Epidural lipomatosis with foci of hemorrhage and acute compression of the spinal cord in a child with CLOVES syndrome: illustrative case.

BACKGROUND: Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies, and/or scoliosis (CLOVES) syndrome is the most recently described combined vascular anomaly characterized by congenital excessive growth of adipose tissue, vascular malformations, epidermal nevi, and skeletal deformities. This condition exhibits a significant variability in clinical manifestations and a tendency for rapid progression and affects extensive anatomical regions. Information regarding the association of epidural lipomatosis with low-flow venous lymphatic malformations is rare, with few reports in the literature. OBSERVATIONS: The authors present a case of a 6-year-old girl who was admitted to the emergency department complaining of rapidly progressing weakness in her lower extremities and partial loss of sensation in the inguinal area. Radiologically, an extradural mass was identified at the T2-6 level, causing acute spinal cord compression. Urgent decompression and partial resection of the mass were performed. Despite satisfactory intraoperative hemo- and lymphostasis, postoperative lymphorrhea/seroma leakage was encountered as a delayed complication and was managed conservatively. LESSONS: CLOVES syndrome is characterized by the combination of various clinical symptoms, not all of which are included in the abbreviation, as well as a progressively deteriorating course, the emergence of new symptoms, and complications throughout the patient's life. This necessitates ongoing monitoring of such patients.

Warzone pediatric trauma care: Lessons from civilian medical staff in Kyiv.

INTRODUCTION: The article discusses the challenges faced by civilian healthcare providers in Kyiv, Ukraine, during the conflict in treating pediatric trauma resulting from war-related incidents. METHODS: The authors share their experiences and insights from managing a series of 12 pediatric patients admitted to the Ohmatdyt children's hospital between February 25 and April 1, 2022. During this period, the hospital was under constant threat due to the military conflict. RESULTS: The patients, ranging in age from 3 months to 17 years, suffered injuries from various causes, including vehicle shootings, explosions, and other traumatic events. The interventions and timely management are discussed, and two detailed clinical cases are presented to illustrate the complexities of treating pediatric trauma in a warzone. CONCLUSION: In summary, the article sheds light on the unique challenges faced by healthcare providers in a warzone when treating pediatric trauma. It underscores the importance of timely intervention, effective triage, and the utilization of advanced medical techniques to improve patient outcomes in such challenging circumstances.

HEPATIC VASCULAR TUMORS IN CHILDREN: POTENTIAL RISKS, OPTIMAL IMAGING AND THE ROLE OF SURGICAL INTERVENTION.

OBJECTIVE: The aim: To revise the case-series of hepatic vascular tumors (HVT), particularly to identify optimal visualization, management and role of surgical intervention. PATIENTS AND METHODS: Materials and methods: Out of 96 children with hepatic tumors who hospitalized in a single center from 2011 to 2020, 20 (20,8%) were diagnosed HVT. Hepatic Hemangiomas (HHs) were presented in 19 patients and Kaposiform hemangioendotelioma (KHE) in one case. To determine the type of HH we used radiological classification. For visualisation contrast-enhanced MRI (n=7, 30%) and cCT (n=15,70%) were used. Follow-up period was 14-77 months. RESULTS: Results: All HVT were revealed by sonogram at the age of 0-5 m, with 4 (20%) diagnosed prenatally. Male to female ratio was 3:2. Beta-blockers were prescribed to 12 patients with HHs. Treatment duration was from 6 to 24 month. Steroid therapy was initial in cases when it was impossible to prescribe the curative dose of beta-blockers. Complications of propranolol treatment were transitory bradycardia (n=7) and transitory hypoglycemia (n=2). After vincristine chemotherapy decreases the tumor size by 54%, that allowed a safe liver resection. 4 (20%) patients - two multifocal HHs, one diffuse HH and in patient with KHE manifested congestive heart failure and pulmonary hypertension Mortality rate is 5% (n=1), this patient died against progressive cardiovascular failure. CONCLUSION: Conclusions: life-threatening complication of HVT was congestive heart failure. Early treatment is beneficial for complications prevention. Surgical treatment is optional for KHE when can be removed safely.

VASCULAR ANOMALIES IN NEWBORNS: CLINICAL PRESENTATION, COMPLICATIONS, AND PECULIARITIES OF THERAPY.

