Management and prognosis of infantile hepatic hemangioendothelioma-arteriovenous fistula complicated with heart failure in neonates / 中国新生儿科杂志

Objective:To study the clinical features and prognosis of infantile hepatic hemangioendothelioma-arteriovenous fistula (IHHE-AVF) complicated with heart failure in neonates.Method:From May 2016 to June 2020, neonates with IHHE-AVF complicated with heart failure admitted were retrospectively studied. The clinical presentation, treatment and outcomes were analyzed.Result:A total of 11 cases of IHHE-AVF complicated with heart failure were enrolled (male 5, female 6). The onset age of heart failure was 12.0 (0.0, 17.0) d. 6 cases showed IHHE on fetal ultrasound. All patients had significantly enlarged heart on chest X-ray. All patients had decreased left ventricular systolic function and pulmonary hypertension on echocardiography. All patients required respiratory support and 6 of them were intubated. 3 cases received conservative treatment (all dead). 1 case received surgery (dead). 7 cases received interventional therapy at the age of (25.6±18.5) d. 1 case was dead, and the other 6 cases were improved and discharged. All the 6 cases were followed up to 3~18 months. None of them had heart failure again. The IHHE were shrunk or completely disappeared. Coagulation function and platelet count were normal.Conclusion:The fatality rate of neonatal-onset IHHE-AVF complicated with heart failure is extremely high. Interventional therapy may be more effective than conservative therapy and surgery.

Hemangioendotelioma hepático infantil: presentación de un caso clínico / Infantile hepatic hemangioendothelioma: a clinical case

El hemangioendotelioma hepático del lactante es una patología rara y más aún que su forma de presentación tenga complicaciones graves y el paciente fallezca. Se presenta el caso clínico de una lactante menor de 3 meses de edad que ingresó al Servicio Desconcentrado Hospital Pediátrico Dr. Agustín Zubillaga con diagnóstico de intoxicación herbácea (hierbabuena) e íleo metabólico. La hepatomegalia fue el signo más llamativo en la exploración clínica asociado a valores elevados de alfafetoproteína sérica. Los hallazgos ecosonográficos y tomográficos reportaron múltiples lesiones redondeadas que impresionaron metástasis hepáticas. La paciente fallece a los 23 días de iniciada la enfermedad actual siendo el diagnóstico postmortem hemangioendotelioma cavernoso gigante hepático, Se concluye que en general el diagnóstico de esta enfermedad se puede plantear sin una confirmación histológica, particularmente en lactantes menores, cuando los hallazgos clínicos, analíticos e imagenológicos son muy sugestivos aun cuando hay excepciones como lo presentado en este caso clínico(AU)

Infantile hemangioendothelioma is a rare, usually benign, disease. We present a case of a 3-month-old infant who was admitted in the Servicio Desconcentrado Hospital Pediátrico Dr. Agustín Zubillaga with the diagnosis of herbaceous poisoning (peppermint) and metabolic ileus. Hepatomegaly was the most characteristic sign in clinical examination and was associated with elevated levels of alpha-fetoprotein. Echographic and tomographic findings revealed multiple rounded lesions that impressed liver metastases. The patient died 23 days after admission and the postmortem diagnosis showed giant hepatic cavernous hemangioendothelioma. Diagnosis of hemangioendothelioma usually can be done without histopathological confirmation, especially in infants, when clinical, laboratory and imaging features are very suggestive of this disease, however there are exceptions as the one presented in this case(AU)

Infantile hepatic hemangioendothelioma with heart failure as the main manifestation: a report of 4 cases and literature review / 中华实用儿科临床杂志

Objective To summarize the clinical features,treatment and prognosis of infantile hepatic hemangioendothelioma(IHHE) combined with congestive heart failure(CHF) in children.Methods A retrospective analysis was made with the clinical data from 4 children with IHHE combined with CHF in Department of Pediatric Cardiology,Affiliated Guangzhou Women and Children's Medical Center of Guangzhou Medical University from May 2013 to December 2015.Results Among 4 infants,there were 3 male and 1 female,mean aged of 109 days (21-219 days),the average weight of 4 350 g (2 750-6 500 g),and 1 case was admitted because of abdominal swelling and respiratory distress,1 case of abdominal swelling,jaundice,shortness of breath admission,1 case of fever and shortness of breath admission,and 1 case of cyanosis and shortness of breath admission.Two patients were associated with hemangioma and Kasabach-Merritt syndrome.Three cases had multiple liver hemangioma scattered in the left lobe and right lobe of the liver,and 1 case had solitary liver hemangioma scattered in the right lobe.Tumor size diameters were ranged from 2-10 mm.Four children had been used Prednisone [1-2 mg/(kg · d)] and Digoxin,Dopamine,Furosemide,Spironolactone.Two cases responsed well to the therapy and were followed up for 2 years or 1 year and 6 months respectively,whose heart size,heart function and pulmonary hypertension became normal.One case with Kasabach-Merritt syndrome responsed poorly to the therapy.Two cases experienced transcatheter hepatic arteriovenous fistula embolization by using coil occlusion of hepatic artery-venous fistula.The postoperative hepatic tumor volume was significantly decreased,and the heart function was improved.One patient was followed up for 1 year and 6 months,and the heart size,pulmonary hypertension and heart function returned to normal.One case was still under follow-up.One case with Kasabach-Merritt syndrome died from heart failure and severe infection before surgery.The mortality rate was 25％ (1/4 cases).Conclusions IHHE is a rare cause of CHF in children.If heart failure is found unexplained in children,abdominal ultrasound or CT examination should be done immediatey in order to confirm the presence or absence of IHHE.The mortality rate in IHHE associated with CHF is very high.Early identification,early intervention,especially Prednisone combined with transcatheter hepatic arteriovenous fistula embolization can significantly improve heart function,reduce the mortality and improve the prognosis of IHHE associated with CHF in children.

