Malformación linfática quística gigante del hígado: dos casos pediátricos con diferente abordaje quirúrgico / Giant cystic lymphatic malformation of the liver: two pediatric cases with different surgical approach

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.

Malformação venolinfática em borda lateral de língua: relato de caso / Venolymphatic malformation in lateral edge of the tongue: case report

Resumo As malformações vasculares são anomalias que podem acometer veias, vasos linfáticos e artérias de forma isolada ou mista. Quando se apresentam de forma mista, com componentes venosos e linfáticos, são denominadas malformação venolinfática ou linfático-venosa, de acordo com sua constituição predominante. Embora seja um distúrbio benigno de bom prognóstico, é localmente invasivo, podendo levar a deformidade e havendo, ainda, a propensão de recorrência local. O presente artigo traz um caso de malformação venolinfática com localização incomum em borda lateral de língua, abordando-se a conduta clínica e o referencial teórico vigente.

Abstract Vascular malformations are vascular anomalies that can affect veins, lymphatic vessels, and/or arteries in isolated or mixed form. When they present in the mixed form with venous and lymphatic involvement, they are called venolymphatic or lymphatic-venous malformations, depending on their predominant component. Although these are benign disorders with good prognosis, they are locally invasive and may lead to deformity, while there is also a propensity for local recurrence. This article presents a case of venolymphatic malformation with unusual localization on the lateral border of the tongue, addressing the clinical conduct and the current theoretical framework.

Técnica EXIT como manejo de la vía aérea en masas gigantes congénitas de cuello / Ex-Utero Intrapartum Treatment for airway management in congenital giant neck masses

Resumen: Introducción: Las masas congénitas de cabeza y cuello se asocian a asfixia perinatal e injuria cerebral con elevada mortalidad. La técnica EXIT (Ex Útero Intrapartum Treatment) consiste en asegurar la vía aérea del neonato, sin interrumpir la oxigenación y perfusión materno-fetal a través del soporte placentario. Esta técnica no ha sido estandarizada en países de medianos ingresos. Objetivo: Describir el caso clínico de 2 neonatos manejados mediante la técnica EXIT. Caso Clínico: Se reportan dos casos, uno con malformación linfática diagnosticada a la semana 20 gestación y el segundo con tiromegalia y polihidramnios diagnosticados a la semana 35 de gestación. En ambos casos, duran te la cesárea se realizó la técnica EXIT con un equipo conformado por neonatólogo, ginecólogo, anestesiólogo, cirujano pediatra, otorrinolaringólogo, enfermero y terapeuta respiratorio. En los dos pacientes se logró asegurar la vía aérea mediante intubación orotraqueal al primer intento. En el caso 1 se confirmó la malformación linfática y recibió escleroterapia, y en el caso 2 se diagnosticó hipotiroidismo congénito asociado a bocio, que fue manejado con levotiroxina. Los pacientes se mantuvieron 7 y 9 días con ventilación mecánica invasiva respectivamente y egresaron sin complicaciones respiratorias. Conclusiones: La técnica EXIT en estos casos fue un procedimiento seguro, llevado a cabo sin inconvenientes. Se necesita un equipo multidisciplinario y la disponibilidad de una unidad de cuidados intensivos neonatales, con el objetivo de reducir potenciales complica ciones y garantizar el manejo postnatal. Para lograr su ejecución, es indispensable el diagnóstico prenatal oportuno.

