Transudative chylothorax and frailty: a diagnostic and therapeutic challenge
- Liz Robinson ,
- Sze Ooi and
- Ben Prudon
- Respiratory Medicine, University Hospital of North Tees, Stockton-on-Tees, UK
- Correspondence to Dr Liz Robinson; liz.robinson12@nhs.net
Abstract
Transudative chylothoraces are a rare entity and their management in the presence of multiorgan dysfunction and frailty is complex. A woman in her 90s was investigated during acute hospital admission and found unexpectedly to have a transudative chylothorax secondary to cryptogenic cirrhosis. Not all chylothoraces have the classically described milky appearances and a high index of suspicion is vital in determining appropriate investigation and management. Our patient required repeated thoracocentesis and subsequently chose to be discharged from hospital with comfort care. Management of non-malignant pleural effusions can be challenging. Case reports surrounding the management of transudative chylothoraces in particular are scarce. Establishing patient priorities and openly explaining the uncertainty regarding prognosis and potential therapeutic options is paramount in this complex and changing field.
Background
Patients with unilateral pleural effusions are frequently admitted to the acute medical take and their presentation is often in the context of multimorbidity and frailty. Patients with non-malignant effusions secondary to organ dysfunction have an estimated 1-year mortality of 25-50%.1 Determining the best approach to their management relies heavily on ascertaining the underlying diagnosis and understanding the pathophysiological processes leading to fluid accumulation.
We report the case of an elderly patient presenting with a unilateral effusion that was unexpectedly found to be a transudative chylothorax. Although only around 14% of chylothoraces2 are estimated to be transudative, they are associated with significant morbidity.
Evidence surrounding the best approach to management of transudative effusions, especially when associated with recurrent or refractory disease, is emerging. Historically, indwelling pleural catheters (IPCs) have been used in the management of patients with non-malignant effusions but a recent randomised controlled trial has demonstrated that their effect on symptoms is not significantly superior to repeated aspiration and is associated with an increased incidence of adverse events3. We present this case with the hope of raising awareness of the complexities of the diagnosis and management of unilateral pleural effusions, and transudative chylothorax in particular, where comorbidities are often significant and case reports are scarce.
Case presentation
A woman in her 90s was admitted to hospital after presenting with sudden-onset breathlessness, worsening chronic cough, leg swelling and a 2-year history of significant weight loss. Her medical history included cryptogenic cirrhosis, aortic stenosis and bronchiectasis. She was a non-smoker with insignificant alcohol intake, with no recent surgery or trauma.
On presentation, she had a respiratory rate of 24 breaths/minute with oxygen saturations of 96% on air. She was haemodynamically stable and afebrile. The physical examination was consistent with a right-sided pleural effusion.
Investigations
A chest X-ray confirmed a right-sided pleural effusion with no significant parenchymal abnormality (figure 1). Inflammatory markers were normal but cardiac and liver enzymes were raised. Bedside ultrasound of the thorax revealed a right-sided non-septated pleural effusion with some echogenicity. One litre of straw-coloured fluid was aspirated, and analysis was consistent with a transudative chylothorax (pH 7.53, cholesterol 0.6 mmol/L, lactate dehydrogenase 130 U/L, triglyceride 2.11 mmol/L, paired serum triglyceride 0.97 mmol/L, protein 13 g/L).
Chest X-ray. Anterior-posterior view.
There was no growth on extended culture of the aspirated fluid. Echocardiography confirmed moderate to severe aortic stenosis with biatrial dilatation and dilated aortic root. Systolic function was normal and ejection fraction was preserved at >55%. CT scan of neck, thorax, abdomen and pelvis revealed a non-occlusive portal vein thrombosis, liver appearances consistent with cirrhosis and mesenteric oedema and ascites in addition to the pleural effusion (figure 2). No evidence of malignancy was demonstrated on CT scan and cytological analysis of the pleural fluid was also negative for malignancy.
CT scan shoring pleural effusion.
Differential diagnosis
The unilateral effusion in this patient with aortic stenosis was initially felt secondary to presumed congestive cardiac failure. Once biochemical analysis and echocardiographic evaluation of heart function had been obtained, the transudative chylothorax was then considered in relation to cryptogenic liver cirrhosis.
Treatment
Unfortunately, our patient experienced rapid reaccumulation of fluid causing significant symptoms and requiring two further therapeutic aspirations over a 2-week period. Supplemental oxygen was administered intermittently throughout admission to maintain saturations. The decision was made that transjugular intrahepatic portosystemic shunt was not in our patient’s best interests due to frailty and the extent of her comorbidities. She responded to diuretics and was commenced on a very low-fat diet with one tablespoon of sunflower oil daily to provide essential fatty acids.
Outcome and follow-up
Our patient did unfortunately test positive for COVID-19 later during her stay in the hospital but remained clinically stable, without respiratory consequence. She was discharged with plans to be reviewed in pleural clinic for discussion surrounding IPC insertion. Her condition initially deteriorated shortly after discharge, and IPC insertion was not felt by the respiratory team, the patient or her family to be in her best interests at that point. She remained at home, however, and subsequently, stabilised with improvement in symptoms. Several months later, we are delighted to hear she is doing well and enjoying time with family at home without need for repeated aspiration.
