# Recurrent cervical swelling syndrome as a rare cause of left supraclavicular edema: Case series and literature review

E. Charvet, T. Mahévas, S. Rivière, D. Gobert, V. Jachiet, V.

Panayotopoulos, N. Abisror, E. Ghrenassia, L. Arrive, S. Vignes, et al.

► To cite this version:

E. Charvet, T. Mahévas, S. Rivière, D. Gobert, V. Jachiet, et al. Recurrent cervical swelling syndrome as a rare cause of left supraclavicular edema: Case series and literature review. Annales de Dermatologie et de Vénéréologie, 2024, 151 (3), pp.103286. 10.1016/j.annder.2024.103286. hal-04652488

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Research letter

# Recurrent cervical swelling syndrome as a rare cause of left supraclavicular edema: Case series and literature review

<figure><img src="image_2.png" /></figure>

E. Charvet<sup>a,*</sup>, T. Mahévas<sup>a</sup>, S. Rivière<sup>a</sup>, D. Gobert<sup>a</sup>, V. Jachiet<sup>a</sup>, V. Panayotopoulos<sup>a</sup>, N. Abisror<sup>a</sup>, E. Ghrenassia<sup>a</sup>, L. Arrive<sup>b</sup>, S. Vignes<sup>c</sup>, A. Mekinian<sup>a</sup>, O. Fain<sup>a</sup>

ARTICLE INFO

Article history:

Received 9 December 2021

Accepted 22 May 2024

© 2024 The Authors. Published by Elsevier Masson SAS. This is an open access article under the CC BY license ([http://creativecommons.org/licenses/by/4.0/](http://creativecommons.org/licenses/by/4.0/)).

Keywords:

Angioedema

Lymphology

Oedema

1. Introduction

Supraclavicular edema is a relatively common condition in med-
ical practice. Superior vena cava syndrome, left supraclavicular
lymph node, hypothyroidism, and angioedema are common causes.
In some rare cases, lymphatic disorders such as cervical thoracic
duct anomalies are involved. The two main causes of cervical
chylous effusion are thoracic duct occlusion and post-traumatic
(postoperative or not) leakage [1]. Once these diagnoses have been
ruled out, the occurrence of recurrent, fluctuating, asymmetric,
left-sided supraclavicular edema suggests paroxysmal thoracic
duct occlusion syndrome, primarily described by Preyer and named
recurrent cervical swelling syndrome (RCSS) by Franceschi or
spontaneous cervical swelling syndrome by Betrains [2-4].

The purpose of this paper is to review the characteristics of RCSS and analyze the presenting signs and symptoms, therapeutic options, and outcome. Herein, we report seven new cases of RCSS and then present a literature review.

## 2. Case series and literature review

Seven adult patients with recurrent left cervical edema and diagnosis of RCSS, admitted to the Internal Medicine Unit of Saint Antoine Hospital (the French reference center for angioedema)

between January 1, 2015 and August 1, 2021 were included
(Table 1). Differential diagnoses were excluded. Two investigators
(EC, TM) then searched MEDLINE, PubMed Central, and Google
Scholar for articles in English or French published until December
2021 and containing the keywords "recurrent cervical swelling
syndrome, recurrent lymphangiectasia of the left supraclavicular
fossa, benign supraclavicular tumor, paroxysmal cervical swelling,
spontaneous cervical swelling syndrome". All articles with suffi-
cient data were included in the literature review. Among 676 ref-
erences screened, we found eight articles describing 19 cases of
recurrent, transient, and non-inflammatory left supraclavicular
edema with exclusion of differential diagnosis (malignant tumors,
cysts) (Tables 2 and 3) [4-8]. Data from seven patients followed at
our center (Table 1) were pooled with six well-documented cases
from the literature review (Table 3).

We identified 26 cases of RCSS. All patients were women with
left cervical edema lasting between 1 and 7 days (Fig. 1). The med-
ian age was 55.5 years old [interquartile range (IQR) 30–82]. The
median number of episodes before diagnosis was 3 [IQR 1–24].
The median time from first symptoms to diagnosis was 12 months
[range: 1–120]. Clinical and therapeutic characteristics are sum-
marized in Table 3. Fourteen patients had pleural effusion and
one patient had pericardial effusion without cardiac dysfunction.
Eleven patients had gastrointestinal symptoms, including: abdom-
inal pain (10/11), ascites (5/11), diarrhea and/or vomiting (3/11).
None of the patients had a history of chest wall trauma or thoracic
surgery. Four of the patients had eaten a high-fat meal in the pre-
vious days.

