Linfangiectasia intestinal primaria en síndrome de Klippel-Trénaunay. Manejo anestésico a propósito de un caso / Primary intestinal lymphagiectasia in Klippel-Trénaunay syndrome. Anesthetic management. Case report

Resumen: El síndrome de Klippel-Trénaunay con linfangiectasia intestinal primaria asociada es una entidad de aparición poco frecuente en pediatría. Se presenta con edemas por hipoproteinemia y, en algunos casos, con ascitis. El objetivo es presentar un caso clínico cuyo diagnóstico fue certificado mediante endoscopía intestinal y biopsia. Debido a la ascitis refractaria, el tratamiento fue quirúrgico y se realizó derivación peritoneo venosa. El manejo anestésico de este tipo de pacientes se ha descrito en informes limitados, por lo que en el presente caso hacemos énfasis en la terapia de soporte con perfusión de albúmina como tratamiento sintomático propuesto para pacientes con drenaje masivo de ascitis.

Abstract: Klippel-Trénaunay syndrome with associated primary intestinal lymphangiectasia is a rare entity in pediatrics. It presents with edema due to hypoproteinemia, and in some cases with ascites. The objective is to present a clinical case whose diagnosis was certified by intestinal esdoscopy and biopsy. For refractary ascities, the treatment was surgical, performing peritoneal venous bypass. The anesthetic management of this type of patients has been described in limited reports, so in the present case we emphasize supportive therapy with albumin infusion as the proposed symptomatic treatment for patients with massive drainage of ascities.

Linfangiectasia intestinal primaria: una enfermedad rara como causa de enteropatía pierde-proteínas / Primary intestinal lymphangiectasia: a rare disease as a cause of protein-losing enteropathy

La linfangiectasia intestinal primaria es un trastorno raro asociado a una enteropatía pierde-proteínas. Las principales manifestaciones son las derivadas de la hipoalbuminemia. Para lograr el diagnóstico se necesita la imagen endoscópica típica de la linfangiectasia intestinal y el aumento de las cifras de alfa-1-antitripsina en las heces de 24 horas. El tratamiento es básicamente dietético. (AU)

Linfangiectasia intestinal en un paciente afectado de síndrome de Sanfilippo B / Intestinal lymphangiectasia in a patient with Sanfilippo B syndrome

La mucopolisacaridosis tipo III B es una enfermedad de depósito lisosomal causada por la deficiencia de la enzima N-acetil-alfa-d-glucosaminidasa, implicada en el catabolismo del heparán sulfato, que produce su acúmulo en diversos tejidos. Se presenta a un paciente de 8 años, afectado de mucopolisacaridosis tipo III B, con historia de diarrea crónica y hallazgos endoscópicos e histológicos compatibles con linfangiectasia intestinal. Tras tratamiento dietético con restricción de ácidos grasos de cadena larga y rica en triglicéridos de cadena media, presentó mejoría clínica, mantenida hasta la actualidad.La patogenia de la diarrea crónica en pacientes con mucopolisacaridosis tipo III B es aún desconocida. Debe investigarse la presencia de linfangiectasia intestinal en estos pacientes e iniciar, en caso de confirmarse, un tratamiento dietético adecuado para mejorar así su calidad de vida.

Mucopolysaccharidosis type IIIB is a lysosomal storage disease caused by a deficiency of the N-acetyl-alpha-d-glucosaminidase enzyme involved in the catabolism of heparan sulfate, causing its accumulation in various tissues. We present an 8-year-old patient with mucopolysaccharidosis type IIIB, with a history of chronic diarrhea and endoscopic and histological findings compatible with intestinal lymphangiectasia. After a dietary treatment with a low-fat diet supplemented with medium-chain triglyceride, our patient presents clinical improvement until today. The pathogenesis of chronic diarrhea in patients with mucopolysaccharidosis type IIIB is still unknown. The presence of intestinal lymphangiectasia in these patients should be investigated, and appropriate dietary treatment should be initiated, if confirmed, to improve their quality of life.

