Mondor Disease 

Updated: May 13, 2019
Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD 

Overview

Background

First described in detail by Henri Mondor in 1939, this condition is a rare entity characterized by a sclerosing thrombophlebitis of the subcutaneous veins of the anterior chest wall, as diagrammed in the image below. Fage in 1870 has been credited with delineating cording as a sign of superficial thrombophlebitis.}

Illustration of the venous channels involved in Mo Illustration of the venous channels involved in Mondor disease. A is superior epigastric vein. B is thoracoepigastric vein. C is lateral thoracic vein.

The sudden appearance of a subcutaneous cord, which is initially red and tender and subsequently becomes a painless, tough, fibrous band that is accompanied by tension and skin retraction, is characteristic. The condition, though benign and self-limited, has been associated with breast cancer. It requires only symptomatic therapy. However, the physician must be aware of its existence to properly diagnose it and to rule out the presence of systemic disorders, especially breast cancer.[1]

Subcutaneous penile vein thrombosis (penile Mondor disease) has also been described.[2, 3] Its pathogenesis is unknown. It appears suddenly as almost painless indurations on the penile dorsal surface. Similar cording may rarely occur in the groin, abdomen, arm, and axilla.[4] In the latter site, it has been termed the axillary web syndrome and may be evident after axillary lymph node dissection and sentinel lymph node biopsy.

A related article is Superficial Thrombophlebitis.

Pathophysiology

The pathophysiology has been explained as pressure on the vein with stagnation of blood or as direct trauma to the vein itself. In cases that do not show such evidence, the most reasonable explanation is on the basis of repeated movement of the breast along with the contracting and relaxing pectoral muscles, which causes stretching and relaxing of the veins.[5] . An tight bra may be implicated by causing direct trauma.[6] Bodybuilders using intense thoracoabdominal exercise training may also develop it.[7]

Mondor disease may only involve one or more of three venous channels: the thoracoepigastric vein, the lateral thoracic vein, and the superior epigastric vein. The upper, inner portions of the breast are never involved. Mondor disease can also occur on the penis, groin, antecubital fossa, and posterior cervical region.[8, 9]

Although most often linked with breast cancer surgery and anesthetic mammaplasties,[10] it may also occur as a result of excision of axillary nodes after gel silicone breast implant rupture.[11] It may also be a complication of ultrasound-guided core needle biopsy.[12] Trauma from breast augmentation was described as a predisposing factor.[13]

Epidemiology

Frequency

Fewer than 400 cases have been described worldwide.[14] . However, its incidence after breast cancer surgery and aesthetic mammaplasties has been estimated at 1%.[11]

Race

No racial or ethnic predilection is evident.

Sex

Mondor disease is 3 times more common in women than in men.[15]

Age

The disease can occur in persons of any age, but most patients are aged 30-60 years.[15]

Prognosis

Mondor disease has proven to be self-limited and benign. Its significance lies in the clinician's recognition and differentiation of it from primary, recurrent, or metastatic carcinoma, or an abscess of the breast. As are other forms of migratory thrombophlebitis, Mondor disease may be an indication of an occult carcinoma elsewhere in the body. Patients with this condition should continue to be observed.

Patient Education

Explaining to the patient that no instance of Mondor disease has reportedly preceded or eventuated in breast cancer may be beneficial. However, patients with a history of Mondor disease should have periodic breast examinations, mammography, and additional tests searching for cancer elsewhere.

For patient education resources, see the Circulatory Problems Center and Cancer and Tumors Center, as well as Phlebitis, Breast Cancer, and Breast Self-Exam.

 

Presentation

History

Mondor disease is most often observed as a cordlike indurated plaque on the upper anterolateral aspect of the chest wall.[14, 16] No systemic symptoms are usually present, although rarely there may be chest pain.[17]

Ask the patient about the following:

  • Recent breast surgery: In one report, 7 of 15 patients had a radical mastectomy prior to the onset of Mondor thrombophlebitis on the ipsilateral side.[18] Mondor disease may occur after breast reduction surgery.[19]

  • Possible physical strain,[20] as might be experienced by bodybuilders[7]

  • Tight dressings and tight-fitting bras[21]

  • Axillary shaving[22]

  • Blood dyscrasia[23]

Subcutaneous penile vein thrombosis of other sites has also been described.[2] It is first evident as sudden and almost painless indurations on the penile dorsal surface.[24] In the axillae, it may occur after axillary lymph node dissection and sentinel lymph node biopsy.[4]