<footnote><sup>a</sup>Sorbonne University, Department of Internal Medicine, AP-HP, Saint Antoine Hospital, 75012 Paris, France</footnote>

<footnote><sup>b</sup>Assistance Publique-Hôpitaux de Paris, Radiology Department, Saint Antoine Hospital, AP-HP, 184 rue du Faubourg Saint Antoine, 75012 Paris, France</footnote>

<footnote><sup>c</sup>Lymphology Department, Primary Lymphedema Reference Center, Cognacq Jay Hospital, Paris, France</footnote>

<footnote>* Corresponding author at: Sorbonne University, Service de Médecine Interne, AP-HP, Hôpital Saint Antoine, 75012 Paris, France.
E-mail address: estelle.charvet@aphp.fr (E. Charvet).</footnote>
<table><caption>**Table 1**
Clinical and therapeutic characteristics of our seven patients presenting with recurrent cervical swelling syndrome.</caption><thead><tr><th>Case No./sex/age BMI (kg/m²)</th><th>Trigger</th><th>Duration Frequency of crises</th><th>Evolution (months) Median number of crises before diagnosis</th><th>Left cervical swelling associated symptoms</th><th>Imagery</th><th>Treatment</th><th>Efficacy</th></tr></thead><tbody><tr><td>1/F/55 BMI: 26</td><td>NS</td><td>3-5 days 1/month</td><td>18 24</td><td>Pleural effusion Ascites Abdominal pain Vomiting</td><td>- CT scan: pleural effusion and ascites<br>- MRI: focal dilatation supra clavicular lymphatic duct<br>- lymphoscintigraphy: left side asymmetry during the dynamic phase</td><td>Low-fat diet and Lanreotide (30 mg SR)</td><td>PR (decreased frequency) After 39 months of follow up</td></tr><tr><td>2/F/35 sickle cell disease BMI: 27</td><td>Fast food</td><td>3-4 days 1/year</td><td>12 3</td><td>Abdominal pain Diarrhea Ascites Pleural effusion Abdominal pain Diarrhea Ascites</td><td>- MRI: left clavicular edema ectasia of the butt of the thoracic duct without visible obstacle<br>- MRI: left clavicular edema<br>- US doppler: normal</td><td>Low fat diet and Lanreotide (30 mg SR)</td><td>CR no recurrence after 3 months of follow-up</td></tr><tr><td>3/F/28 BMI: NS</td><td>NS</td><td>3-5 days 1/6 months</td><td>6 3</td><td>Lumbar pain Abdominal pain</td><td>- MRI: left clavicular edema</td><td>Low fat diet</td><td>CR no recurrence after 14 months of follow-up</td></tr><tr><td>4/F/34 BMI: 21</td><td>Alcohol</td><td>3-4 days 1/3 months</td><td>48 15</td><td>Pleural effusion Ascites Pericardial effusion Abdominal pain Thoracic pain</td><td>- MRI: left clavicular edema</td><td>0</td><td>NS</td></tr><tr><td>5/F/63 BMI:19</td><td>Meal containing wild boar</td><td>2-3 days First event</td><td>1 1</td><td>Pleural effusion Ascites Abdominal pain Abdominal pain Thoracic pain</td><td>- MRI: left clavicular edema, thoracic duct dilatation without obstruction<br>- CT scan: left clavicular edema, pleural effusion, ascites<br>- MRI after crises: no compression</td><td>Low fat diet preventive enoxaparin</td><td>CR no recurrence after 15 months of follow-up</td></tr><tr><td>6/F/41 BMI: NS</td><td>NS</td><td>2-3 days 1/3 years</td><td>120 7</td><td>Pleural effusion Ascites Abdominal pain Abdominal pain</td><td>- MRI: left clavicular edema</td><td>Low fat diet</td><td>NS</td></tr><tr><td>7/F/45 BMI: 18</td><td>NS</td><td>4 1/2 months</td><td>11 5</td><td>Abdominal pain</td><td>- MRI: left clavicular edema</td><td>Low-fat diet and Lanreotide (30 mg SR)</td><td>PR 5 months of follow up</td></tr></tbody></table>

