RESUMEN
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.
Asunto(s)
Insuficiencia Cardíaca , Hemangioma , Neoplasias Hepáticas , Neoplasias Vasculares , Niño , Femenino , Hemangioma/diagnóstico por imagen , Hemangioma/cirugía , Humanos , Neoplasias Hepáticas/diagnóstico por imagen , Neoplasias Hepáticas/cirugía , Masculino , Estudios Retrospectivos , Neoplasias Vasculares/complicacionesRESUMEN
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.
Asunto(s)
Hemangioma , Neoplasias Hepáticas , Enfermedades Vasculares , Niño , Femenino , Hemangioma/terapia , Humanos , Recién Nacido , Cuello , Embarazo , Estudios RetrospectivosRESUMEN
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).
Asunto(s)
Hemangioendotelioma , Síndrome de Kasabach-Merritt , Sarcoma de Kaposi , Hemangioendotelioma/complicaciones , Hemangioendotelioma/diagnóstico por imagen , Hemangioendotelioma/tratamiento farmacológico , Humanos , Recién Nacido , Síndrome de Kasabach-Merritt/complicaciones , Síndrome de Kasabach-Merritt/tratamiento farmacológico , Imagen por Resonancia Magnética , Sarcoma de Kaposi/complicaciones , Sarcoma de Kaposi/tratamiento farmacológicoRESUMEN
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.