Abstract: Introduction: Congenital head and neck masses are associated with perinatal asphyxia and brain injury, increasing the risk of death. The EXIT (Ex Utero Intrapartum Treatment) technique con sists of ensuring the newborn's airway while is still receiving placental support. This technique has not been standardized in developing countries. Objective: To describe the clinical outcomes of two infants who underwent the EXIT technique. Clinical Case: We present two cases, one with lymphatic malformation diagnosed at 20 weeks of gestational age (WGE) and the second one, a preterm newborn with thyromegaly and polyhydramnios, diagnosed at 35 WGE. In both cases, during the C-section, the EXIT technique was performed with a team of a neonatologist, a gyne cologist, an anesthesiologist, a pediatric surgeon, an otolaryngologist, a nurse, and a respiratory therapist. In both patients, the neonatologist achieved to secure the airway through orotracheal intubation at the first attempt. In the first case, lymphatic malformation was confirmed and re ceived sclerotherapy, and the second one was diagnosed with congenital hypothyroidism which was managed with levothyroxine. The patients needed invasive mechanical ventilation for 7 and 9 days, respectively, and were discharged without respiratory complications. Conclusions: In these patients, the EXIT technique was a safe procedure, carried out without inconvenience. A multi disciplinary approach and the availability of a neonatal intensive care unit are needed to reduce potential complications and ensure postnatal management. Timely prenatal diagnosis is essential to perform this technique.

Hemangiolinfangioma con extensión abdominal y pélvica. Reporte de caso / Hemangiolymphangioma with abdominal and pelvic extension. Case report

El hemangiolinfangioma es una malformación vascular de rara ocurrencia. Posee componentes mixtos, venosos y linfáticos, y su manejo es limitado en casos de gran extensión. Se presenta el caso de un hombre con alteración vascular de bajo flujo, de predominio linfático, con extensión intrabdominal, retroperitoneal, pélvica y de miembros inferiores.

Hemangiolymphangioma is a vascular malformation of rare occurrence. It has mixed venous and lymphatic components and its management is limited in cases of large extension. We present the case of a man with low-flow vascular alteration, predominantly lymphatic, with intra-abdominal, retroperitoneal, pelvic and lower limb extension.

Left-Sided Cisterna Chyli: a Case Report on a Rare Normal Anatomic Structure

The cisterna chyli, a dilated lymphatic sac in the retrocrural space, is usually located to the right of the aorta. We report a case of a left-sided cisterna chyli, which was incidentally detected on the radiologic examinations of a preoperative workup for cholangiocarcinoma. Computed tomography (CT) and magnetic resonance (MR) images revealed a cisterna chyli measuring 2.5 cm in length in the left retrocrural space. The dilated lumbar lymphatics joined with the cisterna chyli, which was continuous with the left-sided thoracic duct. To the best of our knowledge, this is the second antemortem case of a left-sided cisterna chyli in literature. The cisterna chyli can mimic retrocrural lymphadenopathy, solid tumor with cystic degeneration, abscess or hematoma. The left-sided cisterna chyli should be referred to as a structure so as to be cautious in surgical approach.

Congenital cutaneous pedicled macrocystic lymphatic malformation on the upper extremity: A rare case report and review of the literature

Congenital cystic lymphatic malformations on the extremities are very rare. The patient described in this case study presented with a cutaneous and pedicled macrocystic lymphatic malformation that was eliminated by electrocauterization. A 4-day-old female infant with a congenital cutaneous mass on the dorsal area of her left first metacarpophalangeal joint presented to an outpatient clinic. An electrocautery device was used to cut the pedicle gently with minimal bleeding to avoid mass rupture and to minimize morbidity. A simple wet dressing was applied for 1 week, and the wound subsequently healed completely. Cutaneous macrocystic lymphatic malformations are very rare, especially on the extremities, and no consensus exists on their treatment, which has not been previously described. This report presents this rare case, along with a review of the literature.

Resolution of Protein-Losing Enteropathy after Congenital Heart Disease Repair by Selective Lymphatic Embolization / 대한소아소화기영양학회지

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, post-double switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ≤0.9 g/dL) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.

Experiencia con OK-432 en malformaciones vasculares linfáticas en un hospital del norte de México / Experience with OK-432 in lymphatic vascular malformations in a hospital from northern Mexico