NS: not specified; Hb: hemoglobin; Ht: hematocrit; CT scan: computerized tomography scanner; MRI: magnetic resonance imaging; SR sustained release; CR complete remission; PR partial remission
<table><caption>**Table 2**
Summary of case reports from the literature review.</caption><thead><tr><th>Case No./sex/age</th><th>Trigger</th><th>Duration and frequency</th><th>Symptoms associated with left cervical swelling</th><th>Imaging</th><th>Treatment</th><th>Efficacy</th></tr></thead><tbody><tr><td>F/56 [5]</td><td>Hypertension of the spine</td><td>NS</td><td>Recurrent pleural effusion</td><td>- Chest X-ray: pleural effusion<br>- Chest CT scan: normal</td><td>NS</td><td>NS</td></tr><tr><td>F/67 [6]</td><td>NS</td><td>2 days</td><td>Dyspnea<br>Pleural effusion</td><td>- Chest X-ray: bilateral pleural effusion<br>- CT scan: normal</td><td>NS</td><td>NS</td></tr><tr><td>F/33 [7]</td><td>NS</td><td>5 days<br>4 events/3 years</td><td>Abdominal pain<br>Weight gain of 1-2 kg</td><td>- Duplex US: cystic tubular lesion of 30 × 14 × 17 mm in the subcutaneous tissue of the left supraclavicular groove<br>- MRI: cervical soft tissue surrounding the cyst appeared to be infiltrated with edematous fluid</td><td>Puncture of cyst</td><td>No recurrence</td></tr><tr><td>F/64 [9]</td><td>Large fat-rich meal</td><td>3 days<br>3 events / 25 years</td><td>Abdominal pain</td><td>- TDM: diffuse infiltration of the subcutaneous tissue of the left supraclavicular fossa and diffuse edema in the retroperitoneal area<br>- Lymphoscintigraphy: abnormal lymphatic drainage with trace extravasation into the left infraclavicular fossa</td><td>Low-fat diet supplemented with medium-chain triglycerides</td><td>No recurrence at one year of follow up</td></tr><tr><td>F/55 [3]</td><td>NS</td><td>NS</td><td>NS</td><td>- ECD: microcystic supraclavicular dilation<br>- ECD demonstrated a dilated non-compressible cervical TD (7 mm diameter)</td><td>No treatment</td><td>NS</td></tr><tr><td>F/44 [3]</td><td>NS</td><td>NS</td><td>NS</td><td>- ECD: dilated and obstructed thoracic duct (8 mm)<br>- CT scan: retroperitoneal effusion.</td><td>Low-fat diet</td><td>NS</td></tr><tr><td>F/58 [4]</td><td>NS</td><td>2 days<br>2 attacks prior to diagnosis</td><td>Dyspnea<br>Pleural effusion</td><td>- ECD: edema of the supraclavicular fossa and left cervical region extending toward the paratracheal region - no distension of the thoracic duct<br>- Chest CT: pleural fluid</td><td>NS</td><td>NS</td></tr><tr><td>F/46 [4]</td><td>Physical exercise</td><td>4 days<br>1 attack prior to diagnosis</td><td></td><td>- ECD: edema of the supraclavicular fossa - thoracic duct distension present</td><td>NS</td><td>NS</td></tr><tr><td>F/65 [4]</td><td>Physical labor</td><td>3 days<br>3 attacks before diagnosis</td><td>Dyspnea<br>Pleural effusion</td><td>- ECD: edema of the supraclavicular fossa and left cervical region extending to the