Management of chylothorax and chylous ascites due to yellow nail syndrome with indwelling catheters: a case report
Case Report | Internal Medicine: Pulmonology

Management of chylothorax and chylous ascites due to yellow nail syndrome with indwelling catheters: a case report

Samantha Wong1, Melissa Wang2, Alim Hirji3, Pen Li3

1Department of Medicine and Dentistry, University of Alberta, Edmonton, Canada; 2Department of Medicine, Division of Pulmonary Medicine, University of Calgary, Calgary, Canada; 3Department of Medicine and Dentistry, Division of Pulmonary Medicine, University of Alberta, Edmonton, Canada

Contributions: (I) Conception and design: P Li, A Hirji; (II) Administrative support: None; (III) Provision of study materials or patients: P Li, A Hirji; (IV) Collection and assembly of data: S Wong, M Wang; (V) Data analysis and interpretation: None; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors.

Correspondence to: Pen Li, MD, FRCPC. Department of Medicine and Dentistry, University of Alberta, 3-125 Clinical Sciences Building, 11304 - 83 Ave NW, Edmonton, AB T6G 2G3, Canada. Email: pen@ualberta.ca.

Background: Yellow nail syndrome (YNS) is a rare condition characterized by yellow nails, lymphedema, and respiratory disease. Chylous ascites and chylothorax often occur in these patients and can be managed by dietary modifications, medications, and surgical procedures. Amongst the various modalities available to manage recurrent effusions due to YNS, indwelling pleural catheters (IPCs) and indwelling abdominal catheters are safe treatment options. Our case is unique because the treatment of YNS chylous ascites with an indwelling abdominal catheter has not yet been described in the literature. Our patient did not experience any complications of infection, malnutrition, or electrolyte disturbances.

Case Description: We describe the case of a 77-year-old female with YNS who developed multiple chylous effusions over the course of several years. She was initially assessed for management of her left chylous pleural effusion after developing dyspnea on exertion, worsening orthopnea, and cough. At this time, she had an established diagnosis of YNS. After multiple thoracentesis, the left pleural effusion was managed with an IPC which was subsequently removed after pleurodesis was achieved. Approximately a year later she developed a recurrent right-sided pleural effusion which was definitively managed with a video-assisted thoracoscopy with talc poudrage. Approximately 3 years later she was referred to a respirologist who made changes to her lymphedema management which included dietary modifications, intramuscular octreotide, and furosemide. Despite these treatments, she had persisting leg lymphedema and was treated with leg compression dressings for her leg lymphedema, which resulted in the development of chylous ascites. Due to the need for recurrent paracentesis, the decision was made to insert an indwelling abdominal catheter. She did not experience any complications from the catheter and it was eventually removed due to a lack of ascites.

Conclusions: This case suggests that indwelling catheters are a safe and effective approach to managing effusions and ascites in patients with YNS.

Keywords: Yellow nail syndrome (YNS); pleural effusion; chylous ascites; indwelling catheter; case report

Received: 03 November 2023; Accepted: 08 July 2024; Published online: 07 August 2024.

doi: 10.21037/amj-23-212

Highlight box

Key findings

• Indwelling catheters can be used to manage recurrent effusions due to yellow nail syndrome (YNS).

What is known and what is new?

• Dietary modifications, medications, and surgery have previously been used to manage chylous ascites and chylothorax due to YNS.

• We present a case of the safe use of indwelling catheters, a novel approach, for management of YNS associated effusions.

What is the implication, and what should change now?

• Indwelling catheters are a management options of YNS effusions (pleural or ascitic) in patients who fail conservative management and cannot tolerate surgical interventions. Further studies are required to validate the safety in a diverse population.

Introduction

Background

Yellow nail syndrome (YNS) is a rare condition diagnosed when 2 or more of the following are present: (I) deformed, hard, slow-growing yellow nails; (II) lymphedema; and (III) pulmonary disease which can manifest as pleural effusions, bronchiectasis, and bronchitis (1). The underlying pathophysiology of YNS is unknown but is likely due to lymphatic dysfunction and microvasculopathy (2,3). The recurring and persistent manifestations of YNS can lead to a significant decline in functional status and potentially life-threatening complications.

The management options for chylous effusions (chylothorax and chylous ascites) in patients with YNS are the same as those with other non-malignant causes of chylothorax, including dietary changes, medication, and various procedures. Dietary changes that have been successful include a low fat diet with medium chain triglycerides (MCT) as they are absorbed into the portal venous system as opposed to the intestinal lymphatic system, thus decreasing the production of chyle, and thus chylous effusions (4,5). Octreotide—a somatostatin analogue that inhibits the absorption of fats and other nutrients, which subsequently leads to a reduction in the triglyceride concentration in the thoracic duct and thus diminishes lymphatic flow—has also successfully been used for management of chylous effusions in patients with YNS (6,7).