Ultrasound and endoscopic findings in dogs with lymphangiectasia / [Conclusões ultrassonográfica e endoscópica em cães com linfangiectasia ]

Lymphangiectasia is a heterogenous inflammatory bowel disease characterized by lymphatic vessel dilation, chronic diarrhea and protein loss such as serum albumin and globulin. The most common cause of lymphangiectasia is considered to be the congenital malformation of the lymphatics. The study was conducted between 2012-2015 on 76 dogs suffering from intestinal disorders and manifesting digestive symptoms such as diarrhea or weight loss. In order to assess the origin of disorder, physical examination, biochemistry profile, ultrasound and endoscopic examinations were performed. Ultrasound examination tried to assess the changes of intestines' echogenicity, changes in wall thickness, wall layering and presence of striations or / and speckles (hyperechoic structures along intestinal mucosal layer). Endoscopic examination findings included dilated lacteals (59.2%) and erythema (21.1%). Although increased friability was observed in 33 dogs, it was not considered in the study due to limitations represented by the evaluation of the endoscopic images only. The study proved that an extremely significant statistical correlation exists between the presence of speckles and dilated lacteals in dogs with lymphangiectasia (P<0.05). Up to now, there is no other study to make an association between the white spots observed in ultrasound examination and dilated lacteals revealed after endoscopy in dogs with intestinal lymphangiectasia.(AU)

A linfangiectasia é uma doença inflamatória intestinal heterogênea, caracterizada por dilatação dos vasos linfáticos, diarreia crônica e perda de proteínas, como albumina sérica e globulina. A causa mais comum de linfangiectasia é considerada a malformação congênita dos linfáticos. O presente estudo foi realizado entre 2012 e 2015, em 76 cães que sofrem de distúrbios intestinais e manifestam sintomas digestivos, como diarreia ou perda de peso. Para avaliar a origem do distúrbio, foram realizados exame físico, perfil bioquímico, ultrassonográfico e endoscópico. O exame ultrassonográfico tentou avaliar as alterações da ecogenicidade do intestino, as alterações na espessura da parede, a estratificação e a presença de estrias e / ou de manchas (estruturas hiperecoicas ao longo da camada mucosa intestinal). Os resultados do exame endoscópico incluíram lacteais dilatadas (59,2%) e eritema (21,1%). Embora tenha sido observada maior friabilidade em 33 cães, ela não foi considerada no estudo devido às limitações representadas pela avaliação apenas das imagens endoscópicas. O estudo demonstrou que existe uma correlação estatística extremamente significativa entre a presença de manchas e lacteais dilatadas em cães com linfangiectasia (P <0,05). Até o momento, não há outro estudo para associar as manchas brancas observadas no exame ultrassonográfico e lacteais dilatadas reveladas após endoscopia em cães com linfangiectasia intestinal.(AU)

Linfangiectasia intestinal em adulto: relato de caso / Intestinal lymphangiectasia in an adult: case report

Resumo A linfangiectasia intestinal consiste em um grupo de doenças raras caracterizadas pela dilatação dos canais linfáticos. A fisiopatologia compreende a obstrução da drenagem linfática do intestino delgado com dilatação secundária dos vasos linfáticos mucosos, submucosos ou subserosos, que distorcem a arquitetura das vilosidades e conduzem à perda de linfa para a luz intestinal, levando à má absorção. Os vasos linfáticos afetados localizam-se primariamente no intestino delgado, que é atingido em extensão variável. A sua etiologia é ainda desconhecida. O relato a seguir apresenta um raro caso de linfangiectasia intestinal em paciente adulto.

Abstract Intestinal lymphangiectasia is a group of rare diseases characterized by dilation of lymphatic channels. Its pathophysiology comprises obstruction of small bowel lymphatic drainage with secondary dilation of mucosal, submucosal, or subserous lymphatic vessels, distorting villous architecture and causing loss of lymph into the intestinal lumen, leading to malabsorption. The affected lymphatic vessels are primarily located in the small intestine, which is affected to a varying extent. Its etiology is still unknown. The following report presents a rare case of intestinal lymphangiectasia in an adult patient.