Hemangiomatosis hepática en lactantes: diagnóstico y evolución. “Hospital J. M. de los Ríos”. Caracas - Venezuela / Hemangiomatosis liver in infants: diagnosis and evolution

Las lesiones vasculares hepáticas en niños son raras pero no infrecuentes en gastroenterología pediátrica. Los hemangiomas son los tumores hepáticos vasculares más frecuentes en la infancia, la mayoría de curso benigno, algunos, incluyendo el hemangioendotelioma infantil, tienen potencial maligno. La clínica predominante es hepatomegalia, dolor abdominal, hemangiomas cutáneos e insuficiencia cardíaca congestiva; y menos frecuente esplenomegalia, ictericia, ascitis, hemorragia digestiva y anemia. Se presentan 5 lactantes entre 1 y 4 meses con diagnóstico de hemangiomatosis hepática; en tres de ellos su diagnóstico fue incidental a través de ecografía, uno presentó aumento de volumen abdominal progresivo y otro hepatomegalia; tres presentaron hemangiomas en piel. Todos cursaron con anemia. Se realizó ecografía describiéndose hepatomegalia, con múltiples imágenes redondeadas, hipoecoicas, de diferentes tamaños, en ambos lóbulos hepáticos; Tomografía axial computada abdominal: hepatomegalia con compromiso de ambos lóbulos, ocupados por áreas nodulares hipodensas. Fueron evaluados por los servicios de endocrinología, cardiología, gastroenterología y cirugía pediátrica. En uno se realizó biopsia hepática. Recibieron tratamiento con prednisona 3 - 4 mg/kg/día con descenso progresivo de la misma, seguimiento clínico y ecográfico durante 1 año. Cuatro pacientes respondieron al tratamiento, evidenciándose disminución e incluso desaparición en tres pacientes de los hemangiomas, y uno no respondió, asociándose propanolol.

Hepatic vascular lesions in children are rare but not uncommon in pediatric gastroenterology. Hemangiomas are the most common vascular liver tumors in childhood, most benign course, some, including infantile hemangioendothelioma, have malignant potential. The clinical manifestations are hepatomegaly, abdominal pain, cutaneous hemangiomas and congestive heart failure and less frequent splenomegaly, jaundice, ascites, gastrointestinal bleeding and anemia. We present five infants between 1 and 4 months with a diagnosis of hepatic hemangiomatosis, in three of them the diagnosis was made incidentally by ultrasonography, showed a progressive increase in abdominal volume and a hepatomegaly, three had skin hemangiomas. All of them presented with anemia. Hepatomegaly describing ultrasound was performed, with multiple images rounded, hypoechoic, of different sizes in both lobes, abdominal computed tomography: hepatomegaly with involvement of both lobes, occupied by hypodense nodular areas. Services were assessed by endocrinology, cardiology, gastroenterology and pediatric surgery. In one a liver biopsy was performed. Treated with prednisone 3-4 mg / kg / day with gradual decrease of the same, clinical and ultrasound for 1 year. Four patients responded to treatment, demonstrating decreased or even disappeared in three patients with hemangiomas, and one did not respond, associating propranolol.

The Experience of Non-surgical Treatment for Infantile Hepatic Hemangioendothelioma

PURPOSE: Hepatic hemangioendothelioma (HET) is a rare benign vascular tumor in infants. The clinical manifestations are variable from asymptomatic to life-threatening heart failure or Kasabach-Merritt syndrome (KM SD). We report our non-surgical treatment experience for infantile HET. METHODS: We analyzed 15 infants (5 males, 10 females) of HET retrospectively, from January 1989 to December 2007. RESULTS: All except 2 were full-term babies and median birth weight was 3,140 g. The median age at diagnosis was 15 days of life (0~157 days). Three of 5 patients diagnosed prenatally presented heart failure symptoms and the remaining 2 showed KM SD. Among 6 neonates, 3 showed cyanosis due to congestive heart failure. Nine patients showed multiple nodules at both liver lobes. Three asymptomatic patients were followed with radiologic examinations, thus HETs regressed spontaneously in two and decreased in one. Four cases that showed hepatomegaly or increase in size of subcutaneous hemangioma, were treated with prednisolone. All patients improved from tumor related symptoms and tumor size decreased. Four patients (2 patients of heart failure, 1 with rapid progressive hepatomegaly and 1 with congenital heart disease) received interferon-alpha. Among them, 3 showed regression of tumor and the remaining 1 showed decrease of tumor size. Two patients who suffered from severe heart failure received prednisolone and interferon-alpha, and then recovered. In the 2 patients with KM SD, we tried hepatic artery embolization with medical treatment. One survived but the other is not followed after failure of embolization. CONCLUSION: We experienced that many patients who had clinical symptoms associated with HET very early in their life treated with an aggressive nonsurgical treatment in symptomatic patients showed favorable outcome.

A Congenital Giant Hepatic Hemangioendothelioma Treated with Interferon-alpha and Complete Tumor Resection

Infantile hepatic hemangioendotheliomas (IHHEs) are benign vascular tumors, but can be associated with the life-threatening complications, such as congestive heart failure, disseminated intravascular coagulation, and massive bleeding. Various therapeutic options have been developed and the treatment response depends on the patient's clinical status and the nature of the lesion. In the case of a symptomatic IHHE, a non-invasive and precise diagnosis should be performed promptly before the therapeutic method is chosen. Additionally, it should be kept in mind that the residual lesions have malignant potential. We report a case of a congenital giant IHHE that was successfully reduced in size by interferon-alpha and completely removed by surgical tumor resection with a hepatic lobectomy.