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      Hemangiomatosis hepática en lactantes: diagnóstico y evolución. “hospital j. m. de los ríos”. caracas - venezuela

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          Abstract

          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.

          Translated abstract

          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.

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          Hemangiomas of infancy.

          Hemangiomas of infancy are unique, benign, pediatric tumors of endothelial cells characterized by an initial phase of rapid proliferation, followed by slow involution, often leading to complete regression. Although most of these tumors are small and innocuous, some may be may be life- or function-threatening, or have associated structural congenital anomalies. Uncertainties regarding their diagnosis or management often prompt referral to a dermatologist. The pathogenesis of hemangiomas of infancy is not well understood, but recent findings suggest a unique vascular phenotype with dysregulated vascular homeostasis. This article reviews new information regarding the pathogenesis of these tumors and highlights the more worrisome presentations, including syndromic hemangiomas, that are likely to be problematic. In addition, management strategies and treatment options are discussed. (J Am Acad Dermatol 2003;48:477-93.) At the completion of this learning activity, participants should be able to describe the clinical features of hemangiomas of infancy and potential complications as well as to understand the strengths and limitations of various treatment options.
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            Management of alarming hemangiomas in infancy: a review of 25 cases.

            During the past 10 years, 25 infants with alarming hemangiomas--lesions that impaired important functions and were life threatening, especially when there was visceral involvement--have been treated. A vascular mark was present at birth in 68% of these infants. Visceral hemangiomas were associated with bulky cervicocephalic hemangiomas or with small hemangiomas scattered over the body. Among the 25 infants, 12 had laryngeal hemangiomas, 3 had hepatic hemangiomas, and 1 had gastrointestinal hemangiomatosis. Ocular sequelae, malocclusion, and cutaneous distortion were the most important functional problems. Corticosteroid treatment was used for 23 of 25 infants with alarming hemangiomas. There was a varied treatment response: total failure (30% of the patients); excellent, dramatic, rapid improvement (30% of the patients); and moderate, doubtful response, with the natural course of the disease remaining unaltered (40% of the infants). Arterial embolization, used in 6 infants, gave inconstant results. Cardiac failure, frequently associated with large cutaneous hemangiomas and always seen with hepatic multinodular hemangiomas, required digitalization. In some cases arterial embolization reduced the increased cardiac output. Liver hemangiomas had a high mortality; all 3 infants with hepatic involvement died.
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              Hemangiomas hepáticos

              Presentamos nuestra experiencia de los últimos 6 años con hemangiomas hepáticos gigantes con el objeto de discutir aspectos diagnósticos, indicaciones terapéuticas y aspectos técnicos. La serie está compuesta por 7 pacientes con edades entre 43 y 70 años. El motivo de consulta o referencia fue dolor en 3, hallazgo ecotomográfico en 2, crecimiento y síndrome de Kasabach Merrit en 1, y hallazgo durante colecistectomía en otro. El diagnóstico fue establecido en todos los casos por la concordancia de las imágenes de la ecotomografía y el TAC abdominal. Cinco pacientes presentaron hemangiomas hepáticos únicos, uno tuvo una lesión doble y otro tenía tres lesiones. La localización más frecuente de los hemangiomas únicos fue el lóbulo izquierdo. El tamaño de las lesiones varió entre 6 y 25 cm de diámetro. Cuatro pacientes fueron operados. Tres por presentar dolor local habiéndose descartado otras causas que generaran dicha sintomatología y otro por evidente crecimiento. Se practicó enucleación de los hemangiomas con control vascular selectivo de las aferencia según la técnica de Takasaki. Tres pacientes considerados asintomáticos han sido seguidos clínica e imagenológicamente durante 6, 4 y 3 años sin que hayan manifestado complicaciones ni crecimiento. Los 4 resecados correspondieron a hemangiomas cavernosos sin evidencias de malignidad
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                Author and article information

                Contributors
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                Journal
                gen
                Gen
                Gen
                Sociedad Venezolana de Gastroentereología (Caracas )
                0016-3503
                January 2011
                : 65
                : 1
                : 38-41
                Article
                S0016-35032011000100009
                2822ddc0-61bd-4110-b4d2-b4f82846a610

                http://creativecommons.org/licenses/by/4.0/

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                SciELO Venezuela

                Self URI (journal page): http://www.scielo.org.ve/scielo.php?script=sci_serial&pid=0016-3503&lng=en

                Hepatic hemangiomatosis,Infantile hepatic hemangioendothelioma,Liver tumors,Hemangiomatosis hepática,Hemangioendotelioma hepático infantil,Tumores hepáticos

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