Procedures that have been used for symptomatic management of chylous effusions include thoracentesis and paracentesis. More definitive procedures include chemical pleurodesis and pleurectomy (8). Various surgical shunts have also been used for the management of these effusions, including pleuroperitoneal (9), pleurovenous (10), and peritoneovenous (11), but they run the risk of surgical complications, infection, and blockage of the shunt.

Rationale and knowledge gap

Currently, reoccurring pleural effusions and ascites due to YNS are managed with dietary modifications, medications, and surgery. However, treatment options for patients who are not surgical candidates or for those who have failed conservative treatment modalities have been rarely explored.

Objective

We present a case of a patient with YNS who had resultant pleural effusions and chylous ascites that were successfully and safely managed with indwelling catheters. We present this article in accordance with the CARE reporting checklist (available at https://amj.amegroups.com/article/view/10.21037/amj-23-212/rc).

Case description

A 77-year-old female with YNS and obesity was initially referred for assessment of a recurrent left-sided pleural effusion in August 2016. She presented with dyspnea on exertion, worsening orthopnea, and a productive cough. To manage her left-sided pleural effusion, the patient underwent 3 thoracenteses in August 2016, December 2016, and February 2017 with over 1 L of fluid being drained each time. The initial fluid analysis was in keeping with a lymphocytic, exudative effusion with no evidence of malignancy or infection. She had a left-sided indwelling pleural catheter (IPC) inserted in February 2017 for management of this effusion, which was eventually removed in November 2017 due to autopleurodesis. The IPC was draining approximately 700 mL three times each week until pleurodesis occurred.

In July 2018, she developed a recurrent right-sided pleural effusion for which she also underwent 2 thoracenteses (1.2 and 1.3 L drained) in a 3-month period. The effusion continued to be a lymphocyte predominate effusion but did not meet diagnostic criteria for a chylothorax with a triglyceride value of 0.21 mmol/L. She was referred to thoracic surgery in October 2018 who conducted a video assisted thoracoscopy with talc poudrage. This was successful and she had no significant reaccumulation of fluid.

In December 2021, she was referred to a new respirologist who started her on octreotide 30 mg intramuscular every 28 days which led to initial improvements in her leg lymphedema. However, this was stopped 8 months later as she felt it was no longer beneficial and was causing worsening nausea. A dietician was consulted in September 2022 who started her on a low fat, MCT diet. She had also been on varying doses of oral furosemide—ranging from 20 to 80 mg twice daily. Throughout the management of her ascites and lymphedema, the patient continued to take furosemide 60 mg twice daily. Furthermore, she was seen by the lymphedema clinic in June 2022 who started compressive leg dressings every 3 days which resulted in worsening accumulation of ascites as well as bilateral fissural pleural effusions (Figure 1). On September 16, 2022, she underwent her first paracentesis (2.2 L) which confirmed chylous ascites with a triglyceride value of 15.4 mmol/L. Symptomatically she noted improvement with drainage of her ascites. She underwent her second paracentesis (2.4 L) on September 29, 2022 and a third paracentesis (2.4 L) on October 12, 2022. Given the patient’s decreased mobility and rapidly reoccurring ascites, a decision was made to insert an indwelling abdominal catheter in November 2022. She underwent drainage through her abdominal catheter three times a week with symptomatic improvement and stability of her serum electrolytes and albumin.

Figure 1 Loculated fissural pleural effusions.

Interestingly, a couple weeks before the insertion of her abdominal catheter, she developed a weeping leg wound that required a wound vacuum assisted closure (VAC). She noted improvement in her ascites when she had her leg wound and worsening once the wound had healed.

Initially she had approximately 200 mL of ascites output from her catheter per drainage session. Unfortunately, she developed further leg wounds from which fluid would drain and this seemed to decrease her ascites. Because she could not receive compressive leg dressings when she had leg wound she eventually developed severe lymphedema to her legs that impaired her mobility, and necessitated being admitted to the hospital for care and to expedite consultations with surgical specialties. She was seen by plastic and vascular surgery who were not able to provide any surgical interventions to help manage her lymphedema.

After returning home she developed another leg wound which drained a significant volume of fluid and improved her leg lymphedema. She was seen by a palliative care specialist with an interest in lower extremity edema. As a part of her care with this specialist, she has received multiple treatments where four 19-gauge metal butterfly needles were inserted into her legs and attached to drainage bags to allow for controlled subcutaneous drainage of her lymphedema and improve her quality of life.

Due to a lack of fluid, her indwelling abdominal catheter was removed in July 2023 (Figure 2). Throughout the duration of her abdominal catheter her albumin, creatinine, sodium, and lymphocytes were relatively stable (Table 1). She did not develop any infections related to the catheter, such as peritonitis or cellulitis and she did not require and further paracentesis since the removal of the indwelling catheter. She was also on furosemide 60 mg twice daily.

