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Introducción. Las malformaciones linfáticas quísticas, también llamadas linfangiomas quísticos, aparecen muy raramente de forma aislada en el hígado. Casos clínicos. Se presentan dos pacientes femeninas de edad preescolar con marcada hepatomegalia, dependiente de lesiones quísticas multitabicadas, secundarias a malformación linfática quística gigante del hígado, que fueron tratadas en el Hospital Pediátrico Universitario William Soler, La Habana, Cuba. Resultados. En ambos casos el diagnóstico se apoyó en los estudios de imágenes, la laparoscopia y el análisis histopatológico. En un caso el tratamiento fue la hepatectomía derecha, mientras que en el otro se empleó la escleroterapia, ambas con evolución favorable. Conclusión. A pesar de su rareza, este diagnóstico no debe obviarse ante un paciente pediátrico con lesiones hepáticas quísticas. El tratamiento de elección es la resección quirúrgica, pero su indicación y envergadura debe valorarse de forma individualizada

Introduction. Cystic lymphatic malformations, also called cystic lymphangiomas, are very rarely found in the liver. Clinical cases. Two pediatric female preschool-age patients. presented with hepatomegaly due to multi-septated cystic lesions of the liver, who received treatment at Hospital Pediátrico Universitario William Soler, La Habana, Cuba. Results. We report two pediatric cases with giant cystic lymphatic malformation of the liver. In both cases, the diagnosis were based on imaging, laparoscopy and pathology. In one case the treatment was right hepatectomy, whereas in the other, sclerotherapy was performed, both with a favorable outcome. Conclusion. Despite its rarity, this diagnosis should be considered in pediatric patients with hepatic cystic lesions. The recommended treatment is surgical resection, but its indication and extent should be assessed individually for each patient.

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ABSTRACT It is estimated that lymphatic malformations in children account for 6% of all benign vascular malformations. New medical therapies have been developed for the management of lymphatic orbital disease. The purpose of this article was to describe a clinical case of orbital venolymphatic malformation in a 10-year-old boy, causing proptosis and palpebral edema. The lesion was initially treated with local sclerotherapy. However, the lesion relapsed, and was successfully treated with oral sirolimus. Prospective studies are warranted to determine the appropriate dose and extend the indications of sirolimus in these patients.

RESUMO A incidência de malformações linfáticas em crianças é estimada em 6% de todas as malformações vasculares benignas. Têm sido desenvolvidos novos tratamento para doenças linfáticas orbitárias. Nosso objetivo é descrever um caso clínico de malformação venolinfática orbitária em um menino de 10 anos de idade, causando proptose e edema palpebral. A lesão foi tratada inicialmente com escleroterapia local. No entanto, a lesão teve recidiva e foi tratada com sucesso com sirolimo oral. Ainda são necessários estudos prospectivos para estabelecer a dose apropriada e a duração do tratamento com sirolimo nesses pacientes.

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Introduction. Oral lymphatic malformations are uncommon lesions. This study assessed the clinicopathological and sociodemographic features of oral lymphatic malformations in a Brazilian oral pathology service. Methods. Between 2000 and 2021, all oral lymphatic malformations diagnosed in a single oral pathology service were selected for the study. Clinical data, such as age, sex, site of the lesion, clinical presentation, diagnosis clinical, and the type of biopsy were recorded from the patient's clinical charts. Results. From 7554 oral lesions, 17 (0.22%) were oral lymphatic malformations. This lesion similarly affected both sexes, and the mean age of the patients was 16.5 years. The tongue was the most common site (n = 13; 76%). The mean size of the lesions was 17 mm. Oral lymphatic malformations were asymptomatic for 29% of the patients. Clinically, most lesions appeared as a reddish, sessile nodule, with a pebbly surface. In addition, the clinical and histopathological diagnoses agreed for most lesions (n = 11; 64%). The type of biopsy performed was excisional in 41% of lesions. Conclusions. In summary, oral lymphatic malformations present a low prevalence among the oral lesions diagnosed in an oral pathology service. Moreover, they affect equally both sexes, especially young patients, and usually appear as an asymptomatic or symptomatic nodular swelling on the tongue.

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BACKGROUND: To evaluate the frequency and analyze demographic and clinical characteristics of individuals with a histopathological diagnosis of oral lymphatic malformations (OLMs). METHODS: A multicenter study was performed, collecting biopsy record data from a consortium of Brazilian Oral and Maxillofacial Pathology Centers. A review was also conducted to compare this data with cases already available in the literature. RESULTS: This study retrieved 208 cases of OLM in the multicenter study and 1035 cases in the literature review. In both, OLMs affected male and female individuals equally, with the most affected site being the tongue. Individuals ≥60 years of age were uncommonly affected. Symptomatic and larger lesions were more commonly reported in the literature review. CONCLUSIONS: This study comprises the largest sample of OLMs to date. OLMs are rare conditions, without sex predilection. The elderly proved to be less frequently affected, and the tongue is the most commonly affected site.

