Venous malformations
What are venous malformations
Congenital venous malformations are aberrant embryonic development or stopping at a certain stage of development of the venous circulatory system that is genetically determined (abnormal embryogenesis). In other words, venous malformations are diseases that affect us from the intrauterine stage of life, with the indication of surgical treatment when they trigger the onset of clinical symptoms (some of the malformations are discovered accidentally during the autopsy and are not clinically manifested).
What are the most common venous malformations
The appearance of vascular malformations is not unique, these being frequently accompanied by other developmental abnormalities of the organs (several mutant genes dictate the appearance of malformations) so their inclusion in various syndromes is based on the association with various diseases:
- Avalvulia Klippel-Trenaunay syndrome is characterized by the presence of malformations located at the level of capillaries, hypertrophy of the upper and/or lower limbs and varicose dilatations of the lower limbs;
- Parks-Weber syndrome that is characterized by the appearance of congenital arteriovenous fistulas and venous angioma-excessive development of the venous network;
- Cerebral arteriovenous malformations present as vascular conglomerates in the form of dilatations in which there is an intensified blood flow that over time associates increased pressure with the weakening of the venous walls and the risk of spontaneous rupture-hemorrhagic strokes;
- Congenital cavernous or cutaneous hemangiomas occur due to small capillary neoformation;
- Telangiectasias are dilations of the capillaries throughout the body that can be included in Rendu-Osler syndrome;
- The deficit of the formation of the primary septum determines the direct connection of the pulmonary veins in the right atrium, a condition characterized by dilation with hypertrophy and progressive heart failure;
- Congenital pulmonary venous obstruction.
Venous malformations symptoms
Clinical symptoms include a wide range of manifestations determined in particular by the location, extent and degree of venous abnormality. In the case of intracerebral venous malformations, the clinical symptoms are acute and are most often due to hemorrhagic episodes caused by capillary rupture: nausea, vomiting, dizziness, headache, loss of consciousness, balance disorders, speech impairment, motor deficits or decreased vision. ).
Congenital pulmonary venous defects cause cardiac and pulmonary decompensation over time and are characterized by specific symptoms such as cyanosis (pulmonary venous obstruction), respiratory failure, hemoptysis, increased heart rate, pulmonary arterial hypertension.
Congenital venous syndromes are manifested in the form of varicose veins (especially in the lower limb hypertrophied in Klippel-Trenauney syndrome) accompanied by venous valve incompetence, stasis and edema and venous angiomas (cutaneous, cavernous) in various areas of the body.
Surgical treatment
Options in the treatment of congenital venous malformations are generally limited to interventional ones, being represented by cosmetic surgery (in the case of pigment spots and spider veins), reconstruction (septal or venous when possible), treatment of complications (evacuation of intracerebral hematomas, removal of venous malformation, removal of thrombi that obstruct the flow) or transplantation (lung, heart) depending on the severity of the clinical manifestations.