Resumen Introducción: Las malformaciones vasculares linfáticas (MVL), anteriormente llamadas linfangiomas, son malformaciones congénitas que se presentan en uno de cada 6,000 a 16,000 nacimientos. El sistema de clasificación más útil para el manejo del linfangioma se basa en el tamaño de los quistes. La resolución espontánea es infrecuente, por lo que el tratamiento expectante no se recomienda. El tratamiento clásico es la cirugía de escisión, pero puede afectar a estructuras vecinas o haber recidivas, por lo que se empezaron a estudiar sustancias esclerosantes, como el OK-432. La mayoría de los estudios incluyen pocos pacientes; los más grandes realizados en México se enfocan a lesiones específicas (macroquísticas) o únicamente a una región anatómica. Hasta la fecha, no existen estudios del uso de este fármaco en la población del norte de México. Método: Se describe la experiencia con OK-432 mediante un estudio retrospectivo, descriptivo, en los pacientes con MVL, de 2011 a 2016, en un hospital de referencia del norte de México. Resultados: Veintiséis pacientes con MVL recibieron tratamiento con OK-432. La mayoría fueron macroquísticos (69%), microquísticos (19%) y mixtos (12%). Del total de pacientes, 11 presentaron curación total. El 72% de la población estudiada tuvo una reducción de > 50% del tamaño de las lesiones con solo dos aplicaciones de tratamiento; no se presentaron recidivas. Se reportaron complicaciones en dos pacientes (hiperpigmentación de la piel). Conclusiones: El manejo con OK-432 demostró ser efectivo para el tratamiento de las MVL en un hospital de referencia del norte de México.

Abstract Background: Lymphatic vascular malformations (LVM) or formerly called lymphangiomas are congenital malformations present in about 1 out of 6000 to -16000 births. The most relevant classification system for lymphangioma management is based on the size of the cysts. Spontaneous resolution is uncommon; thus, expectant management is not recommended. The classic treatment is excisional surgery, but it can affect adjacent structures or have relapses, so, sclerosing substances like OK-432 are being studied. The majority of the studies are small in number of patients and are from Japan; the largest studies in Mexico are focused on specific lesions (macrocystic) or a determined anatomical region. To date, there are no studies of the population of the north of Mexico. Methods: The experience with OK-432 was described through a retrospective, descriptive study in patients with LVM, from 2011 to 2016, in a reference hospital of northern Mexico. Results: A total of 26 patients with LVM were treated with OK-432. The majority of the lesions were macrocystic (69 %), microcystic (19 %) and mixed (12 %). From the total number of patients, 11 fully healed, and 72 % of the study population had >50 % reduction in lesion size, with only 2 applications. There were no recurrences. Complications were reported in 2 patients who had skin hyperpigmentation. Conclusions: OK-432 probed to be an effective treatment for LVM in a reference hospital in the north of Mexico.

Malformación Vascular Linfática en un Recién Nacido / Vascular Lymphatic Malformation in a Newborn

Las malformaciones vasculares son enfermedades poco frecuentes que representan errores en el desarrollo de los vasos sanguíneos. Las malformaciones vasculares linfáticas (MVL) consisten en lesiones congénitas del sistema linfático compuestas por canales y cavidades de diferente tamaño que resultan en la acumulación de linfa. Las malformaciones macroquísticas se manifiestan como tumoraciones palpables, de consistencia blanda pero no compresible. La localización más frecuente es en la cabeza y el cuello Se presenta el caso de un neonato con una malformación vascular linfática localizada en zona postero-lateral de cuello. Se arribó al diagnóstico en base a la clínica y las características ecográficas. El tratamiento fue muy satisfactorio luego de dos procedimientos de escleroterapias, presentando una excelente evolución

Vascular malformations are rare conditions associated to anomalies in blood vessel development. Lymphatic vascular malformations are congenital lesions consisting of channels and sacs of different size caused by lymph fluid acumulation. Macrocystic vascular malformations present as non-compresible soft tissue masses. They are generally found withinthe head and neck (70%-80%). We present the case of a newborn with a lymphatic vascular malformation in the posterior cervical área. The diagnosis was made on the basis of clinical and ultrasound findings. Sclerotherapy was succesful wihout signs of recurrence

Isolated fetal lymphatic malformation of the thigh: prenatal diagnosis and follow-up

