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Klippel-Trenone syndrome
Medical expert of the article
Last reviewed: 23.04.2024
[Epidemiology
The frequency of the appearance of the defect is not great: for the last century about 400 observations have been described, and for the children's age there are no more than 100. The lower extremities suffer mainly. The Klippel-Trenone syndrome is more common in boys, it is mostly one-sided, manifested with the birth of a child.
What causes the Klippel-Trenone syndrome?
The pathogenetic mechanism of this suffering is a congenital partial and complete block of venous outflow from the affected limb. The blockade of the main veins leads to chronic phlebogertonia in the distal parts of the limb and the system of vein-collaterals. Adverse hemodynamic conditions (increased arterial resistance and blood overflow of capillaries and venules) cause trophic disturbances, lymphostasis and development of sclerotic process of all limb tissues. Violation of blood flow through deep veins can be caused by aplasia or hypoplasia of the vein, hyperplasia, compression of the veins from the outside by strands, embryonic spikes, abnormal muscles, aberrant arteries.
How does the Klippel-Trenone syndrome manifest itself?
The vice is characterized by a triad of symptoms: the expansion of superficial veins, elongation or thickening of the extremity, angiomas. Vascular and pigment spots are detected on the skin of the diseased limb.
The most constant symptom is the expansion of the superficial veins, which has the following features: stem-type extensions, localization of varicose along the outside of the extremity, absence of pulsation in any sound phenomena above the nodes. The second important symptom is the progressive elongation and thickening of the affected limb. Vascular spots (capillary dysplasia) and angiomas are noted in 70% of patients. In addition to these symptoms, trophic skin changes (hyperhidrosis, hypertrichosis, hyperkeratosis, trophic ulcers), changes in the musculoskeletal system, bleeding, inflammation are possible.
Laboratory and instrumental research
Recognition of the disease is based on the comparison of clinical and special research methods. The leading place in diagnosis is angiography, which allows not only to assess the extent of venous system damage, but also to clarify the localization of the trunk vein block. The absence of a vein shadow on one of the segments or the entire limb, characteristic of the Klippel-Trenone syndrome, contrasting embryonic veins is revealed.
Rheography reveals an increase in peripheral arteriolar resistance. A measurement of venous pressure shows a significant increase in the latter compared with the opposite side.
Differential diagnostics
Differential diagnosis is carried out with congenital arteriovenous communications, which are similar only in appearance, but have other hemodynamic disorders, consisting in the rapid discharge of arterial blood into the venous bed. In this case, the pulsation of veins, an increase in local temperature, sound phenomena over the vessels are noted. When comparing clinical, morphological and functional data, a definite staging of the course of the process was detected: compensated hemodynamic disturbances in children under 2-5 years, subcompensated - up to 5-7 years, stage of chronic venous insufficiency - over 7 years. Therefore, it is most expedient to operate such children in the age from 2 to 5 years.
How is the Klippel-Trenone syndrome treated?
Treatment of the developmental defect of the deep veins of the lower extremities is only surgical, aimed at restoring the physiological blood flow through the deep veins. With hypoplasia and external compression, phlebolysis is produced, which helps normalize blood flow. With pronounced hypoplasia or aplasia with the help of microsurgical techniques, excision of the vicious area is performed, replacing it with a fragment of the contralateral large saphenous vein along with the valve. A similar operation is possible with a complete deep vein aplasia.
What is the prognosis of the Klippel-Trenone syndrome?
The prognosis is relatively favorable with the timely treatment of the defect.