Discussion
Chylothorax is a rare cause of pleural effusion. Diagnosis is made following identification of chylomicrons within the pleural space. Raised triglyceride levels >1.24 mmol/L (110 mg/dL) and low cholesterol <5.18 mmol/L (200 mg/dL) confirm the diagnosis. Only 50% of chylothoraces have the classically associated milky-white appearance so full pleural fluid analysis is imperative.4
Complications associated with chylothoraces can be significant; it is reported that around fifty percent of untreated patients die as a result of such complications.5 These complications can include:
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Malnutrition due to loss of proteins, fats and vitamins.
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Electrolyte abnormalities.
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Immunocompromise secondary to immunoglobulin and T lymphocyte loss.
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Coagulopathies.
Transudative chylothoraces are rare. Maldonado et al, in their retrospective study of 74 patients, report that 14% of included chylothoraces were transudative.2 They have been described in association with cirrhosis, nephrotic syndrome, mantle radiotherapy for Hodgkins’s disease, cardiac failure and constrictive pericarditis.4
The pathophysiological mechanism relating specifically to chylothorax in cirrhosis can be linked to transdiaphragmatic migration of chylous ascites6 , relating to raised portal venous pressures, increased flow in the thoracic duct, and extravasation of chyle into the abdominal cavity.
Recommended management of transudative chylothorax in the context of cirrhosis is multifaceted in approach and guided by severity of symptoms, respiratory compromise, recurrence rate and patient suitability for intervention if conservative measures fail.
Modification of a patient’s diet to provide medium chain triglycerides, absorbed directly into the portal venous system, can be used to reduce chyle production and flow.5 Somatostatin analogues, prescribed with the aim of reducing portal pressure, can also reduce thoracic duct flow and the triglyceride content of chyle.7 8
Salt restriction and diuretics, directed at reducing fluid retention and transcapillary gradient in the splanchnic circulation, are recommended in cirrhotic liver disease.9 Beta blockers, shown to be effective as a treatment for ascites secondary to portal hypertension,10 have also been reported in treating transudative chylothorax secondary to alcohol-related cirrhosis and hepatocellular carcinoma with successful reduction in pleural and ascitic fluid volume.11 12 Their use in conjunction with diuretics may be considered in refractory chylothorax.
More definitive approaches to reducing portal pressure and thus lymphatic flow are recommended in suitable patients. The use of transjugular intrahepatic portosystemic shunts has been identified as an effective treatment for refractory chylothorax secondary to cirrhosis and has also been reported to be effective in patients with minimal ascites.13 14
Thoracic duct embolisation, first described in relation to the management of traumatic chylothoraces,15 has been reported to be a minimally invasive option for treating non-traumatic chylothorax.16 Nadolski and Itkin, in their case series of 34 patients, report greater success with this procedure where distal thoracic duct occlusion or leakage was identified (75% compared with 16%). Their case series did not include any patients with chylothorax secondary to cirrhosis.16
Where pleural fluid volume causes significant respiratory compromise or accumulates rapidly, repeated thoracocentesis can be used for immediate relief, but chylothorax frequently recurs. Single-centre reports have described the use of IPCs for effective symptom control with transudative effusions associated with congestive cardiac failure and hepatic hydrothorax but only one patient with chylothorax was reported on in these studies.17 18 A recent randomised controlled trial comparing IPC use to transthoracic thoracentesis in the context of transudative effusions showed no additional symptomatic benefit in the IPC group but did identify an increased incidence of complications.3 Mukherjee et al describe a case report of successful pleurodesis with iodopovidone in the context of cirrhosis related transudative chylothorax.19
The evidence base for optimal management of chylothorax, and transudative pleural effusions, is ever expanding. With this in mind, shared decision-making with individual patients with transudative chylothorax must be paramount. A patient-centred approach will allow for careful evaluation of underlying pathophysiology, symptomatic burden, fluid reaccumulation rate, patient suitability for intervention, individual procedural risk and above all, a patient’s priorities for quality of life. We look forward to future developments in the understanding of optimal pleural fluid management in the context of transudative effusions, especially when associated with frailty, liver disease and chylothorax.
Learning points
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When investigating unilateral pleural effusions, keeping an open mind allows the physician to identify rare or multifactorial pathological processes.
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Aspiration and full pleural fluid analysis should be considered when the cause of a pleural effusion is not consistent with presumed existing diagnosis.
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As always, in managing frail patients with multiple comorbidities, establishing their priorities for well-being should be paramount, while current evidence is explained clearly and helpfully.
Ethics statements
Patient consent for publication
Footnotes
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Contributors LR: lead author in all aspects excluding the case presentation and consent process. SO: coauthored the case presentation and was involved in consent process. BP: reviewed the article.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.
References
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