Ultrasound-guided thoracentesis drained 750 mL of exudative, nonchylous fluid. Despite these interventions, persistent hemodynamic and respiratory compromise refractory to drainage, volume resuscitation, and vasopressor support, prompted transfer to the intensive care unit for advanced monitoring and invasive evaluation.

In this setting, flexible bronchoscopy revealed chylous secretions coating the tracheobronchial mucosa with cast formation, confirming lymphatic plastic bronchitis (Figure 3). Serial bronchoscopies were performed for therapeutic cast removal and monitoring.

Figure 3.

Bronchial Casts on Flexible Bronchoscopy

Flexible bronchoscopy showing chylous bronchial casts coating the tracheobronchial tree. These findings are diagnostic of lymphatic plastic bronchitis and explain the patient's respiratory compromise. Bronchoscopy also enabled therapeutic cast removal.

Given these findings, lymphangiography was pursued and demonstrated bilateral apical lymphatic ectasia without a discrete thoracic duct rupture. Thoracic duct embolization using ethiodized oil and maceration was performed successfully under fluoroscopic guidance (Figure 4).

Visual Summary.

Thoracic Duct Dysfunction Results in Lymphatic Leakage Into the Pericardial Space (Chylopericardium) and Airways (Plastic Bronchitis), Representing A Unifying Mechanism

Illustration adapted and created by the authors.

Figure 4.

Lymphangiography Showing Lymphatic Ectasia

Dynamic contrast lymphangiography with thoracic duct cannulation demonstrating bilateral apical lymphatic ectasia without a discrete site of duct disruption. The diffuse lymphatic abnormality supported thoracic duct dysfunction and guided successful embolization

Outcome and Follow-Up

Following the procedure, the patient experienced rapid hemodynamic stabilization with discontinuation of vasopressor support. Follow-up bronchoscopy confirmed resolution of airway chylous leakage and disappearance of bronchial casts. No recurrence of pericardial effusion, pleural effusion, or plastic bronchitis was observed during follow-up. The chronological sequence of diagnostic and therapeutic interventions is summarized in Table 1. The equipment used for the multimodality diagnostic evaluation and thoracic duct embolization is summarized in Table 2.

Table 1.

Timeline of the Case

Timeline	Events
Day 1	17-y-old woman presented with generalized tonic–clonic seizure, hypotension, and tachycardia. Physical exam revealed jugular venous distension and muffled heart sounds. Electrocardiogram showed low-voltage QRS; chest radiography demonstrated marked cardiomegaly.
Day 1	Transthoracic echocardiography revealed large circumferential pericardial effusion with swinging heart. Urgent pericardiocentesis yielded chylous fluid → diagnosis of chylopericardium.
Day 2	Clinical deterioration with hemodynamic instability due to drain obstruction. Surgical pericardial window performed; persistent chylous effusion.
Day 4	Development of respiratory failure with pleural effusion and atelectasis. Thoracentesis performed. Persistent instability prompted intensive care unit transfer.
Day 4	Flexible bronchoscopy revealed chylous bronchial casts → diagnosis of lymphatic plastic bronchitis. Serial bronchoscopies for cast removal.
Day 6	Lymphangiography demonstrated bilateral apical lymphatic ectasia without discrete leak. Thoracic duct embolization performed successfully under fluoroscopic guidance.
Day 12	Rapid hemodynamic and respiratory recovery. Resolution of chylopericardium and plastic bronchitis with no recurrence on follow-up.

Table 2.

Equipment List for Multimodality Diagnosis and Thoracic Duct Embolization

A. Pericardiocentesis

Ultrasound system with phased-array transducer

Pericardiocentesis kit (needle, guidewire, dilator, drainage catheter)

Sterile field and local anesthesia (lidocaine)

Pericardial drainage system with collection bag

B. Bronchoscopy

Flexible bronchoscope (adult diagnostic scope)

Suction system

Retrieval instruments (forceps, suction catheter)

Saline for airway lavage

C. Lymphangiography

Ultrasound system for inguinal lymph node access

25-27G needle for lymph node cannulation

Ethiodized oil contrast agent (Lipiodol)

N-butyl cyanoacrylate (NBCA) (optional)

