Chapter: Venous malformations
Article: 10 of 13
Update: Mar 25, 2021
Author(s): Barbera, Letterio Christian
Venous malformations very rarely have life-threatening consequences. These include thromboembolism with associated pulmonary embolism and life-threatening coagulation disorders caused by a consumption coagulopathy. The natural course, on the other hand, is often characterized by the occurrence of late complications. Progressive congestion leads to local, regional and systemic complaints which can impair the quality of life of the affected persons and even cause severe disability.
The increase in volume leads to progressive painful tension in the affected part of the body, so that compression and pain therapy have to be adjusted. Invasive therapy procedures are then necessary to enable the patient to enjoy pain-free everyday life.
The long-standing blood congestion of the skin and subcutis leads to trophic changes with reduced resistance to infection. Treatment-refractory, painful wounds can develop and cause relevant morbidity, especially in the ankle and foot area. Extensive wounds may even necessitate amputation if there is irreversible loss of function of the lower extremity. An appropriate prosthesis can restore considerable mobility.
Extensive intramuscular venous malformation in the lower extremities and the trunk can result in asymmetric posture and muscular weakness with considerable limitation of walking ability. Improper or too radical therapy can also lead to long-term mobility impairment.
In the case of joint and bone involvement, there is also an increased risk of fractures of bones that often have dysplasia. In this situation fracture healing is considerably delayed and may even lead to nonunion.
The above-mentioned damage to the musculoskeletal system is more pronounced when there is also segmental overgrowth or undergrowth. A center with the appropriate expertise should be involved in the care at an early stage.
In larger venous malformations there is ongoing localized coagulation and re-dissolution of clots in the lesion. This leads to the consumption of coagulation factors with low fibrinogen and high D-dimer laboratory values. This results in LIC (localized intravascular coagulation), which can lead to life-threatening bleeding, especially after being triggered by surgery and trauma. Before an operation, one must consider this possibility, check the coagulation values and treat prophylactically with anticoagulants, depending on the findings.
As extensive venous malformations usually persist for life, early and comprehensive pain treatment is necessary to reduce the risk of developing a chronic pain syndrome. On account of the variability of the findings, there is no universally accepted therapeutic approach. Rather, the affected person should be enabled to lead an active life as free of complaints as possible by means of several complementary conservative and invasive measures. The treatment strategy takes into account both the individual personality structure and the inherent dynamics of the malformation.