A Rare Case of Contarini’s Syndrome: A Diagnostic Dilemma

Rahul Gupta¹, Sukhjinder P Singh², Arjun Kumar³ https://orcid.org/0000-0002-5581-6176

^1–3Department of Respiratory Medicine, MM Medical College & Hospital, Kumarhatti-Solan (HP), Solan, Himachal Pradesh, India

Corresponding Author: Rahul Gupta, Department of Respiratory Medicine, MM Medical College & Hospital, Kumarhatti-Solan (HP), Solan, Himachal Pradesh, India, Phone: +91 7000465120, e-mail: rahulgupta240@gmail.com

Received: 07 October 2023; Accepted: 30 April 2024; Published on: 18 June 2024

ABSTRACT

Presence of pleural effusions bilaterally are frequently experienced in clinical practice. Most of the pleural effusions having bilateral involvement have similar causes. The occurrence of bilateral pleural effusion with various causes for each side, also known as Contarini’s syndrome, is rare. Here, we present the interesting case of Contarini’s syndrome. Herein, we present the exceptional case of Contarini’s syndrome, in a 53-year-old female of carcinoma breast, who developed bilateral pleural effusion of different etiology on both sides. Finally, we conclude that Contarini’s syndrome is an uncommon entity. Pleural fluid aspiration from each side for bilateral effusion should be performed to investigate the etiology of bilateral pleural effusion in cases of uncertainty regarding alternate diagnosis.

How to cite this article: Gupta R, Singh SP, Kumar A. A Rare Case of Contarini’s Syndrome: A Diagnostic Dilemma. Indian J Respir Care 2024;13(2):121–123.

Source of support: Nil

Conflict of interest: None

Keywords: Carcinoma breast, Case report, Contarini’s syndrome, Pleural effusion

INTRODUCTION

Bilateral effusion in pleura is frequently observed in daily clinical practice. A diagnosis is usually made on chest X-ray and ultrasound. Most frequent etiology of transudative bilateral pleural effusion includes cardiac, renal, and liver failure. Bilateral exudative pleural effusions are also manifested by malignant disease, tuberculosis, and pneumonia.¹ Usually, these bilateral effusions have a single etiology. So diagnostic pleural fluid tapping is done from higher effusion side. Rarely, pleural fluid aspiration from both sides is recommended. Very rarely, we come across a case of bilateral pleural effusion of separate causes on each side. Presence of bilateral pleural effusion with various causes for each side is known as Contarini’s syndrome.² Contarini’s syndrome was coined by Kutty and Varkey in 1978 after Francesco Contarini.² He was a Doge of Venice, who died in 1624 after a prolonged illness. The previous study on heart failure patients showed right-sided transudative pleural effusion and pyothorax on the left side.³^,⁴ Hence, it is evident that bilateral pleural effusions can be harboring different etiologies for each side. There are few case reports of Contarini’s syndrome in literature. Here, we report the rare case of Contarini’s syndrome, in a 53-year-old female of carcinoma breast, who developed bilateral pleural effusion of different etiology on both sides.

CASE HISTORY

A 53-year-old female, a known case of infiltrating ductal carcinoma of the breast, presented with complaints of dyspnea, mild chest pain, and unintentional weight loss for 2 weeks. On general physical examination, the patient was poorly built and severely anemic, and a mass lesion was present on the left breast. The left breast lesion was proven to be malignant by fine-needle aspiration cytology (FNAC) and she was advised for excision but the patient refused surgery. Rest of other systems were normal on clinical examination. Respiratory examination revealed bilateral reduced breath sounds and dull percussion notes on both sides. Her chest X-ray frontal view was suggestive of bilateral pleural effusion (Fig. 1). Her baseline blood investigation showed reduced hemoglobin of 6.5 gm/dL, serum protein of 4.2 gm, and hypoalbuminemia of 1.6 gm, and rest of the hematological tests were unremarkable. Diagnostic thoracentesis from the left side yielded hemorrhagic fluid which on cytology came out to be malignant in origin with atypical cells (Fig. 2). Left side chest tube thoracostomy was done to relieve persistent breathing difficulty. Further, suspecting it to be a case of bilateral malignant pleural effusion, diagnostic aspiration was performed from the contralateral side as well. The pleural fluid character from the right side was clear and straw colored (Fig. 3). Right side pleural fluid was transudative, low adenosine deaminase (ADA), negative for tuberculosis and malignant cells. Patient’s liver, kidney, and thyroid function test were unremarkable. Her electrocardiography (ECG) and two-dimensional (2D) echocardiography were also normal. Repeat cytology test of the pleural fluid from the right side was negative for malignant cells. Our patient was having bilateral pleural effusion due to two different causes; hence, we labeled it as a case of Contarini’s syndrome. She was managed with consecutive therapy, and pleurodesis was performed on the left side. The chest tube was removed without any refilling on the left side. The patient was advised to take high protein meal and discharged from hospital under stable condition after 2 weeks of hospital treatment and advised to follow-up after week. Unfortunately, she never came to follow-up after the discharge.