Fetal lymphatic malformation can be found in different parts of the fetal body. It occurs most frequently in the nuchal and axillary region and less frequently in the abdomen or inguinal areas. Lymphatic malformation has been associated with fetal aneuploidy, hydrops fetalis, structural malformations, and intrauterine fetal death. A 31-year-old gravida 3, para 2 woman was admitted to our hospital at 22 weeks of gestation (confirmed by ultrasonographic examination). The fetus was alive, and had a mass derived from the left inguinal region extending to the anterior left leg with fluid-filled cavities about 3-5 cm in size. There was no evidence of intra-abdominal extension of the mass. Amniocentesis was performed. Fetal magnetic resonance imaging revealed a left inguinal cystic mass, which extended to the left thigh. Antenatal follow-up was uneventful. The mother gave birth at term with a cesarean section. Postnatal clinical examination and imaging examination confirmed the diagnosis of lymphatic malformation. Fetal lymphatic malformation carries a high risk of aneuploidy and fetal malformations. Patients diagnosed with lymphatic malformation in antenatal follow-up should be assessed in terms of coexistent anomalies. Fetal karyotyping should be done and the fetus should be monitored for fetal hydrops.

Malformaciones vasculares: actualización en diagnóstico por imágenes y tratamiento / Vascular malformations: an update on imaging and treatment

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.

Cervicofacial Lymphatic Malformations: A Retrospective Review of 40 Cases

BACKGROUND: Lymphatic malformation (LM) is a form of congenital vascular malformation with a low incidence. Although LM has been studied, no consensus has emerged regarding its cause or treatment. METHODS: In this study, we retrospectively evaluated 40 patients who visited our vascular anomalies center for the treatment of cervicofacial LM, which is a common manifestation of LM. The medical records of patients over a period of 12 years were reviewed and analyzed for commonalities regarding the diagnosis and the results of treatment. RESULTS: Suspected cervicofacial LM was confirmed through imaging studies. No difference in incidence was observed according to sex, and 73% of patients first presented with symptoms before the age of two years. The left side and the V2-V3 area were most commonly affected. No significant differences in incidence were observed among the macrocystic, microcystic, and combined types of LM. A total of 28 out of 36 patients received sclerotherapy as the first choice of treatment, regardless of the type of lesion. Complete resolution was achieved in only 25% of patients. CONCLUSIONS: LM is important to confirm the diagnosis early and to choose an appropriate treatment strategy according to the stage of the disease and each individual patient's symptoms. When treatment is delayed or an incorrect treatment is administered, patient discomfort increases as the lesion gradually spreads. Therefore, more so than is the case for most other diseases, a team approach on a case-by-case basis is important for the accurate and appropriate treatment of LM.

Tratamento intralesional de malformações linfáticas com ênfase na escleroterapia com picibanil (OK-432): revisão sistemática / Intralesional treatment of lymphatic malformations with emphasis on Picibanil (OK-432) sclerotherapy: a systematic review

INTRODUÇÃO: Conduziu-se revisão sistemática retrospectiva da literatura incluindo estudos relatando o uso de picibanil para tratar malformações linfáticas (ML). MÉTODOS: A pesquisa foi realizada com estudos publicados no PubMed de janeiro de 1990 a 14 de abril de 2013. Na estratégia de busca, usou-se os descritores "OK-432" ou "Picibanil" e "lymphatic malformation". Os seguintes elementos foram comparados aos de outras modalidades relatadas e, então, compilados: mecanismo de ação, indicações, contraindicações, eficácia, administração, efeitos colaterais, complicações, vantagens e desvantagens. RESULTADOS: Foram encontrados 44 estudos, 27 dos quais atenderam aos critérios de inclusão. O picibanil é uma preparação liofilizada de uma cepa de baixa virulência de Streptococcus pyogenes inativada pela penicilina G. Seu mecanismo de ação ainda não definido claramente, mas especula-se que provoque uma resposta inflamatória controlada com adesão das paredes dos cistos. O picibanil é indicado quase que unanimemente para o tratamento da ML macrocística, cuja resposta é mais efetiva do que em lesões microcísticas ou mistas. Em geral, o picibanil é administrado por meio de punção com visualização direta ou guiada por ultrassonografia, com o paciente sob anestesia geral. A preparação comumente utilizada consiste em 0,1 mg de picibanil em 10 ml de soro fisiológico. Os efeitos colaterais são, em geral, leves; sendo dor, inchaço e febre os mais frequentemente relatados. CONCLUSÃO: Os estudos apresentam pouca evidência científica. A revisão sistemática identificou que o picibanil é útil no tratamento da ML de qualquer tipo, mas tem resultados melhores em lesões macrocísticas. A eficácia foi comparável à de outras terapias. Não foi apresentada nenhuma contraindicação específica. Embora o mecanismo de ação ainda não tenha sido determinado, o picibanil trata-se de opção de tratamento.