paratracheal region - no distension thoracic duct<br>- Chest CT: bilateral pleural fluid<br>- Lymphoscintigraphy: extravasation left cervical region</td><td>NS</td><td>NS</td></tr><tr><td>F/58 [4]</td><td>Physical exercise</td><td>3.5 days<br>Recurrent attacks</td><td>Dyspnea<br>Pleural effusion</td><td>- ECD: edema of the supraclavicular fossa and left cervical region-thoracic duct distension absent<br>- Chest CT: bilateral pleural fluid</td><td>NS</td><td>NS</td></tr><tr><td>F/53 [4]</td><td>Warm weather</td><td>3.5 days</td><td>Pleural effusion</td><td>- ECD: edema of the supraclavicular fossa and left cervical region extending toward the paratracheal region-thoracic duct distension present<br>- Chest CT: bilateral pleural fluid</td><td>NS</td><td>NS</td></tr><tr><td>F/38 [4]</td><td>NS</td><td>NS</td><td>Pleural effusion</td><td>- ECD: edema of the supraclavicular fossa and left cervical region-thoracic duct distension absent<br>- Chest CT: bilateral pleural fluid</td><td>NS</td><td>NS</td></tr><tr><td>F/47 [4]</td><td>NS</td><td>7 days<br>2 attacks before diagnosis</td><td>Pleural effusion</td><td>- ECD: edema of the supraclavicular fossa and left cervical region-thoracic duct distension absent<br>- Chest CT: bilateral pleural fluid<br>- Lymphoscintigraphy: Extravasation left cervical region</td><td>NS</td><td>NS</td></tr><tr><td>F/82 [4]</td><td>high-fat meal and alcohol</td><td>Few days</td><td>Dyspnea<br>Pleural effusion</td><td>- ECD: edema of the supraclavicular fossa and left cervical region-thoracic duct distension present<br>- CT scan: minor bilateral pleural effusion (predominantly on the left side), infiltration of the left side of the neck extending to the left axilla and the mediastinum</td><td>anticoagulant therapy</td><td>NS</td></tr><tr><td>F/74 [8]</td><td>NS</td><td>2 days</td><td>Abdominal pain</td><td>- CT scan: infiltration of subcutaneous tissue in the supraclavicular fossa and in the omohyoid muscle, and extensive inflammatory infiltrate in the retroperitoneum.<br>- MRI: small cyst in the cervical area.</td><td></td><td></td></tr><tr><td>F/65 [8]</td><td>NS</td><td>NS</td><td>Dyspnea</td><td>- Lymphoscintigraphy: impregnation time of 2 h and 45 min on the axillary lymphatic vessel (normal values within 10-30 min)<br>- CT scan: liquid infiltration in the left supraclavicular hollow in the retroperitoneum and in the mediastinum.</td><td></td><td></td></tr><tr><td>F/64 [8]</td><td></td><td></td><td></td><td>- Lymphoscintigraphy: significant delay of the lymphatic flow in the thoracic duct<br>- CT scan: left supraclavicular mass associated with abdominal infiltration distributed around the colon and minor bilateral pleural effusions.</td><td></td><td></td></tr><tr><td>F/62 [8]</td><td></td><td></td><td></td><td>- Pleural puncture: chylous effusion</td><td>low-fat diet</td><td>No recurrence at 3 months of follow up</td></tr></tbody></table>

