RU2797741C1 - Method of treatment of lymphovenous malformation - Google Patents

Abstract

FIELD: medicine, vascular surgery.

SUBSTANCE: in the operating room conditions, the location of the malformation and the point for puncture are determined in the patient under medical sedation and under the control of ultrasound navigation. Then, aspiration of the hemorrhagic contents of the malformation is carried out, after which a solution of the hemoblock preparation is injected at the rate of 1 ml/kg of the patient's weight under ultrasound control. After that, the needle is removed from the malformation cavity, an antiseptic dressing is applied at the injection site, followed by kinesio taping to achieve a lymphatic drainage effect.

EFFECT: method allows to significantly reduce the volume of vascular malformation, reduce the time of onset of a positive effect and recovery time, simplify treatment, increase lymph flow and minimize the development of serious complications.

8 dwg, 2 tbl

Description

The invention relates to medicine, namely to vascular surgery, and can be used in the treatment of lymphovenous malformations.

In the structure of the pathology of pediatric surgical diseases, vascular anomalies (VA) occupy a special place, since, on the one hand, part of this pathology has a high frequency of occurrence in children. For example, infantile hemangiomas occur in 5-12% of infants [1, 2]. On the other hand, some forms of SA are extremely rare. According to W. Wohlgemuth, among vascular malformations (SM), venous malformations (VM) are most common - in 70% of cases, lymphatic malformations (LM) are less common - in 12%, arteriovenous malformations occur in 8%, combined syndromes - in 6% and only in 4% - capillary malformations [3]. In addition, this group of pathologies has various clinical manifestations and symptoms, which complicates not only the process of diagnosis, but also the treatment itself.

It should be noted that depending on the pathology, the impact on the quality of life of the patient also changes, but in general, SA is extremely rarely fatal. For example, Breugem et al. note that VMs on the lower extremities slightly worsen the quality of life of patients [4], and, for example, according to the observations of Bhuyan et al., arteriovenous malformations significantly worsen the quality of life of the patient and are associated with significant bleeding, which in some cases can be the cause of death [ 5].

According to the modern classification of the International Society for the Study Vascular Anomalies (ISSVA, 2018) [6], it is customary to divide VA into vascular tumors (benign, borderline and malignant) and vascular malformations (SM) (with slow and fast flow characteristics). According to this classification, SM with slow flow characteristics includes both simple malformations (capillary, lymphatic, venous) and various variants of combined malformations.

SM is a congenital malformation of certain vessels that occurs during the formation of blood and lymphatic vessels during embryogenesis. To date, scientific searches for the causes of the appearance of SM are underway, some genes have been identified, mutations in which can lead to the appearance of SM. AK Greene analyzed articles in Pubmed from 1994 to 2017. and found that most SAs are associated with mutations that damage signaling tyrosine kinase receptors [7]. Most often, mutations were detected in the PIK3CA gene and in G-protein-coupled receptors. It should be noted that most AS are characterized by a mutation in one gene.

VMs are the most common SM [3]. This pathology is dilated veins, soaked in blood, which look like a sponge. Very often, phleboliths (calcifications) are found inside such cavities [3]. As a rule, CM appears more often in girls at an early age or during the period of hormonal changes (puberty, pregnancy). This malformation can have any localization, for example, the tongue, lips, cheeks, trunk and extremities. Very often, VM affects adjacent muscles.

CM can have various clinical manifestations - from a limited small blue vascular formation to an extensive diffuse pathological formation with large venous cavities with soft tissue deformation in the affected area. In these cavities, the blood flow differs from normal, here the flow rate is reduced, which leads to stagnation and the formation of phleboliths, which significantly increases the risk of thrombosis. Patients very often complain of edema, pain in the area of malformation. With large lesions, movement restriction in the limbs, shortening of the limbs, and an increase in lymphostasis are possible.