Linfangiectasia renal bilateral / Bilateral renal lymphangiectasia

Introducción: La linfangiectasia renal es una afección infrecuente en la que existe dilatación de los vasos linfáticos renales. Usualmente es bilateral y de buen pronóstico. Objetivos: Presentar un caso de linfangiectasia renal bilateral, sus características clínicas y aspectos del diagnóstico imaginológico. Caso clínico: Paciente femenina de 59 años de edad, hipertensa controlada, que asistió a la consulta externa de urología por dolor lumbar bilateral, de moderada intensidad y coloración rojiza de la orina, ambos de forma intermitente. El examen físico general y regional fue negativo. Se confirmó microhematuria en el examen general de la orina. Los estudios de la analítica sanguínea fueron normales. El ultrasonido renal reportó pielocaliectasia bilateral moderada y quistes parapiélicos, bilaterales, a predominio izquierdo. La tomografía axial computarizada renal contrastada, reveló la presencia de formaciones hipodensas renales bilaterales, de aspecto quístico parapiélicas y en el seno renal, compatibles con linfangiectasia bilateral. La paciente ha evolucionado favorablemente. Conclusiones: La linfangiectasia renal se debe tener en cuenta en el diagnóstico deferencial de la enfermedad quística renal. Para el diagnóstico imaginológico de certeza es esencial la tomografía axial computarizada renal contrastada(AU)

Introduction: The renal linfangiectasia is an uncommon, in which dilatation of the lymphatic renal vessels exists. It is usually bilateral and of good presage. Objectives: To present a case of renal bilateral lymphangiectasia, their clinical characteristics and aspects of the imaginologic diagnosis. Clinical case: Patient feminine of 59 years of age, with controlled hypertension that attended the external consultation of Urology for lumbar bilateral pain, of moderate intensity, and reddish coloration of the urine, both in an intermittent way. The general and regional physical exam was negative. Microhematuria was confirmed in the general exam of the urine. The sanguine studies of the analytic one were all normal ones. The renal ultrasound reported bilateral moderate pielocaliectasia and parapielic cysts, with left prevalence. The renal contrasted computed tomography revealed the presence of bilateral renal hipodenses formations, of aspect cystic parapielic and in the renal sinus, and compatible with bilateral linfangiectasia. The patient has evolved favorably. Conclusions: The renal linfangiectasia it should be kept in mind in the deferential diagnosis of the cystic renal illness. For the imaginologic diagnostic of certainty it is essential the renal contrasted computed tomography(AU)

Linfangiectasia intestinal primaria: Ascitis quilosa como forma de presentación / Primary intestinal lymphangiectasia: Chylous ascites as a presentation form

Presentamos el caso de lactante de 6 meses de edad referido por aumento progresivo de perímetro abdominal. A la valoración con datos de ascitis. La paracentesis diagnóstica muestra líquido ascítico quiloso. El diagnóstico de linfangiectasia se realizó a través de endoscopia digestiva alta, con biopsia intestinal

We present the case of 6-month-old infants referred for ascites. The paracentesis shows uid of chilosas characteristics. The diagnosis of lymphangiectasia was made through upper digestive endoscopy, with intestinal biopsy

Linfangiectasia intestinal primaria: primer reporte de caso en Perú / Primary intestinal lymphangiectasia: first case report in Peru

