The work is devoted to improving the treatment results of abdominal and retroperitoneal vascular anomalies in children by studying their clinical, radiological, morphological features; improvement of diagnostic methods and implementation of minimally invasive technologies in surgical treatment.
Abdominal and retroperitoneal vascular anomalies (VA) are a rare congenital pathology, characterized by variety of clinical manifestations, difficult to diagnose, can significantly affect the quality of life of the child, and in some cases pose a direct threat to it. According to various data, the frequency of abdominal and retroperitoneal VA is from or less than 10% of patients with VA of all localizations. Mortality from VA complications in pediatric patients, according to various authors, ranges from 1.5% to 53.8%. The inaccuracy is due to the rarity of the pathology as such, the absence of large clinical groups of patients in studies. The lack of opportunity to conduct controlled randomized or large-scale prospective cohort studies in such patients determines the low level of evidence of the available studies. In the study, vascular anomalies were represented by cystic abdominal and retroperitoneal LMs and benign vascular tumors of the liver.The following are used for the treatment of this pathology: surgical, including with the use of minimally invasive technologies, sclerotherapy, systemic drug therapy.
The complexity of diagnosis and differential diagnosis, a diverse clinical course, the lack of a single view on the choice of treatment tactics in the World, unstable results of treatment, the impossibility of assessing the risks of recurrence, issues regarding the choice of treatment methods for abdominal and retroperitoneal VA, and their sequence, determining the time of treatment initiation, ensuring a long-term result, prevention of complications, especially in young children, remain open questions and determine the relevance of the chosen research topic. New aspects regarding the pathogenesis, clinical manifestations, differential diagnosis, treatment and monitoring of VA in children, presented in the literature sources of the last 15 years, provide an opportunity to strengthen the validity of the approach to the treatment of children with abdominal and retroperitoneal VA. VA in children deserves special attention due to the possible deterioration of the clinical course over time, both in the case of no treatment and in the case of choosing the wrong treatment method.
A single-center retrospective cohort study of a group of 64 patients with abdominal and retroperitoneal VA who were treated at the base of departments of the National Children's Specialized Hospital "Okhmatdit" in Kyiv during 2011- 2021, was conducted. The median follow-up was 38 (8-73) months.
Abdominal and retroperitoneal LMs were diagnosed in 43 patients, which accounted for 17.9% of LM of all localizations (43/240). In the group, there was an unreliable (p=0.196) gender predominance of male patients (n=25, 58.1%) over female patients (n=18, 41.9%), a ratio of 1:0.72, which correlates with the data presented in literary sources. The age at the time of diagnosis in the group ranged from 1 month to 17 years, the median age of the patients was 4 (95%CI 2-5) years.
Primary imaging was carried out with the help of ultrasound for all patients, in the future, most children underwent MRI (n=28; 65.1%), in urgent cases CT was performed (n=7; 16.2%), which in 5 (35.7%) cases generally did not provide an opportunity to establish the exact localization of LM and the degree of affection of the intestine and mesentery.
The largest localization was represented by intestinal LMs, with local isolated or combined lesions of the mesentery (n=16, 32.7%). Retroperitoneal LMs (n=12, 27.9%) became the second most frequent, in (n=3, 6.9%) cases - with a connection with the cisterna chyli. Omental LMs were diagnosed in (n=7; 16.3%) patients, total mesentery affection in (n=2, 4.6%). According to the literature data, in the abdominal cavity LMs are most often localized in the intestinal mesentery, and make
up from 24% to 80%, retroperitoneal LMs account for 12-14% of LMs. According to the results of the comparison, no difference was found in the localization of LMs in the intestine, mesentery, and omentum (p=0.091 and p=0.213, respectively), since these localizations are among the most widespread according to the data of scientific literature. Significant difference was revealed in the proportion of LMs of the retroperitoneal space (р=0.010) is explained by the heterogeneity of patients in the groups of the analytical review of the literature, and in the proportion of patients with cystic LMs of the spleen and LMs with a connection with the cisterna chyli ((р<0.001) in both cases) - concentration of patients with LM in the center, which is profiled for VA in children.