In the diagnosis of CM, a clinical examination by a specialist plays a leading role. When collecting data from the anamnesis and examining the patient, it is possible to identify characteristic signs for this pathology. On palpation, the formation may be hotter than the adjacent areas, or may have a similar temperature. This formation is very easily compressed under pressure, but when weakened, it refills with blood. In some cases, to clarify the diagnosis, it is necessary to use a stress test (Valsalva test).

Currently, the first line of treatment for SM with slow flow characteristics is sclerotherapy [8]. This method consists in puncturing the malformation with the introduction of a sclerosing agent into the cavity, which acts from the inside on the walls of the malformation, thereby causing aseptic inflammation, which leads to a decrease in the size of the malformation and the cessation of pathological blood flow inside the formation. This treatment is minimally invasive, does not leave scars, patients recover quickly after the procedure.

Various drugs can act as a sclerosing agent. So, for example, OK-432 (pitsibanil), bleomycin, doxycycline, alcohol, ethanolgel (alcogel), etc. are used for this purpose in the treatment of LM; ethoxysclerol, bleomycin, sodium tertradecyl sulfate (STS), alcohol, polidocanol are used in the treatment of CM , ethanolgel (alcogel), etc. [11]. Each of these drugs has its own advantages and disadvantages. The most effective sclerosant with the strongest sclerosing effect is 98% alcohol. It causes the formation of thrombosis (to a greater extent), damage to the endothelial lining of blood vessels and necrosis of the vessel walls and malformations (to a lesser extent) [12]. But due to the peculiarities of its administration (the restriction on the volume of administration should not exceed 1 ml / kg), the high risk of developing soft tissue necrosis, 98% alcohol is used very rarely in clinical practice. So far, the search for an "ideal" sclerosant that could meet all the requirements is ongoing.

Very often, sclerotherapy is used as a preparatory stage before surgical removal of a malformation. Primary excision of the malformation is rarely used, because after resection large scars remain, there is also a high risk of damage to the nerve bundles and the risk of recurrence of the disease is possible.

The disadvantages of this operation include the high cost of some sclerosing agents, the need to repeat the operation, and the risk of side effects after the introduction of a sclerosing agent should be taken into account.

So known from the prior art METHOD FOR SCLEROTHERAPY OF VARICOSE VEINS OF THE LOWER LIMB [RU2179457C1, publ. 20.02.2002], characterized by a puncture and the introduction of sclerosing drugs into a limited venous segment, characterized in that 1.0 ml of 0.25% sodium tetradecyl sulfate solution or 0.5% polidocanol solution is repeatedly injected into each point of the varicose vein without preliminary elevation limbs and subsequent elastic compression, conducting 3-4 sessions of injection-sclerotherapy.

The main technical problem of the prototype is the use of a solution of sodium tetradecyl sulfate or a 0.5% solution of polidocanol as sclerosing drugs, which leads to insufficient effectiveness of the treatment of malformations.

The objective of the invention is to eliminate the disadvantages of the prototype.

The technical result of the invention is to improve the results of sclerotherapy, in the form of a decrease in the size of lymphovenous malformations and a decrease in the risk of postoperative complications.

The specified technical result is achieved due to the fact that the method of treatment of lymphovenous malformation located subcutaneously, characterized in that under operating conditions, the patient under medical sedation under the control of ultrasound navigation determines the location of the malformation and the point for puncture, then aspiration of the hemorrhagic contents of the malformation is performed, after which a solution of the hemoblock preparation at the rate of 1 ml/kg of the patient's weight under ultrasound control, after which the needle is removed from the malformation cavity, an antiseptic dressing is applied at the injection site, followed by kinesiotaping to achieve a lymphatic drainage effect.

Brief description of the figures.

Figure 1 shows the ultrasound picture of a patient with VM: in the area of the VM is determined hyperechoic structure of a rounded shape with signs of "shadow echo".

Figure 2 shows the ultrasound picture of a patient with LM: with Doppler mapping, blood vessels are visible in the septa.