CT scan: Computed Tomography scan, ECD: Echo Color Doppler; NS: not specified.
<table><caption>**Table 3**
Characteristics of all patients with recurrent swelling syndrome.</caption><thead><tr><th>Characteristics</th><th>N = 26</th></tr></thead><tbody><tr><td>Age (years;median [IQR]), female (n,%)</td><td>55.5 [30–82], 26 (100%)</td></tr><tr><td>Duration in days (n, median [IQR])</td><td>4 [1–7]</td></tr><tr><td>Pleural effusion (n)</td><td>14</td></tr><tr><td>Abdominal pain / Ascites (n)</td><td>10/5</td></tr><tr><td>Trigger fat-rich meal / physical exercise (n)</td><td>4/3</td></tr><tr><td>Thoracic duct dilatation (MRI)</td><td>7</td></tr><tr><td>Treatment</td><td>11</td></tr><tr><td>Low fat diet</td><td>6 (4 CR,2 NS)</td></tr><tr><td>Low fat diet + lanreotide</td><td>4 (2CR, 2 PR)</td></tr></tbody></table>

IQR: interquartile range, CR: complete response, PR: partial response, NS: not specified; MRI magnetic resonance imaging.

Standard laboratory tests, including blood cell count, liver and kidney function, thyroid stimulating hormone, lipids, and C-reactive protein were normal in all our patients. One patient had sickle cell disease with a history of hemodilution. Protein electrophoresis showed no monoclonal peak, and plasma C1 inhibitor protein activity was normal in 7/7 patients. Chest computed tomography (CT) showed no deep lymphadenopathy and no superior vena cava in all cases. Thoracic magnetic resonance imaging (MRI) of lymph nodes was performed during attacks in 7 patients and showed left supraclavicular edema in all cases and a dilated aspect of the thoracic duct terminal without occlusion or cervical thoracic duct cyst in 5 cases. Two of our patients received icatibant, antihistamines and intravenous corticoids for suspected bradykinin or histamine angioedema, and one patient received intravenous immunoglobulins for suspected Clarkson's disease without benefit. Finally, 10 patients were treated with a low-fat diet, and four patients were also treated with lanreotide (30 mg extended-release intramuscular injection once a month), which resulted in partial remission in two patients (reduced frequency or duration of attacks) and complete remission in the other two patients.

### 3. Discussion

Recurrent cervical swelling syndrome (RCSS), also known as paroxysmal thoracic duct occlusion syndrome or recurrent left supraclavicular lymphangiectasia, is poorly described in the literature [9]. As indicated above, other causes of supraclavicular edema must be excluded before RCSS can be diagnosed. Preyer et al. were

the first to report this rare entity [2]. Franceschi proposed the term of recurrent cervical swelling syndrome [3]. Since then, very few cases have been reported. To date, there is no consensus on diagnostic criteria. The diagnosis of RCSS is based on clinical and radiographic signs. Clinically, RCSS is a recurrent left supraclavicular noninflammatory edema, sometimes associated with other signs such as pleural effusion, pericardial effusion and ascites. Thoracic imaging such as CT scan is important to rule out superior vena cava syndrome and left supraclavicular lymph node. Thoracic duct imaging is necessary to rule out endo- or extra-luminal occlusion of the thoracic duct and lymph-node MRI seems to be the best option for this [10].

In our series of 7 personal cases and 19 cases from the literature, the duration of left cervical swelling was 1–7 days, with associated signs in all cases. Pleural effusion and dyspnea were more common than abdominal pain and ascites. The pathophysiology of this syndrome is poorly understood. Many variants of terminal thoracic duct (TD) exist but the main termination seems to be on the left side, either in the internal jugular vein or in its confluence with the subclavian vein [11]. This preferential localization is the cause of this left supraclavicular edema. With regard to our cases, in which we indicate that a high-fat diet was consumed a few days before the edema, and with regard to the literature review, a possible hypothesis for this rare phenomenon could be that a high-fat diet may cause an influx of chylomicrons into the thoracic duct that exceeds the transport capacities, thus causing a temporary and paroxysmal lymphatic stasis in the thoracic duct [9]. However, diet was not always mentioned in the patients' medical history and no rechallenge with a high-fat diet was suggested in our patients. Lipid levels were normal throughout. The other hypothesis could be that of a constitutional abnormality of the thoracic duct such as ectasia, which may favor the extravasation of lymphatic fluid into the subcutaneous tissue [12]. No anatomical abnormalities were described in our series. Finally, in our experience and in the literature, all cases were described in women. No correlation with menstruation was observed; however, estrogenic influence could be implicated in the mechanism of edema. In their series, Betrains et al. suggest a possible role of physical activity and outdoor temperature, which we did not find either in our series or in the other reported cases [4]. Taken together, a high-fat diet, the influence of female hormones, and thoracic duct ectasia could lead to edema.

In conclusion, we propose three diagnostic criteria for the diagnosis of RCSS:

<figure><img src="image_3.png" /><figcaption>**Fig. 1.** (a–c) Patients with recurrent swelling syndrome during an attack. (d) Thoracic lymph-node magnetic resonance imaging (T2): left supraclavicular edema (star) and dilated aspect of the thoracic duct endings (arrow).</figcaption></figure>
(1) Clinical: Recurrent non-inflammatory left supraclavicular edema,

(2) Radiological: CT or lymph node MRI of the neck with evidence of left supraclavicular edema at the end of the thoracic duct, which is normal or dilated,

(3) Exclusion of the following diseases: vena cava syndrome, bradykinin angioedema, tumor or lymph node, lymphatic malformation, and hypothyroidism.

In the absence of large series, the therapeutic options in RCSS are limited. In some cases, a low-fat diet seems to be effective [9]. Second, lanreotide, a somatostatin analog also used in Waldmann's disease, could decrease the chyle flow in the thoracic duct [13]. With this treatment, two of our patients experienced a reduction in the number and duration of attacks [14].

## Conflicts of interest

The authors declare that they have no conflicts of interest.

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