Fig. 1: Chest X-ray posteroanterior (PA) views with bilateral pleural effusion

Fig. 2: Pleural fluid cytology showing malignant cells in the pleural fluid (40×)

Fig. 3: Disparity in appearance of pleural fluid on bilateral sides

DISCUSSION

Pleural effusions, either unilateral or bilateral, are common in usual practice. Pleural effusions on both the sides are seen in 15–55% of patients.⁴ More than 90% of the pleural effusions are due to various causes such as cancer, heart failure, infection, and tuberculosis.⁵ Contarini’s syndrome refers to the occurrence of bilateral pleural effusion with a different etiology on both sides.⁶^-⁸ Parapneumonic pleural effusion can lead to heart failure, which in turn can cause transudative effusion on the opposite side.⁶ In our case, the patient had a malignant pleural effusion on the left side, as a result of metastasis from breast cancer, and a transudative effusion on the other side, most likely either due to occlusion of the lymphatics by cancer cells (paramalignant effusion) or as a result of both hypoproteinemia and both of these processes. Paramalignant effusions have been known to be associated with 13% of cases of breast cancer and lymphatic obstruction responsible for the transudative effusions.⁷ Mostly, a diagnostic pleural fluid aspiration in case of bilateral pleural effusion of well-known ethology is done from the side of more effusion.⁹ Rarely, pleural fluid aspiration from both sides is recommended in such cases. Very rarely, we come across a case of bilateral pleural effusion of different etiology on each side like in our case. In such cases, we need to further investigate for alternative diagnosis by aspiration of fluid from both sides. Experts recommend bilateral pleural fluid aspiration in certain situations, including the presence of atypical clinical presentation such as fever, chest pain associated with decompensated heart failure, single-side lung involvement, pleural effusion of significantly different quantity, and markedly different pleural fluid attenuation values on computed tomography (CT) in terms of Hounsfield units.⁸^-¹⁰

Therefore, the rationale to particularly consider a both-sided diagnostic pleural tapping is to avoid missing signiႡcant pathology in cases harboring different pathological conditions on both sides as in our case. One must individualize the patient to make a decision in the best interest of the patient, whether aspiration on one side is sufficient or aspiration on both sides is required to get the exact diagnosis.

CONCLUSION

To conclude, Contarini’s syndrome, an unusual entity, acts as a prompt to be observant while examining cases of bilateral pleural effusions. One should not presume that all bilateral pleural effusions are of the similar causes.

Clinical Significance

Contarini’s syndrome is a very unique presentation of bilateral pleural effusion with different etiology.
Pleural fluid aspiration from each side for bilateral effusion should be performed to investigate etiology of bilateral pleural effusion in clinical suspicion of alternate diagnosis.

ORCID

Arjun Kumar https://orcid.org/0000-0002-5581-6176

REFERENCES

1. McGrath EE, Barber C. Bilateral pleural effusions. CMAJ 2010;182(17):1879–1882. DOI: 10.1503/cmaj.090920

2. Kutty CP, Varkey B. ”Contarini’s condition:” bilateral pleural effusions with markedly different characteristics. Chest 1978;74(6):679–680. DOI: 10.1378/chest.74.6.679

3. Jarcho S. Empyema or hydrothorax in the Ninety-Fifth Doge of Venice. Bull N Y Acad Med 1970;46(5):378–385. PMID: 4912293.

4. Kalomenidis I, Rodriguez M, Barnette R, et al. Patient with bilateral pleural effusion: are the findings the same in each fluid? Chest 2003;124(1):167–176. DOI: 10.1378/chest.124.1.167

5. Light RW. Diagnostic principles in pleural disease. Eur Respir J 1997;10(2):476–481. DOI: 10.1183/09031936.97.10020476

6. Porcel JM, Civit MC, Bielsa S, et al. Contarini’s syndrome: bilateral pleural effusion, each side from different causes. J Hosp Med 2012;7(2):164–165. DOI: 10.1002/jhm.981

7. Gurung P, Huggins J, Doelken P, et al. Pleural fluid characteristics of paramalignant effusion. Chest 2009;136(4):44S–45S. DOI: 10.1016/S0012-3692(16)47987-0

8. Dixit R, Joshi N, Nawal CL. Contarini’s syndrome in a HIV positive patient. J Assoc Physicians India 2004;52:841–842. DOI: 10.1002/jhm.981

9. Puchalski JT, Argento AC, Murphy TE, et al. Etiologies of bilateral pleural effusions. Respir Med 2013;107(2):284–291. DOI: 10.1016/j.rmed.2012.10.004

10. Ferreiro L, San José ME, Gude F, et al. Derrame pleural bilateral: ¿toracocentesis uni o bilateral? Estudio prospectivo. Arch Bronconeumol 2016;52(4):189–195. DOI: 10.1016/j.arbr.2016.02.004

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