INTRODUCTION: We performed a retrospective systematic review of studies reporting the use of Picibanil for treatment of lymphatic malformations (LMs). METHODS: We searched the PubMed database for available studies, including those published between January 1990 and April 14, 2013. The search strategy involved the use of the keywords "OK-432" or "Picibanil" and "lymphatic malformation." Information was compiled regarding the reported mechanism of action, indications, contraindications, efficacy, administration, side effects, complications, and advantages and disadvantages compared to those of other modalities. RESULTS: Forty-four studies were found, of which 27 fulfilled the inclusion criteria. Picibanil is a lyophilized preparation of a low-virulence strain of Streptococcus pyogenes inactivated with penicillin G. Its mechanism of action is unclear, but it has been speculated that it causes a controlled inflammatory response with adhesion of cyst walls. Picibanil is almost unanimously indicated for the treatment of macrocystic LMs, which show a greater effectiveness response compared to that shown by microcystic or mixed LMs. Picibanil is usually administered by puncturing, either with direct visualization or guided by ultrasound, with the patient under general anesthesia. The most widely used preparation comprises 0.1 mg of Picibanil in 10 mL of saline. Side effects are mostly mild, with pain, swelling, and fever being the most frequently reported. CONCLUSION: The studies had low scientific evidence. A systematic review found that Picibanil is useful against any LM, with better results in macrocystic lesions. Efficacy was comparable to that of other therapies. No specific contraindication was presented. Although the mechanism of action has not been established, the inclusion of Picibanil as a treatment option is warranted.

Higroma quístico congénito. Presentación de un caso / Congenital cystid hygroma. A case report

Fundamento: Los linfagiomas son malformaciones congénitas del sistema linfático, se clasifican en quísticos, cavernosos y mixtos o macro y microquísticos. Se caracterizan por espacios dilatados limitados por endotelio, que varían en tamaño desde canales microscópicos hasta grandes quistes. El linfangioma quístico es una anomalía congénita del sistema linfático muy infrecuente. Presentación de caso: Se presenta un caso de higroma quístico en un niño de cinco días de nacido que fue remitido al servicio de pediatría del Hospital General de Benguela, Angola, por presentar una tumoración que interesa desde la región lateral izquierda del cuello y se extiende a la axila del mismo lado. Se analizaron algunos datos embriológicos del sistema linfático y también la localización de los quistes y su frecuencia. Conclusiones: En la medida que casos como este se divulguen entre los profesionales de la salud se puede lograr el rápido diagnóstico de esta enfermedad para el tratamiento y profilaxis de las complicaciones.

Background: The lymphangioma are congenital malformations of the lymphatic system which are classified into: Cystid, cavernous, mixed, macro, microcystid, characterized by dilated spaces that are limitated by the endothelium, which vary in size from the microscopic channels to big cysts. Case Presentation: A case of Cystid hygroma is presented in a boy of 5 days of birth and who was sent to the pediatrician in the General Hospital of Benguela, Angola, having a tumor that interests since the lateral left region of the neck to the armpit of the same part. Were analyzed some embryological data of the lymphatic system and also the place of the cyst and its frequency. Conclusions: As far as cases like this are reported among health professionals, a rapid diagnose can be obtained of this disease for a treatment and prophylaxis of the complications.