Figure 3 shows the stage of sclerosis with LM: under the control of ultrasound performed puncture cavity (visible section of the needle).

Figure 4 shows a front view of the patient.

Figure 5 shows a side view of the patient.

Figure 6 shows the MRI of the patient. T1 cor neck mode.

Figure 7 shows the MRI of the patient. Mode T2 cor STIR neck.

Figure 8 shows the result of the treatment of the patient.

Implementation of the invention.

Before treatment, a clinical examination is initially carried out.

All patients undergo soft tissue ultrasound to confirm the diagnosis and assess the extent of the lesion.

Based on the peculiarities of communication of VM with the venous bed, S. Puig classified VM into 4 types [8, 9]. This classification allows a preliminary assessment of the type of malformation and the selection of the optimal sclerosing agent.

All patients with CM need to perform an ultrasound examination (ultrasound) of soft tissues to assess the volume and nature of the lesion. On ultrasound, the VM is represented by the formation in the form of a conglomerate of dilated venous vessels, in the case of the presence of phleboliths, hyperechoic inclusions of a rounded shape with an echo shadow are determined (see Fig. 1). This formation does not have a blood flow or a slow blood flow is determined [3]. During compression, the VM is easily compressed under the sensor of the device, but when the compression is released, it quickly fills with blood.

In addition, the main method for diagnosing CM is magnetic resonance imaging (MRI). This study is the starting point for the final diagnosis, and also allows you to reliably assess the dynamics of the disease. In the T2 mode, the BM has a characteristic hyperintense signal, and in the T1 mode, an isointense signal. After the introduction of contrast in T1 mode, the signal becomes hyperintense due to the accumulation of contrast [3].

Native computed tomography (CT) is usually not used for diagnosing CM due to low information content; contrast-enhanced CT is indicated only in case of insufficient information content of MRI.

Since the main function of the lymphatic vessels is to provide immune protection, when bacteria enter the lymphatic channel, they can accumulate in dilated cavities, which most often leads to infection and suppuration, this condition requires emergency surgical treatment.

After an additional examination (CT / MRI) and the final diagnosis in a patient with lymphatic or lymphatic-venous malformation, the method of drug administration is determined.

For patients with lymphovenous malformations, also in the operating room under medical sedation (Sevoran, ABB VIE, UK) after treatment of the surgical field with antiseptic solutions, the location of the spinal cord is determined using an ultrasound device (GE Logic P6) to clarify the point of insertion of the brownules, but for percutaneous puncture and subsequent aspiration of hemorrhagic contents. The second stage of this technique is the introduction of a solution of the drug hemoblock at the rate of 1 ml/kg under ultrasound control, with further removal of the needle from the cavity of the lymphovenous malformation, and the application of an antiseptic dressing at the injection site, followed by kinesiotaping to achieve a lymphatic drainage effect.

Clinical examples of the invention.

A retrospective analysis of the treatment performed included 27 patients (24 patients with LM and 3 patients with LVM), who were treated at the International Center for Vascular Pathology from January to August 2018. The average age of patients was 4.15 years (from 2 months up to 18 years). The gender distribution was 1:1 (13 girls, 14 boys). According to the localization of the formation, they were most often found in the face and neck (70.4%), less often in the chest (14.8%), upper (11.1%) and lower extremities (3.7%). Information about patients is given in the table.

Indicators Lymphatic
malformation lymphovenous
malformation Total Number of patients 24 3 27 Boys:Girls 12:12 2:1 14:13 Average age, year, month 4.1 4.2 4.15 Localization: Face and neck 16 (59.3%) 3 (11.1%) 19 (70.4) torso 4 (14.8%) - 4 (14.8%) Upper limb 3 (11.1%) - 3 (11.1%) lower limb 1 (3.7%) - 1 (3.7%)

During therapy, all patients underwent surgical treatment of SC sclerosis. Before the operation, the patient or his parents signed an informed consent for the operation using the hemoblock preparation, since the therapy was carried out according to the offlabel protocol. All 27 patients were included in the main group.