Physical Examination

Mondor disease has a characteristic clinical picture of a sudden appearance of a linear, cordlike, thrombosed vein. At first, this vein is red and tender, and then, it subsequently changes into a painless, tough, fibrous band. The cord is accentuated by traction, elevation of the breast, or abduction of the ipsilateral arm. It may also be evident as retracted breast skin.[25]

If the patient does not seek medical attention upon the initial presentation, the tenderness gradually subsides, while the thrombus organizes and recanalizes, leaving a nontender, hard, ropelike band. This band remains for varying periods up to several weeks.[5]

Complications

Mondor phlebitis is not migratory and does not recur.

 

DDx

Diagnostic Considerations

Mondor disease may be distinguished from inflammatory cancer of the breast by the presence of sudden pain, early skin adherence, and progressive improvement. These symptoms are not commonly associated with carcinoma of the breast.

The distinction from a breast abscess is made by discerning the quality of tenderness. Mondor disease is ascribed to a soreness, while an abscess presents with an exquisite tenderness.

Mondor disease may mimic a strangulated spigelian hernia.[26] Mondor disease may be evident in the spigelian hernia belt and cause diagnostic confusion.

Traumatic funicular phlebitis may be difficult to distinguished from and may resemble Mondor disease.[27] Similarly, Mondor disease may be associated rarely with abdominal pain that can mimic acute appendicitis.[28]

Nonvenereal sclerosing lymphangitis of the penis may also be considered.[29] It is not of venereal origin and is seen in men aged 20-40 years.

Differential Diagnoses

 

Workup

Laboratory Studies

No laboratory studies are necessary for diagnosis. With Mondor disease of the penis, an uncommon condition usually involving the superficial dorsal veins, ultrasound and Doppler ultrasonography examination is not useful for diagnosis but may help the clinician show the patient that the disease is a benign condition.[30]

Immunohistochemical staining for CD31 and D240 is a good method to distinguish small veins from lymphatic vessels, making it a valuable technique for diagnosing Mondor disease.[8]

Imaging Studies

Mammography results are always negative. This finding helps to differentiate Mondor disease from lesions, such as cancer, cysts, and fibroadenomata, which have positive radiography shadows.[31]

Ultrasonography may show Mondor disease to good advantage[32]

Histologic Findings

From histologic studies, 4 phases of the disease have been delineated: thrombus formation; thrombus organization; recanalization; and residual, thick-walled fibrosis.[33]

 

Treatment

Medical Care

Treatment is entirely symptomatic. Hot, wet dressings and anodynes may be used for pain relief. The use of modalities such as enzymes, corticosteroids, antibiotics, vaccines, and anticoagulants has been studied, although none has been proven to hasten the resolution of the pathologic process. The major benefit that the physician can provide is reassurance.

Surgical Care

Treatment is symptomatic because, to date, the disease has proved to be benign and self-limited.

Long-Term Monitoring

The natural course of Mondor disease is 3 weeks to 6 months. Reassurance during follow-up outpatient visits is most beneficial.

 

Medication

Medication Summary

Nonsteroidal anti-inflammatory agents (NSAIDs) are used for symptomatic relief.[34] Indomethacin is a good choice. Diclofenac may also be useful, with one study showing that sodium patches may be better than diclofenac oral capsules.[35]

Nonsteroidal anti-inflammatory drugs

Class Summary

These agents have analgesic, anti-inflammatory, and antipyretic activities. Their mechanism of action is not known, but they may inhibit cyclo-oxygenase activity and prostaglandin synthesis. Other mechanisms may include inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell membrane functions.

Indomethacin (Indocin)

Indomethacin is a potent inhibitor of cyclo-oxygenase, which may decrease the local production of arachidonic acid–derived chemotactic factors for eosinophils present in sebum.

Aspirin (Anacin, Ascriptin, Bayer Aspirin, Bayer Buffered Aspirin)

Aspirin treats mild to moderate pain. It inhibits prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2.

Diclofenac topical (Flector Transdermal Patch, Pennsaid topical solution, Solaraze Gel)

Diclofenac is one of a series of phenylacetic acids that have demonstrated anti-inflammatory and analgesic properties in pharmacologic studies. It is believed to inhibit COX, which is essential in the biosynthesis of prostaglandins.

 

Questions & Answers