Microcystic lymphatic malformations (lymphangioma circumscriptum) managed with fractional carbon dioxide laser ablation

Microcystic lymphatic malformation (lymphangioma circumscriptum) is the most common cutaneous lymphatic malformation, consisting of abnormal, dilated, and tortuous lymphatic vessels in the dermis and subcutaneous tissue. It is due to irregular vessel contraction with subsequent dilatation and fluid build-up that manifests clinically as multiple, grouped, small macroscopic superficial vesicles filled with clear or serosanguineous fluid. The lesions are frequently located over proximal limbs, axillae and chest but may occur on any part of the body. We report a case of lymphangioma circumscriptumin a four-year-old child, partially treated with fractional carbon dioxide laser ablation under local anesthesia. Most of the lesions resolved but the treated areas healed with hypertrophic scarring after one month, which was cosmetically acceptable. Compared to surgical excision, carbon dioxide laser ablation may provide less chances of contractures especially in areas overlying a joint and may be considered as an alternative to more invasive procedures.

The diagnosis and treatment progress of lymphatic malformations of the head and neck in children / 临床耳鼻咽喉头颈外科杂志

Lymphatic malformations including macrocystic and microcystic malformation are congenital benign vascular disease. The incidence in children is much higher than adults and they most often occur in head and neck. There are a variety of diagnosis and treatment methods in lymphatic malformations. This paper reviewed the recent literatures with respect to the pathology, imaging and treatment of the lymphatic malformations in children. And we detailed the advantage and disadvantage of surgical excision, sclerotherapy, laser ablation and drug treatment of lymphatic malformations particularly in children. Surgical excision continues to be integral to treat in many cases when the lesions involve the eye socket or are large enough to affect breathing, but it should not be overused without consideration of the histologic types and extent. In addition, cervicofacial macrocystic lesions tend to sclerotherapy while superficial mucosal microsystic lesions are amenable to laser therapy. In order to obtain the best treatment outcomes, the treatment protocol should be individualized and comprehensive.

Malformación linfática facial mixta: Caso clínico / Mixed facial lymphatic malformation: Case report

Lymphangiomas are a common form of vascular malformation of the lymphatic vessels, mainly in the head and neck region. Most cases are progressive evolution and require a multidisciplinary approach. Currently, the first therapeutic option is sclerotherapy, leaving surgery for the treatment of remaining lesions. Objective: To present a case of facial lymphatic malformation (LM) treated with sclerotherapy, surgery and orthodontics in a 15-year follow up. Case report: A one-year-old female patient who consulted health professionals due to a progressive volume increase of the soft parts of her right cheek. The imaging study confirmed the diagnosis of microcystic lymphatic malformation. It was managed with OK-432 sclerotherapy and Bleomycin. At 2 years of age, the patient response was considered adequate; an intralesional submandibular surgical excision was then performed, with partial resection of the lesion. The biopsy confirmed the diagnosis of microcystic LM. Six months after, a re-resection was planned using the same approach and removing the remaining lesion, with favorable development until the age of 9 years when the patient required surgery and orthodontic management due to intraoral recurrence. No major developments until the age of 13 when a new orthodontic surgery and handling are planned to perform right oral commissure suspension. Conclusion: LM management by sclerotherapy, surgery, and orthodontics has shown the advantages of a multidisciplinary long-term treatment in this case.

El linfangioma corresponde a una malformación vascular de los vasos linfáticos, preferentemente de la región de cabeza y cuello. La mayoría de los casos son de evolución progresiva y requieren un manejo multidisciplinario. Actualmente la primera opción terapéutica es la esclerosis, reservando la cirugía para el tratamiento de las lesiones remanentes. Objetivo: Presentar un caso de malformación linfática (ML) facial, tratado con escleroterapia, cirugía y ortodoncia en un seguimiento a 15 años. Caso clínico: Paciente de sexo femenino que consulta al año de edad por aumento de volumen progresivo de partes blandas en su mejilla derecha. El estudio de imágenes confirmó el diagnóstico de Malformación Linfática microquística. Se manejó con esclerosis seriada con OK-432 y Bleomicina. A los 2 años de edad se consideró que la respuesta era adecuada, y se procedió a realizar extirpación quirúrgica intralesional submandibular, con resección parcial de la lesión. La biopsia confirmó el diagnóstico de ML microquística. Seis meses después se planificó nueva resección utilizando el mismo abordaje y extirpando lesión remanente, con evolución favorable hasta la edad de 9 años en que requiere cirugía y manejo por ortodoncia, por recidiva de lesión a nivel intraoral. Evolución favorable hasta que a la edad de 13 años se planifica nueva cirugía y manejo por ortodoncia para suspender la comisura bucal derecha. Conclusión: El manejo de la ML mediante escleroterapia, cirugía, y ortodoncia muestra en este caso las ventajas de un tratamiento multidisciplinarion a largo plazo.