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Pulmonary hypertension could have thoracic lymphatic abnormalities caused by right ventricular failure. Since there is no description of such abnormalities, the purpose of this study was to investigate them with magnetic resonance. Prospective review magnetic resonance T2-weighted lymphangiography was performed between January 2017 and October 2019 through quantitative thoracic duct diameter, diameter index and qualitative lymphatic abnormalities types: 1 - little or none abnormalities, 2 - abnormalities in supraclavicular region, 3 - abnormalities extending into the mediastinum and 4 - abnormalities extending into the lung. Five patients with group 1 pulmonary arterial hypertension participated in this study. The mean age was 12.44 ± 4.92 years, three male and two female. The quantitative analysis yielded the following results: mean thoracic duct diameter of 2.92 ± 0.16 mm and thoracic duct index 2.28 ± 1.03 mm/m2. Qualitative lymphangiography abnormalities were type 1 in three patients, type 2 in one, all with low-risk determinants, and type 3 in one with high-risk determinants and right ventricular failure. Magnetic resonance T2-weighted lymphangiography in group 1 paediatric pulmonary arterial hypertension allowed for the identification of the thoracic duct, which was used to perform both quantitative and qualitative analysis of thoracic lymphatic abnormalities, in particular when increased high-risk determinants and right ventricular failure were present. These features represent an extracardiac finding useful to understand systemic venous congestion impact on lymphatic system.

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As malformações linfáticas orais são anomalias vasculares incomuns. Portanto, compreender suas principais características clínicas e demográficas pode auxiliar o entendimento e diagnóstico dessas lesões. A partir de um estudo multicêntrico e revisão de literatura, este trabalho avaliou a ocorrência e analisou as características demográficas e clínicas de malformações linfáticas orais. O estudo multicêntrico foi aprovado pelo Comitê de Ética em Pesquisa da Universidade Federal de Minas Gerais (parecer nº 3.313.870 e CAAE 10723019.0.1001.5149) e replicado para os Comitês de Ética dos demais centros. Foram acessados os arquivos de biópsia de sete laboratórios brasileiros de Patologia Oral e Bucomaxilofacial, compreendendo 228.150 laudos, os quais eram datados entre o ano de inauguração do centro e 2018. Foram selecionados os casos com diagnóstico histopatológico de malformação linfática oral. Adicionalmente, foi realizada uma revisão de literatura sobre o tema. Nesta, a partir de uma estratégia de busca estruturada, foram selecionados os casos de malformação linfática oral reportados em língua inglesa. Posteriormente foram extraídos dados similares aos do estudo multicêntrico, permitindo sua comparação. A análise dos dados foi realizada no programa estatístico SPSS versão 22.0. Para estatísticas analíticas o nível de significância foi estabelecido em p<0,05. No total, o estudo multicêntrico compreendeu 208 casos, representando 0,09% de todo o arquivo investigado, e a revisão de literatura 1035 casos. Em ambos houve concordância de que há distribuição semelhante entre os sexos e a maioria acomete a língua. O estudo multicêntrico e os estudos de relato de casos apresentaram idade mediana de 22 e 10 anos, respetivamente. Já o tamanho das lesões e proporção de pacientes sintomáticos foi maior entre os casos da revisão de literatura. Ao avaliar tais divergências, foi observado que em ambos houve associação estatisticamente significativa (p<0,05) entre idade e tamanho de lesão, em que pacientes mais jovens tiveram lesões maiores. O presente estudo multicêntrico conta com a maior amostra de malformações linfáticas orais até o momento e revelou que este diagnóstico foi incomum em todos os centros participantes. O estudo multicêntrico e a revisão de literatura mostraram que homens e mulheres são afetados igualmente, o sítio preferencial de acometimento é a língua e há relação entre idade do paciente e tamanho da lesão.

Oral lymphatic malformations are uncommon vascular anomalies. Thus, recognizing the main demographical and clinical characteristics of oral lymphatic malformation can help to understand and diagnose these lesions. This multicenter study and literature review assessed the occurrence, clinical and demographic characteristics of oral lymphatic malformation. The multicenter study was approved by the Research Ethics Committee from Universidade Federal de Minas Gerais (CAAE 10723019.0.1001.5149) and replicated to the Ethics Committees of the other centers. The biopsy files from seven Oral and Bucomaxilofacial Pathology Services were acessed. All cases with histopathological diagnosis of lymphatic malformation dated from 1953 to 2018 were selected. Additionally, was performed a literature review, based on a structured search strategy. Were selected cases of oral lymphatic malformation in the English language. Data extracted were similar to those from the multicenter study, allowing their comparison. Data analysis was performed with the software Statistical Package for the Social Sciences (SPSS), version 22.0 (SPSS Inc., Armonk, USA). The level of significance was set at p< 0.05 for analytical statistics. The multicenter study retrieved 208 cases, representing 0.09% of the total sample. The literature review retrieved 1035 cases. Both affected male and female individuals equally and most cases were at tongue. The multicenter study and the case report studies showed median age of 22 and 10 years, respectively. On the other hand, the lesion size and proportion of symptomatic patients were higher among cases from the literature review. Cases, in both multicenter study and the literature review, revealed an association (p<0.05) among age and lesion size, showing young patients with larger lesions. This multicenter study represented the larger sample of oral lymphatic malformations to date and evidenced that this is an uncommon diagnosis in Brazilian Oral and Bucomaxilofacial Pathology Services. The multicenter study and the literature review showed that men and women are affected equally, the main location site is the tongue and patient age is related to the lesion size.