Fluoroscopy suite

Microcatheter and guidewire system

D. Thoracic Duct Embolization

Microcatheter system for thoracic duct cannulation

Embolic agents:

Ethiodized oil (Lipiodol)

N-butyl cyanoacrylate (NBCA) (optional)

Fluoroscopic guidance system

Contrast media

Mechanical disruption (maceration technique)

Discussion

Thoracic duct dysfunction may manifest as a spectrum of lymphatic disorders. The coexistence of chylopericardium and plastic bronchitis in this patient reflects a unifying pathophysiological mechanism linking pericardial and airway involvement rather than 2 independent entities, particularly in the setting of previous thoracic interventions predisposing to multifocal lymphatic decompensation.²

Chylopericardium is an uncommon cause of pericardial effusion due to abnormal lymphatic drainage into the pericardial space. It is most frequently associated with thoracic surgery, trauma, malignancy, or congenital lymphatic abnormalities.² In this case, previous thoracic interventions likely contributed to thoracic duct dysfunction, consistent with secondary chylopericardium. Unlike inflammatory or malignant effusions, chylous collections typically accumulate gradually, allowing progressive pericardial distensibility and partial hemodynamic compensation despite large volumes. This explains the absence of overt chamber collapse on echocardiography despite a massive effusion, reflecting a chronic adaptive process with delayed decompensation.

Plastic bronchitis is a rare but life-threatening manifestation of lymphatic failure, characterized by the formation of obstructive bronchial casts due to chylous leakage into the airways. Flexible bronchoscopy plays a dual diagnostic and therapeutic role, allowing direct visualization and removal of casts while confirming lymphatic airway involvement. Early recognition is critical, as conventional respiratory therapies are ineffective and delay in diagnosis may result in progressive respiratory failure.^3,4

A critical learning point from this case is that recurrent pericardial effusion despite drainage should not be managed with repeated decompressive strategies alone. Instead, it should prompt early evaluation for an underlying lymphatic disorder. Failure to recognize this mechanism may lead to repeated invasive but noncurative interventions, as illustrated by the need for both pericardiocentesis and surgical pericardial window before establishing the definitive diagnosis. In contrast, the transition to a lymphatic-focused approach—guided by bronchoscopy and dynamic contrast lymphangiography—represented the key turning point that allowed definitive management.⁵

Pericardial fluid analysis remains essential for diagnosis, with elevated triglyceride levels, low cholesterol:triglyceride ratio, and lymphocytic predominance reliably confirming chylopericardium while excluding infectious and malignant etiologies.⁶ However, identification of the underlying lymphatic abnormality requires advanced imaging. In this patient, lymphangiography demonstrated diffuse lymphatic ectasia without a discrete site of duct disruption, supporting the concept of functional thoracic duct failure rather than focal injury.⁷

According to current international guidelines¹ and recent advances in multimodality imaging,⁷ large symptomatic pericardial effusions warrant prompt drainage for both diagnostic clarification and hemodynamic relief. However, recurrence following drainage or clinical deterioration despite adequate decompression should be interpreted as a signal to investigate alternative mechanisms beyond conventional etiologies. Surgical pericardial window may provide temporary relief but does not address the underlying lymphatic pathology and therefore does not provide a durable solution.²

Thoracic duct embolization has emerged as an effective minimally invasive therapy for lymphatic disorders, with reported technical success rates ranging from 70% to 90%.⁸ Importantly, it offers the unique advantage of addressing multiple manifestations of lymphatic dysfunction simultaneously, as demonstrated by the complete resolution of both chylopericardium and plastic bronchitis in this case. This underscores its role as definitive therapy and supports consideration of early referral for lymphatic intervention in refractory or complex presentations.

Conclusions

This case establishes thoracic duct dysfunction as a unifying mechanism underlying concurrent chylopericardium and plastic bronchitis. Their coexistence should prompt early suspicion of lymphatic pathology, particularly in patients with prior thoracic interventions. Biochemical analysis of pericardial fluid remains essential for diagnosis, whereas flexible bronchoscopy is critical for identifying and managing airway involvement. Thoracic duct embolization offers an effective, minimally invasive definitive therapy. This case reinforces the importance of a multimodality approach for timely diagnosis and targeted treatment in complex lymphatic disorders.

Funding Support and Author Disclosures

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.