A 27-Year-Old Japanese Woman Presenting with Left Chest Wall Pain Due to Palpable and Visible Sclerosing Superficial Thrombophlebitis (Mondor’s Disease)

Introduction

Mondor’s disease, a variant of thrombophlebitis that primarily affects superficial veins of the breast and anterior chest wall, is a rare and benign condition. Cases of cord-like lesions on the chest wall were first reported in the early 1850s, with French surgeon Henri Mondor reporting the first case series in 1939 [1]. Sparse reports suggest an incidence of approximately 0.5–0.8% [2]. Often excruciatingly painful, Mondor’s disease remains an important clinical entity due to its distinctive presentation and potential for misdiagnosis. More frequently seen in women, it can also occur in men (3: 1 ratio) and cases involving the penis have also been reported [3–5]. No correlation to race or hereditary link has been identified. Although no cause is found in 50–60% of cases, triggering factors such as previous trauma, surgery, infection, and excessive exercise are thought to play a contributing role in 40–50% of cases [6]. A literature review by Amano revealed that 45% of cases were idiopathic, 20% iatrogenic, 22% traumatic, and 5% related to breast cancer [7].

The disease can manifest at any age, but predominantly affects middle-aged individuals. Its course is generally benign and self-limiting, with spontaneous resolution typically occurring within 4–8 weeks. The diagnosis of Mondor’s disease is based on patient history and examination. Imaging studies such as Doppler ultrasound, typically revealing a superficial, anechoic tubular structure without blood flow within the affected vein, may be used to confirm the clinical diagnosis. However, imaging findings may have a low sensitivity and are not demonstrated in some cases. Management of Mondor’s disease focuses on symptomatic relief, as the condition is self-limiting. First-line treatments include NSAIDs and other analgesics. This report is of a 27-year-old Japanese woman presenting with left chest wall pain due to palpable and visible sclerosing superficial thrombophlebitis (Mondor’s disease).

Case Report

A 27-year-old Japanese woman who was a physical therapist presented to our outpatient hospital with 8 days of left chest wall pain (Numerical Rating Scale (NRS) 6/10) and swelling. Her past medical history included a single episode of left intercostal neuralgia 9 months prior to presentation, and no suggestive triggers such as previous trauma, surgery, infection, excessive exercise, or prolonged pressure. Her family history included colon cancer in her grandmother. The patient did not smoke or drink alcohol. She reported allergies to shrimp and ceftriaxone. No abnormalities were found on breast cancer screening performed 4 months prior to her presentation.

Physical examination revealed a hard cord, slightly raised and tender to palpation, extending from the left submammary region to the upper abdominal area (Figure 1). Her body temperature was 35.9°C, blood pressure was 118/62 mmHg, pulse rate was 80/min, and respiratory rate was 13/min. Laboratory findings revealed hemoglobin 13.6 g/L (35.6–45.4), platelets 258×109/L (153–346), leucocytes 6.1×109/L (3.6–8.9), Activated Partial Thromboplastin Time (APTT) 28.2 s (−36.2), Prothrombin Time (PT) 12.4 s (−13.5), International Normalized Ratio (INR) 0.92 (0.9–1.1), D-dimer <1 μg/mL (0–1), creatinine 0.65 mg/dL (0.50–0.80), and C-reactive protein 0.07 mg/dl (−0.29). The electrocardiogram was normal. Wells score for Deep Venous Thrombosis (DVT) was 0 points (≤0 low risk) and Wells score for Pulmonary Embolism (PE) was 0 points (0–1 low risk) [8,9].

Chest wall ultrasound revealed a superficial cord, tubular and anechoic, measuring 1.15 cm at its widest diameter. The cord length was 15 cm, extending from the left breast to the upper abdomen. Doppler imaging demonstrated normal blood flow surrounding the cord, with no blood flow within (Figure 2). CT revealed a subtle structure beneath the lower left breast skin, without breast or lung involvement (Figure 3).

Based on the characteristics of the lesion, we diagnosed her with MD. She was prescribed NSAIDs twice a day for approximately 3 weeks. Her pain gradually improved over this period and the cord disappeared after approximately 2 months. She remains symptom-free.

Discussion

MD is generally diagnosed based on a detailed medical history, physical examination, and ultrasonography. In addition, we suggest CT to quickly rule out potentially serious coexisting diseases such as malignant tumors. MD, although rare, is characterized by thrombophlebitis of the superficial veins, typically presenting as a palpable subcutaneous cord-link structure. The most common sites of involvement in this disease are thoraco-epigastric, superficial epigastric, and lateral thoracic veins [10]. Though bilateral disease has been reported, MD generally appears unilaterally, in the breast, upper abdominal regions, and/or extremities [2,11]. Recurrence has been rarely reported in the literature [12]. The disease can appear at any age, but it usually affects people in middle age, and has a benign and self-limiting course [13].

