Mondor disease is a rare benign breast condition characterised by thrombophlebitis of the subcutaneous veins of the breast and anterior chest wall. It can also occur in the axilla, when it is called axillary web syndrome, or the penis.
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Epidemiology
Although Mondor disease is rarely reported in the literature, this is likely in part due to a lack of awareness of the entity. It tends to dominate in women between 30-60 years.
Incidence rates of 0.5-0.8% have been reported, however, it reflects only the symptomatic population.
Associations
In most patients (50-60% 12), the condition is idiopathic 4. Recognised associations include:
trauma, preceding thoracic or breast surgical procedures, ultrasound-guided or stereotactic biopsies
previous central venous catheters
hypercoagulable states
dehydration
although an association with breast cancer (~12% 2) has been reported, the veracity of this claim is difficult to confirm due to the over-representation of patients being investigated for other breast lesions in reported cases 1
Clinical presentation
Patients usually present with a painful breast (cord-like) mass. There may be overlying skin erythema. It may be accentuated when the ipsilateral arm is raised.
Pathology
The pathogenesis includes the formation of venous thrombosis with total or partial occlusion and vascular recanalisation causing fibromuscular hyperplasia of the vessel wall and infiltration plus fibrosis of surrounding subcutaneous cellular tissue. The thrombotic vessel can adhere to the superjacent skin causing retraction and formation of characteristic cordiform grooves secondary to local fibroblastic proliferation.
The veins most commonly involved are the thoracoepigastric, lateral thoracic, and superior epigastric veins.
Location - distribution
it most commonly affects the thoracoepigastric and/or lateral thoracic veins towards the upper outer quadrant; the upper inner quadrants of the breasts are almost never involved 6.
less commonly, penile Mondor disease or axillary web syndrome are seen
Radiographic features
Mammography
Typically, Mondor disease appears as superficially located tubular beaded density corresponding to a palpable rope-like mass. Mammography is normal in a significant proportion of cases.
Ultrasound
On ultrasound, Mondor disease appears as a tubular anechoic or isoechoic structure with multiple areas of narrowing, giving a beaded appearance. Sometimes low-level internal echoes may be present representing clot. The surrounding soft tissues may be hyperechoic due to the associated inflammatory response.
No flow is present on colour or spectral Doppler studies and in some situations, an abrupt cut off with the normal vessel may be seen.
Treatment and prognosis
It is a benign self-limiting condition, and the natural history is for the thrombosed vein to recanalise and for clinical symptoms to resolve gradually (~6 weeks). Anti-inflammatory medications, e.g. NSAIDs, are sometimes given for symptomatic relief.
Otherwise, management is supportive, and routine anticoagulants, antibiotics or surgical intervention are not indicated due to the thrombophlebitis subsiding spontaneously and without complications, or persistent deformity. Systemic anticoagulants are occasionally given to high risk patients 8.
A close interval follow-up scan is usually recommended to ensure resolution and to exclude any other entity.
History and etymology
In the year 1869, Faage was the first one to describe this condition 10,11. Further characterisation of this condition by the French surgeon Henri Mondor (1885-1962) was published in 1939, hence the eponym.
Interestingly Mondor is now more remembered for his biographies, than his surgical exploits. His subjects were both biomedical (Louis Pasteur, Guillaume Dupuytren, and René Leriche) and literary (French poets Stéphane Mallarmé and Paul Valéry) 7.
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