Aspectos epidemiológicos, clínicos e anatomopatológicos do linfoma em bovinos: 128 casos (1965-2013) / Epidemiological, clinical and pathological aspects of lymphoma in cattle: 128 cases (1965-2013)

Por meio de um estudo retrospectivo, os aspectos epidemiológicos, clínicos e anatomopatológicos de 128 casos de linfoma bovino são descritos. Dos protocolos que informavam o sexo (n=111), 84,7% correspondiam a fêmeas e 15,3% a machos. Dos protocolos em que constava a raça (n=108), a mais prevalente foi a holandesa (63%). Em relação à idade (n=107), houve uma variação entre um e 14 anos. A maioria dos bovinos era adulta (89,7%) e a maior concentração dos casos ocorreu ao redor de 5-8 anos (57,9%). Em relação aos sinais clínicos (n=89), linfadenomegalia foi o achado mais frequentemente observado (74,1%). Outros sinais clínicos, principalmente aqueles relacionados com os sistemas respiratório (dispneia, estertoração pulmonar e taquipneia), cardiovascular (taquicardia, edema subcutâneo e pulso venoso positivo), digestório (atonia ruminal, timpanismo e diarreia) e nervoso (paresia dos membros pélvicos e andar cambaleante), foram pouco prevalentes. Na necropsia (n=125), 71,2% dos bovinos apresentavam aumento de volume dos linfonodos; essa linfadenomegalia foi classificada como localizada em 89,6% dos casos e generalizada em 10,3% dos casos. Dos protocolos que informavam os linfonodos acometidos (n=58), a distribuição foi a seguinte: mesentéricos (51,7%), mediastínicos (37,9%), pré-escapulares (29,3%), ilíacos internos (27,6%), inguinais superficiais (25,8%) e traqueobrônquicos (18,9%). Além dos linfonodos, outros órgãos comumente afetados pelo linfoma neste estudo incluíram: coração (40%), fígado (15,2%), rim (14,4%), abomaso (12,8%), útero (11,2%), intestino (10,4%) e pulmão (7,2%)...

The epidemiological, clinical and pathological aspects of 128 cases of bovine lymphoma are described in this study. Out of the cases were the gender was informed (n=111), 84.7% of affected animals were females and 15.3% were males. Out of the cases were breed was informed (n=108), 63% of affected animals were Holstein cows. The age of affected cows (n=107) varied from 1 to 14 years (57.9%), with most animals being adults (89.7%) with 5 to 8 years of age (57.9%). The most common clinical sign (n=89) was lymphadenomegaly (74.1%). Other clinical signs associated with the respiratory system (dyspnea, pulmonary stridor, and tachypnea), cardiovascular system (tachycardia, subcutaneous edema, and positive venous pulse), digestive system (ruminal atony, bloat, and diarrhea), and nervous system (pelvic limb paresis and staggering pace) were less prevalent. Gross findings (n=125) included enlargement of lymph nodes in 71.2% of the cases; this finding was classified as localized in 89.6% of the cases and generalized in 10.3% of the cases. Out of the cases where the affected lymph nodes were identified (n=58) the distribution of the lymphadenomegaly was the following: mesenteric (51.7%), mediastinal (37.9%), prescapular (29.3%), internal iliac (27.6%), superficial inguinal (25.8%), and tracheobronchial (18.9%). Other commonly affected organs included heart (40%), liver (15.2%), kidney (14.4%), abomasum (12.8%), uterus (11.2%), intestine (10.4%), and lung (7.2%)...