Purpose is to identify the factors that determine the clinical features of the LMs, their complications and the choice of treatment options. Materials and methods. 225 children with LMs who underwent treatment since 2011 to 2020 were enrolled. They were grouped according to ISSVA 2018 classification. Clinical presentation, treatment options, complications and outcomes were analyzed. One-variant analysis using Pearson’s χ2 test was used to evaluate qualitative data. Results. 214 (95.1%) patients had cystic LMs, 11 (4.9%) had combined LMs. 129 (57.3%) LMs were cervicofacial, 17 (11.9%) patients developed airways compression and 6 (4.2%) required tracheostomy. 8 (3.6%) patients developed 10 lymphatic leaks episodes, 3 were lethal. 112 (86.8%) patients with cervicofacial, 5 (11.6%) abdominal, 10 (84.6%) axillar, and 12 (92.2%) extremities LMs underwent sclerotherapy. Surgical resections prevailed in abdominal LMs, 32 (74.5%), versus 4 (3.1%) cervicofacial LMs. 10 (4.4%) patients with combined and complicated cystic LMs underwent systemic sirolimus therapy. Results of treatment were excellent in 116 (51.6%) patients, good in 82 (36.4%), satisfactory in 23 (10.2%), and unsatisfactory in 4 (1.8%), with 4 mortalities caused by sepsis (n=1) and by massive lymph leakage (n=3). Conclusions. The most life-threatening LMs complications are airways compression and uncontrollable lymphatic leakages. Several approaches were used, including surgery, sclerotherapy, and target therapy. Surgery is optional for abdominal LMs but potentially harmful in cervicofacial. Treatment of combined LM is continuous and requires a combination of different methods. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institutions. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors. Keywords: lymphatic malformations, children, sclerotherapy, target therapy.
Modern methods of surgical treatment of the prehepatic form of portal hypertension (PPH) aim to prevent bleeding from esophagus varicose veins and to restore liver portal perfusion. The use of mesoportal shunt (MPS) in patients with PPH is limited by the presence of favorable anatomy in the latter. The standardization of the diagnostic procedures volume, stages of surgery technical performance will help to significantly improve the results of treatment of children with PHP, to which indications for the MPS were established. Purpose - to evaluate the possibility of performing, efficiency and safety of MPS in children with portal hypertension, the degree of esophageal varicose veins involution and reducing the spleen volume reduction. Materials and methods. Case histories of 20 children who underwent surgical treatment of PPH by means of MPS in the period from January 2010 to June 2022 were retrospectively studied. The median follow up was 85.6±14.17 months. The surgical technique used is presented in detail. Results. Male predominance is seen (12 boys, 8 girls) in the study group. The average age at the moment of diagnosis was 7.6±0.88 years. In 13 (65%) cases, an episode of bleeding from varicose veins of the esophagus was the first clinical manifestation. Endoscopic examination revealed II-III degree esophageal varices in 17 (85%) children. All patients had splenomegaly with an average spleen volume of 507.7±67.7 cm3. 8 (40%) patients had severe hypersplenism. The median MPS operational time was 326 (95% CI 300-371) minutes. In 7 (35%) cases shunt thrombosis occured, in 1 (14.2%) case thrombectomy was performed. The overall MPS efficiency of 70% was reached. None of the patients of the follow up group had recurrency of esophageal bleeding. Conclusions. The possibility of performing the MPS depends on favorable anatomy; CT is the optional diagnostic method to detect it, but the role of direct intraoperative visualization and the performance of intraoperative intrahepatic portography remains highly valuable. The 5-year MPS survival rate was 72.2±10.6%. 1 year after surgery, MPS resulted into the involution of the esophageal varices (p<0.001) and a decrease in the volume of the spleen (p<0.001). The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institutions. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.
Portal hypertension is an increase in pressure in the hepatic portal vein system. The most common animal model of prehepatic portal hypertension in use today is partial ligation of the hepatic portal vein in rats but existing studies focus on determining the short-term effects of partial ligation of the hepatic portal vein. Purpose - to evaluate the model of prehepatic portal hypertension by means of a histological study of partial portal vein ligation influence on liver tissue. Materials and methods. Male Wistar rats (n=45), aged 6 weeks and weighing 150±15 grams, were included in the study. The animals were divided into 3 groups: the Group 1 - partial ligation of the portal vein of the liver was performed (formation of stenosis; n=15), the Group 2 - ligation of the portal vein of the liver without its obstruction was performed (pseudo-operated; n=15), the Group 3 - a control group (intact animals, n=15). The rats were withdrawn from the experiment six months after the operation. For histological examination, liver fragments were taken, after standard preparation of the preparations, photographed with an OLYMPUS BX51 light microscope and examined with a PEM-125k electron microscope. The obtained microphotographs were processed and analyzed using the biomedical image processing software ImageJ v.1.50 (National Institutes of Health, USA). Digital data were analyzed in Graphpad Prizm v. 8.3 (Graphpad, USA) statistical package. Results. In rats of the Group 1 the presence of large droplet and total fatty dystrophy of hepatocytes in the center of the lobules was established. The development of connective tissue with the formation of centro-central and porto-central septa was observed. The prolonged effect of partial ligation of the portal vein most closely corresponded to the picture of zonal fatty parenchymal dystrophy of the liver and balloon degeneration of hepatocytes with subsequent fibrosis development. In animals of the Group 1, the distribution of the specific number of hepatocyte nuclei differed from the normal one (p=0.0124), the presence of differences in this indicator between the animals of the three groups was established (p<0.005). Histological examination of the liver of the Group 2 rats revealed preservation of the histological structure of the organ, with moderate changes. Rats of the Group 3 showed normal histoarchitectonics of the organ. Conclusions. The homogeneity of changes in the liver and their reproducibility indicate the stability of the developed model and its suitability for further development of treatment methods. The experiments with laboratory animals were provided in accordance with all bioethical norms and guidelines. No conflict of interests was declared by the authors.
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