OBJECTIVE: The aim: To analyze cases of vascular anomalies in newborns retrospectively to study their main clinical signs and to determine indications for urgent treatment. PATIENTS AND METHODS: Materials and methods: A retrospective review of 281 pediatric vascular anomalies diagnosed between 2011 and 2019 was performed. The results of clinical examination, prenatal history, laboratory, sonography, and radiological data were evaluated. RESULTS: Results: Clinical manifestations in the newborn period documented in 170 (60.5%) patients, anomalies were visualized prenatally in 8.9% cases. Five newborns with head and neck LMs required urgent treatment, which amounted 7.9% of all newborns with LMs. The indication for urgent surgery was acute respiratory failure. Partial malformation resection with tracheostomy was performed to those patients, along with intraoperative injection of sclerotherapeutic agents and argon coagulation of residual malformation tissues. EXIT procedure was performed in one case. No correlation (p=0.2) was found between the number of skin lesions and the severity of liver lesions in children with multifocal hepatic hemangiomas, congestive cardiac failure suddenly occurs in one patient in the third week of life. Two newborns with diffuse hepatic hamangioma were manifested right after the birth with clinical signs of liver failure, hypothyreosis, and cardiac failure. Short courses of corticosteroids performed in 7 patients with KHE and Kasabach-Merritte fenomemn for thrombocytopenia correction. CONCLUSION: Conclusions: Clinical signs of vascular anomalies in neonatal period were seen in 57.5% children with vascular anomalies, and only 2.9% of them required treatment. Treatment itself is advantageous only in cases when life-threatening complications develop, such as airways obstruction, cardiac and/or liver failure, thrombocytopenia.

NEWBORNS WITH KASABACH-MERRITT PHENOMENON-ASSOCIATED KAPOSIFORM HEMANGIOENDOTHELIOMA: A REPORT OF 6 CASES.

OBJECTIVE: Combination of kaposiform hemangioendothelioma (KHE) and Kasabach-Merritt phenomenon (KMP) in newborn children is a life-threatening constellation. The purpose of the study is the choice of the diagnostic and treatment methods in these patients and evaluating the effectiveness of treatment using radiological methods of investigation. The study enrolled 6 newborn patients with KHE within a period 2013 - 2018. MRI (CT) performed to make the diagnosis and evaluate treatment response. Hypervascular mass accompanied by reticular lymphedema, hyper intensive in T2 WI; isointensive in T1 WI, intense contrast enhancement, heterogeneous diffusion restriction were unique MRI characteristics of KHE. The sustained remission was achieved with treatment by propranolol (n=2), vincristine (n=1), and their combination (n=3).

Sternal cleft and segmental facial hemangiomas in children with phaces syndrome: effectiveness and safety of conservative management and surgical correction.

OBJECTIVE: The aim is to determine the risk factors of sternal cleft and segmental facial hemangiomas association in children with PHACES syndrome. PATIENTS AND METHODS: Materials and methods: 32 inpatient children with segmental facial hemangiomas and 19 children with sternal cleft were investigated concerning the Metry criteria of PHACES syndrome. RESULTS: Results: In 6 children PHACE syndrome was diagnosed. Patients with bilateral S3 hemangiomas (50%, 3/6) had airway involvement with respiratory disorders. Conservative treatment was propranolol monotherapy (66.7%, 4/6), or combination of prednisolone and propranolol (33.3%, 2/6). Duration of propranolol treatment in children with PHACES syndrome was on an average 24.25 ± 4.49 months exceeding the duration of propranolol therapy in children with isolated soft tissue lesions (p<0.05). Primary surgical treatment of sternal cleft performed in children aged 2 (n=3) and 4 (n=1) months. The later period of surgery associated with the localization of hemangioma in the surgery region. Primary repair of sternal cleft was completed successfully in all cases; partial resection of the thymus made closure easier. CONCLUSION: Conclusions: Primary surgical correction of a sternal cleft performed in young children provides good results. Partial resection of the thymus prevents respiratory and cardiovascular complications. Preoperative propranolol treatment averts the hemorrhagic complications in children with hemangiomas in surgical region.

A Diagnostic Program of Vascular Tumor and Vascular Malformations in Children According to Modern Classification.

The aim of the study was to analyze the cohort of inpatient children with vascular anomalies according to the globally accepted classification introduced by the ISSVA. METHODS: The study included 205 inpatient children within the time period of the years 2010-2015. Types of vascular anomalies (VAs), age of patients, diagnostic procedures, and anatomical localization of VAs were analyzed. RESULTS: 65 patients of first year of life had vascular tumors, with prevalence of infantile hemangiomas (IHs) in 57 (87.7%) patients. 45 children had IHs localized within soft tissues, whereas 7 patients suffered from IHs of the liver, and 5 children from IHs of the respiratory tract. Most patients with soft tissue IHs were diagnosed only with ultrasound; СT or MRI diagnostics were performed on 5 (8.8%) patients, and biopsy was carried out in 2 (4.4%) children. Vascular malformations (VM) were diagnosed in 140 (68.3%) patients. Ultrasound investigation (US) was the screening method. MRI was performed to confirm the diagnosis of low-flow VM, whereas for high-flow VM CT angiography and selective angiography were useful. Venous malformations were diagnosed in 17 (12.1%) patients, and 112 (80.0%) had cystic LM, among them children under the age of 2 years prevailed. Arteriovenous malformations were diagnosed in 5 (3.8%) patients, ages 2-14 years. CONCLUSIONS: Clinical manifestations of vascular anomalies have clear age features. Among hospitalized children vascular tumors add up to 31.7% and VM - up to 68.3%.