While the etiology of the disease remains unknown, it appears to be idiopathic in many cases. A few papers have reported associated factors such as preceding injury, surgical biopsy and trauma, inflammatory and infectious breast conditions, carcinoma or cancer (especially breast cancer), local muscle strains, large pendulous breasts, tight clothing or bandages, vigorous upper-extremity exercise such snow shoveling, abuse of intravenous drugs, lymphoma, lymphangitis, adenopathy, shaving, lupus erythematosus, radiation, hormone therapy, thrombophilic conditions, pregnancy, oral contraceptives use, and rheumatic arthritis [6,12,14,15]. In this case, we found through the patient interview that her work as a physiotherapist involving physical strain was the cause of MD. This underscores the importance of evaluating a patient’s occupation.

A detailed medical history and physical examination are crucial for diagnosis of MD. The primary concern of patients with MD is usually an area of induration. During physical examination, a cord-like lesion that is several centimeters long can be readily observed, and a firm induration can be felt under the skin. When there is severe inflammation in the tissue surrounding the affected vein, noticeable skin retraction occurs [16].

Because lesions can appear in areas close to the pubic region, such as the breasts and penis, it is necessary to assemble a medical team with sufficient knowledge to provide treatment [17].

Superficial thrombophlebitis typically presents as palpable cords along superficial veins that are red, swollen, warm, inflamed, and tender. Similar to MD, it occurs more frequently in women, but the most common site of occurrence is the lower extremities, especially the great saphenous vein (60–80%). Patients often have an underlying disease with a tendency to thrombosis, such as varicose veins. For mild cases, symptomatic treatments such as NSAIDs are appropriate, while anticoagulant therapy is reserved for severe cases [18]. In this case, we hypothesized that MD was likely due to the redness of the lesion, the warmth, and normal inflammatory markers found in blood testing. Additionally, there were no electrocardiogram abnormalities, and a low Well’s score indicated a low probability of DVT and PE, further supporting the diagnosis [8,9].

Generally, MD regresses spontaneously within 4–8 weeks and requires no special treatment. Warm compresses, non-steroidal anti-inflammatory drugs and other analgesics, and avoidance of irritating clothing and activities are first-line treatments. Most lesions will heal without the need for further treatment. Some authors have reported the effectiveness of anticoagulant treatment during the acute phase of the disease with low-molecular-weight heparin or aspirin [14,15,19,20]. The characteristics of MD reported in previous studies were consistent with the patient’s sex, age, pain location, and unilaterality, as well as treatment duration and method. This patient was a physical therapist, and we suspect that chronic physical overuse may have been a strong contributing factor. The cord exhibited pain that was exacerbated by traction, elevation of the breast, twisting of the body, or abduction of the ipsilateral arm. In such situations, patients may have discomfort and pain around the cord. This case showed rapid improvement with NSAIDs but clinicians should be aware that previous studies indicate MD can recur. It is therefore appropriate to inform patients of this possibility.

Several reports have recommended the following treatment for superficial vein thrombosis: Administer low-molecular-weight heparin (Dalteparin 5000–10 000 units SC daily, Enoxaparin 40–80 mg SC daily, Nadroparin 2850–5700 units SC daily, Tinzaparin 4500–10 000 units SC daily) or Fondaparinux (2.5 mg SC daily), Rivaroxaban (10 mg PO daily) for 45 days. Another treatment option involves administering oral or topical NSAIDs, such as Ibuprofen (400 mg PO TID) or Naproxen (500 mg PO BID) or 2–4 g topical diclofenac applied to affected area 3 or 4 times daily for 714 days [21–23].

There are no clear diagnostic guidelines for MD, but we think of it as shown in the Figure 4. First, check for pain in the chest wall through a medical interview, and check for risk factors such as recent surgery, trauma, or excessive exercise. Then, a physical examination is performed to look for typical findings, such as a cord, in the same area as the pain. If a cord is found, ultrasonography is performed to confirm the absence of hypoechoic luminal structures and blood flow, which are characteristic of thrombophlebitis. Doppler echo is useful in assessing the presence or absence of blood flow. Treatment can be initiated if both physical examination and ultrasonography findings are consistent with MD. However, if typical findings cannot be obtained, it may be necessary to consider the possibility of other diseases, especially malignant diseases that should not be overlooked, and to perform other imaging tests such as CT. CT is useful for confirming diseases that are difficult to detect with ultrasound, such as malignant tumors (eg, lung cancer and breast cancer), and inflammation of the thoracoabdominal organs. Furthermore, if a hospital does not have staff trained in breast echography techniques, breast cancer may be overlooked or misdiagnosed, so we think that CT is very important.

In comparison to previous reports, this case was typical of MD regarding sex, age, symptoms, and clinical course. The diagnostic process involved medical history, physical examination, and imaging, consistent with prior cases. However, in this case we considered the patient’s occupation as a physical therapist as a possible contributing factor to symptom onset. Additionally, a CT was performed to rule out urgent diseases such as malignancy, and a unique diagnostic algorithm was established, contributing a novel aspect to this report.

Conclusions

The case presented in this report was diagnosed as MD based on typical physical exam findings and experiencing improvement with NSAIDs treatment. As with our proposed algorithm, ultrasonography can be used to make a sufficient diagnosis, but since MD occurs more often in young people, it is necessary to consider the prognosis and rule out malignant diseases: therefore, performing CT is very important. Even after diagnosing MD and starting treatment without performing CT, if the progress is poor, it is important to consider other diseases and perform CT at an early stage.