The results of the treatment were assessed by the change in the size of the malformation before surgery and after 3-4 months. after sclerosis, during examination of the patient, according to ultrasound or MRI. When evaluating the results, preference was given to MRI data. The results obtained were divided into 4 groups: "no changes", "poor result" - a decrease in the size of the malformation by 40%, "good result" - a decrease in education by 40-70%, "excellent result" - a decrease in malformation by more than 70% or complete absence of SM after surgery.

A good result was obtained in 19 (70.3%) patients, excellent in 8 (29.7%) patients. There were no poor results and no changes during the study.

In 8 patients (30%), a pain reaction was noted in the early postoperative period, which stopped on its own after a couple of hours without the use of analgesics (this may be due to the individual characteristics of awakening). In 25 patients (90%), the appearance of swelling of soft tissues in the area of sclerosis was noted, which lasted up to 7-10 days. Moreover, the appearance of edema was noted 6-9 hours after the operation. In order to stop edema, patients were prescribed desensitizing drugs, and in case of severe edema, steroid hormones in age-specific dosages in short courses. No other side effects were noted.

Information about the results of the treatment is given in the table.

Result % Great 29.7 Good 70.3 Bad - No change -

The results obtained during the study were compared with data from a literature review by J. Acevedo [19], these patients formed the comparison group. The author analyzed 1876 articles describing the use of the sclerosing agents OK-432 and bleocin in the treatment of LM located in the head and neck. After applying the exclusion criteria, 22 articles entered the study, with confidence levels ranging from 4 (20 articles) to 2b and 1b. 289 patients took part in the studies. The mean age of the patients was 5.7 years. There were 165 boys (57%). 150 (59.2%) LMs were large cystic. According to the results of the study, an excellent result was obtained in 125 (43.25%) patients, good - in 85 (29.41%), poor - in 43 (14.88%), no result was obtained in 36 (12.46%). Another 36 (12.46%) patients required surgery after sclerotherapy. There were 7 severe complications: in the bleomycin group there were 2 deaths from pneumonia, in the OK-432 group there were 5 such complications: 2 children needed emergency intubation, one child underwent emergency tracheostomy and one child underwent decompression of the eye, in one case facial paralysis was noted after injection of OK-432 into the LM of the salivary gland. Minor side effects were common and included fever (OK-432), local pain and redness (many authors did not report these changes as minor side effects).

The data obtained during the study look encouraging. The use of Hemoblock as a sclerosing agent in the treatment of SM with slow flow characteristics is effective (an excellent result was obtained in 29.7%, a good result in 70.3%), safe (among the side effects were pain in the early postoperative period, which did not require the appointment of additional drugs, as well as the appearance of edema, which stopped on its own) and an affordable method of treatment.

However, one should take into account the limitation of our study in terms of the number of patients, as well as the treatment according to the off-label protocol, which requires additional multicenter studies and analysis of the results obtained.

Thus, hemoblock preparation can be used as a sclerosing agent in children with SM with slow flow characteristics as an alternative to other sclerosing agents. The results obtained are encouraging: in the course of the study, excellent and good results were obtained, not a single serious complication was noted.

This method can significantly reduce the volume of vascular malformation, reduce the time of onset of a positive effect and recovery time, simplify and reduce the cost of treatment, and minimize the development of serious complications.

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Claims (1)

A method for treating lymphovenous malformation located subcutaneously, characterized in that, under operating conditions, the patient under medical sedation under the control of ultrasound navigation determines the location of the malformation and the point for puncture, then aspiration of the hemorrhagic contents of the malformation is performed, after which a solution of the drug hemoblock is administered at a rate of 1 ml/kg the patient's weight under ultrasound control, after which the needle is removed from the malformation cavity, an antiseptic bandage is applied at the injection site, followed by kinesio taping to achieve a lymphatic drainage effect.

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