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Introducción: La fístula quilosa posoperatoria debida a lesión iatrogénica del conducto torácico es una complicación infrecuente y grave de la cirugía de cabeza y cuello. Objetivo: Describir las opciones de tratamiento de la fístula quilosa cervical posquirúrgica. Caso clínico: Se presenta un paciente de 18 años de edad con diagnóstico de linfangioma quístico de la región lateral izquierda del cuello, intervenido en el Servicio de Cirugía General del Hospital "Mnazi Mmoja", de Zanzíbar, Tanzania. Durante la evolución posoperatoria se constató una fístula quilosa de bajo débito la cual resolvió mediante tratamiento médico. El enfermo egresó curado a los 28 días después de la intervención quirúrgica. Conclusión: El tratamiento conservador es efectivo en la mayoría de las fístulas quilosas cervicales de bajo débito, mientras que en las de débito alto la cirugía ofrece una rápida resolución, aunque no existe consenso en torno al momento ideal para realizarla(AU)

Introduction: Postoperative chylous fistula due to iatrogenic thoracic duct injury is an infrequent and serious complication of head and neck surgery. Objective: To describe the treatment options of postoperative cervical chylous fistula. Clinical case: It was presented an 18-year-old patient with diagnosis of cystic lymphangioma of the left lateral region of the neck, which was removed in the General Surgery Service of the National Hospital Mnazi Mmoja of Zanzibar, Tanzania. During the postoperative evolution, a low-output chylous fistula was found and resolved by medical treatment. The patient withdrew cured at 28 days after the surgical intervention. Conclusion: Conservative treatment is effective in the majority of low-out put cervical chylous fistulas, while in high-debit, surgery offers a rapid resolution, although there is no consensus about the ideal time to perform it(AU)

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Introduction: Lymphangioma is a rare vascular anomaly that affects the lymphatic vessels. Its etiology is not yet clear and it usually occurs in the head and neck region. This pathology is commonly diagnosed in childhood and there is no consensus about the most effective treatment of it. Case Report: We report a case of lymphangioma located on the dorsum of the tongue in a 9-year-old boy, with the clinical appearance of small transparent vesicles similar to "frog-eggs". The diagnosis was defined according to the clinical aspect and ultrasound images. The treatment performed consisted of two sessions of ablation with a high-power diode laser. Conclusion: High-intensity diode laser ablation was a safe and effective tool for the treatment of this lesion in the tongue, which provided the patient with a faster, efficient transoperative period and a more comfortable postoperative period.

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Vascular malformations comprise a broad and heterogeneous range of lesions that often represent a diagnostic and therapeutic challenge for the pediatrician. For a long time, the use of an inaccurate nomenclature has led to confusion. Since management depends on the specific vascular malformation, a proper classification and identification is critical. The objective of this article is to provide the necessary information about the current classification and terminology of vascular anomalies, including basic concepts about available imaging diagnostic and therapeutic tools for the management of such complex condition.

Las malformaciones vasculares componen un amplio y heterogéneo espectro de lesiones, que frecuentemente se presentan como un desafío diagnóstico y terapéutico para el pediatra. El uso de una nomenclatura inadecuada durante mucho tiempo ha llevado a confusión. Dado que el tratamiento de esta patología depende de cada tipo de malformación vascular, su correcta clasificación e identificación es crucial. El objetivo es brindar la información necesaria sobre la clasificación y denominación actual de las malformaciones vasculares y los conceptos básicos sobre las herramientas disponibles para el diagnóstico y tratamiento de esta compleja patología.

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Las malformaciones vasculares componen un amplio y heterogéneo espectro de lesiones, que frecuentemente se presentan como un desafío diagnóstico y terapéutico para el pediatra. El uso de una nomenclatura inadecuada durante mucho tiempo ha llevado a confusión. Dado que el tratamiento de esta patología depende de cada tipo de malformación vascular, su correcta clasificación e identificación es crucial. El objetivo es brindar la información necesaria sobre la clasificación y denominación actual de las malformaciones vasculares y los conceptos básicos sobre las herramientas disponibles para el diagnóstico y tratamiento de esta compleja patología.

Vascular malformations comprise a broad and heterogeneous range of lesions that often represent a diagnostic and therapeutic challenge for the pediatrician. For a long time, the use of an inaccurate nomenclature has led to confusion. Since management depends on the specific vascular malformation, a proper classification and identification is critical. The objective of this article is to provide the necessary information about the current classification and terminology of vascular anomalies, including basic concepts about available imaging diagnostic and therapeutic tools for the management of such complex condition.

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