Vascular anomalies (VA) comprise a heterogeneous group of diseases associated with congenital angiogenesis disorder. There are no currently developed unified protocols and treatment regimens for systemic forms of VA. Numerous advantages show sirolimus, an mTOR inhibitor, as a well tolerated and effective antiproliferative and antiangiogenic therapy in patients with VA. The article presents the results of treatment of 211 patients with VA (6 patients with vascular tumors and 205 patients with vascular malformations) aged 2 months to 17 years (median – 9 years), who received sirolimus therapy for 1–86 months (median – 24 months). Sirolimus was administered at a starting dose of 0.8 mg/m2/day orally in two doses with an interval of 12 hours. The concentration of the blood preparation was maintained in the therapeutic range of 6–15 ng/ml. Since 2015, concomitant therapy with co-trimoxazole for the prevention of Pneumocystis pneumonia has been prescribed only to tracheostomy carriers. When infectious episodes occurred in patients, sirolimus therapy continued without changes in the dose of the drug and did not affect the disease, provided that the therapeutic concentration was maintained. A positive response to therapy was observed in 89.1 % of patients with VA in the form of the size of the vascular mass according to the data of visual examination and instrumental control. All patients showeda clinical response to therapy in the form of relief of painsyndrome, reduction/relief of lymphorrhea, reduction/improvement of hemostasis parameters, and an increase in functional activity and quality. For the entire observation period 2012–2020, when taking sirolimus, not a single severe adverse event occurring in post-transplant patients has been reported that would require discontinuation of the drug. The article presents two clinical cases of sirolimus use in the treatment of patients with kaposiform hemangioendothelioma and extensive venous malformation. Parents are encouraged to use the information in scientific research and publications.
Introduction. Congenital portosystemic shunt (SPSS) is a congenital anomaly of the portal system. Blood from the portal system, bypassing the hepatic bloodstream, enters the systemic venous bloodflow. Clinical manifestations are not specific. SPSS can be suspected in patients with arterial hypoxemia and intrapulmonary vascular dilatation, in patients with foci of nodular hyperplasia in the liver, with carbohydrate metabolism disorders in the form of hyperinsulinemia and hypoglycemia, hepatic encephalopathy, hyperammoniemia. Material and methods. Nine patients ( five boys and four girls) , aged 7.7 ± 5.2, were treated in our clinic. All patients had hyperammonemia before surgery 124.5 ± 24.7 μmol / L. Five patients had endovascular SPSS occlusion. Four patients had open SPSS ligation. Results. An immediate technical success was achieved in all patients. There were no complications after endovascular treatment. There was one relapse which required a repeated endovascular occlusion. After endovascular occlusion, the length of hospital stay was shorter, postoperative period was much easier; there were no serious complications compared with the open SPSS ligation. Blood ammonia returned to normal levels in eight patients. In one patient who had multiple shunts and incomplete occlusion, blood ammonia level decreased by twice, up to 82 μmol / L. Conclusion. If SPSS is revealed, a surgical intervention is recommended to separate the portal and systemic venous blood flow. In the surgical treatment for SPSS, X-ray surgical endovascular occlusion is more preferable. If this technique was not possible, surgeons performed open surgical intervention.
The article discusses results of surgical treatment of 25 children, aged 1 to 14, with fibro-adipose vascular anomaly (FAVA) of lower extremities. The patients were treated in Pirogov Russian Pediatric Clinical Hospital in 2015-2019. Diagnostic findings are presented; basic features of the discussed pathology are described. As the obtained results have shown, in the authors’ opinion, the discussed surgical approach turned to be quite effective, since it significantly improves the quality of life, and in some cases brings complete disappearance of complaints and symptoms in patients with FAVA.
Congenital porto-caval shunts are rare and may have a different morphological structure (intra- and extrahepatic shunts, with or without portal blood flow). The main method of treating patients with this pathology is endovascular shunt occlusion. However, in some cases, this method is ineffective. The article contains a description of six clinical examples of surgical treatment of congenital porto-systemic shunts in children. In the diagnosis of congenital portosystemic shunts, the leading role belongs to Doppler ultrasound, multislice computed tomography, and angiography. The indication for surgical treatment was the anatomical features of the shunt, which makes endovascular occlusion technically impossible. In one observation a wide Arantian duct was diagnosed, its open ligation was performed. In another case, the portal vein emptied directly into an aneurysmal dilatation, performed reconstructive plastic surgery on the vessels of the portal vein. In the next observation, a pronounced retrograde blood flow was determined along the dilated inferior mesenteric vein, blood was discharged through the sacral plexus into the internal iliac vein. The left internal iliac vein was isolated and ligated, the dysplastic inferior mesenteric vein was ligated and partially removed. In 2 patients, the portal vein flowed directly into the inferior vena cava in the area of aneurysmal expansion; an operation was performed - open ligation of the shunt. In one observation, a deep hypoplasia of the intrahepatic branches of the portal vein was diagnosed, and therefore the restoration of portal blood flow after the closure of the shunt is impossible. The child was sent to decide on a liver transplant. Conclusion. Each case of congenital porto-caval shunts is unique. The surgeon determines the tactics directly during the operation, depending on the morphological structure of the organs, since the preoperative examination does not always give an unambiguous idea.
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