Figure 2 Timeline of the patient’s chylous effusion management. YNS, yellow nail syndrome; VATS, video-assisted thoracoscopic surgery; IM, intramuscular.

Table 1

Laboratory values pre-catheter insertion and pre-removal

Laboratory investigation Indwelling pleural catheter Indwelling abdominal catheter
Pre-insertion Pre-removal Pre-insertion Pre-removal
Albumin (g/L) 40 38 23 19
Creatinine (μmol/L) 72 70 62 76
Sodium (mmol/L) 144 142 143 144
White blood cells (×109/L) 6.8 7.2 5.0 4.4
Lymphocytes (×109/L) 1.1 0.6 0.8 0.5

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Verbal informed consent was obtained from the patient. Written informed consent for publication of this case report and accompanying images was not obtained from the patient or the relatives after all possible attempts were made.

Discussion

According to a review of 41 patients with YNS, there is a 46% incidence of pleural effusions, 31% of which are chylous (3). The incidence of ascites in patients with YNS has not been reported, but one systematic review reports a 7% incidence of ascites in patients with both YNS and pleural effusions (12).

Although there are multiple management options, both conservative and surgical, available for recurrent effusions, indwelling catheters are a novel and appealing option for patients who are not surgical candidates or those who have failed other conservative management options. Historically the use of indwelling catheters has been avoided due to fear of malnutrition, hemodynamic instability, and immunosuppression from chyle loss (13). However, in a case review of 11 patients with non-malignant chylothorax managed with IPCs, they found that patients had no significant nutritional, hemodynamic, or immunologic deficiencies after insertion of their IPC (14). There have only been a few case reports of the use of IPCs for the management of chylous effusions in patients with YNS, and the majority are used in combination with chemical pleurodesis (8,15,16).

Regarding the use of indwelling abdominal catheters, current data are from populations with malignant ascites, related to liver disease, and a few from heart failure. The use of indwelling abdominal catheters for the use of malignant effusions is more accepted, with recent studies reviewing its safety in non-malignant disease. One systematic review that examined indwelling abdominal catheters in both malignant and non-malignant ascites found the risk of infection to be lower in patients with malignancy (5.4%) versus non-malignant disease (12.2%), postulated to be related to the longer duration of the catheter in the non-malignant group, as well as the inherent risk of developing peritonitis in patients with end stage liver disease and ascites (17). A more recent systematic review of non-malignant ascites found an average infection rate of 11%, transient hyponatremia in 11%, and a transient rise in creatinine in 8% (18). There are no case reports of the use of indwelling abdominal catheters for patients with chylous ascites due to YNS.

Our patient experienced both pleural effusions and chylothorax related to her YNS. Her pleural effusions ultimately resolved due to pleurodesis of the left cavity from an IPC, and talc pleurodesis of the right pleural cavity. She had no issues with pleural space infection nor malnutrition with her IPC. Her bilateral pleurodesis is what likely allowed for subsequent development of chylous ascites as her disease progressed. Unfortunately, conservative management with a low-fat and high MCT diet, octreotide, and furosemide were not significantly beneficial for her ascites. The indwelling abdominal catheter allowed our patient symptomatic relief and the ability to be managed at home, which was of great value to her given her poor mobility. She did not experience any complications from her abdominal catheter, specifically no malnutrition, immunosuppression, nor renal or electrolyte disturbances.

The limitations of our case report are that by its nature, it involves only one patient who had chylous effusions successfully managed with indwelling catheters without complications. Additional reports of similar cases or trials would be helpful to verify the safety and efficacy of indwelling catheters as a treatment option.

Conclusions

Our case demonstrates a minimally invasive method to managing recurrent pleural effusions and chylous ascites in the setting of YNS without significant complications. IPCs provide an attractive management option for pleural effusions, particularly if a patient is not a candidate for pleurodesis. In the setting of chylous ascites where there is no procedure analogous to pleurodesis, indwelling abdominal catheters can provide symptomatic relief without necessitating frequent hospital visits.

Acknowledgments

Funding: None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://amj.amegroups.com/article/view/10.21037/amj-23-212/rc

Peer Review File: Available at https://amj.amegroups.com/article/view/10.21037/amj-23-212/prf

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://amj.amegroups.com/article/view/10.21037/amj-23-212/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Verbal informed consent was obtained from the patient. Written informed consent for publication of this case report and accompanying images was not obtained from the patient or the relatives after all possible attempts were made.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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doi: 10.21037/amj-23-212
Cite this article as: Wong S, Wang M, Hirji A, Li P. Management of chylothorax and chylous ascites due to yellow nail syndrome with indwelling catheters: a case report. AME Med J 2025;10:18.

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