La linfangiectasia intestinal primaria es una entidad clínica poco común de etiología desconocida. La edad típica de presentación de esta enfermedad es durante los 3 primeros años de vida, pero también se han reportado casos en adultos. Posee sintomatología variable, pero la manifestación clínica principal es el edema, puede presentarse también diarrea y pérdida de peso. La pérdida de fluido linfático en el tracto gastointestinal conlleva también a hipoproteinemia y linfopenia. El diagnóstico se establece en base a la clínica, a los estudios de laboratorio, al estudio endoscópico y se confirma con la evaluación histológica de la biopsia realizada. El manejo se da mediante una dieta rica en proteínas, baja en grasas y triglicéridos de cadena media. A continuación, se presenta el caso de un paciente varón de 1 año de edad que presenta edema generalizado, con predominio de miembros inferiores, y diarrea. Los exámenes de laboratorio muestran la presencia de hipoproteinemia marcada. Posteriormente, se realiza una endoscopía digestiva alta y una biopsia duodenal. El estudio histológico confirma el diagnóstico de linfangiectasia intestinal primaria. El paciente recibe el tratamiento establecido para esta enfermedad y, finalmente es dado de alta.

Primary intestinal lymphangiectasia is a rare clinical condition of unknown etiology. The common age of presentation is during the first 3 years of life, but cases in adults have also been reported. It has a variable symptomatology, but the main clinical manifestation is edema, also diarrhea and weight loss can occur. The loss of lymph fluid into the gastrointestinal tract also leads to hypoproteinemia and lymphopenia. Diagnosis is based on clinical manifestations, laboratory and endoscopic findings, and is confirmed on histopathological examination of biopsy. The main treatment is a protein rich, low in fat and medium chain triglyceride diet. We present the case of a 1-year-old male patient who presents with generalized edema, predominantly in lower limbs, and diarrhea. Laboratory findings show the presence of marked hypoproteinemia. Then an endoscopy and a duodenal biopsy are performed, and the histopathological study confirms the diagnosis of primary intestinal lymphangiectasia. The patient is treated and after a satisfactory evolution, is discharged.

Linfangiectasia pulmonar congénita unilateral en un neonato prematuro / Unilateral congenital pulmonary lymphangiectasia in a preterm infant

La linfangiectasia pulmonar congénita (LPC) unilateral es una enfermedad extremadamente rara de los vasos linfáticos pulmonares. OBJETIVO: presentar un caso de LPC en un recién nacido prematuro. CASO CLÍNICO: recién nacido masculino, prematuro, con insuficiencia respiratoria severa a las 2 horas de vida extrauterina, recibió tratamiento con surfactante exógeno, catecolaminas y ventilación de alta frecuencia oscilatoria (VAFO). La tomografía axial computarizada (TAC) de tórax reveló bulas y atrapamiento de aire de pulmón izquierdo, el estudio histopatológico describió dilatación quística de los canales linfáticos broncoalveolares. Se diagnosticó LPC unilateral secundaria. La evolución clínica hasta los 19 meses de edad fue normal y la TAC de tórax mostró escasas bulas enfisematosas. CONCLUSIONES: La LPC debe ser uno de los diagnósticos diferenciales en neonatos con dificultad respiratoria inexplicable. El pronóstico dependerá del tipo de LPC y de la afectación pulmonar.

Unilateral congenital pulmonary lymphangiectasia (CPL) is an extremely rare disease of the pulmo nary lymphatic vessels. OBJECTIVE: to present a case of CPL in a premature newborn. CLINICAL CASE: premature male newborn with severe respiratory failure at 2 hours of extrauterine life was treated with exogenous surfactant, catecholamines and high frequency oscillatory ventilation (HFOV). Chest computed tomography (CT) scan showed bullae and air trapping of the left lung; the histopathological study showed cystic dilation of the bronchoalveolar lymphatic channels. The diagnosis of secondary unilateral CPL was made. The clinical course up to 19 months of age was normal and the chest CT scan showed few emphysematous bullae. CONCLUSIONS: CPL must be one of the differential diagnoses in neonates with unexplained respiratory distress. The prognosis will depend on the type of CPL and lung involvement.

Linfangiectasia intestinal primaria o enfermedad de Waldmann / Primary Intestinal Lymphangiectasia or Waldmann Disease

La linfangiectasia intestinal primaria en una patología infrecuente causada por la malformación de los conductos linfáticos intestinales. Normalmente se diagnostica antes de los 3 años de edad, pero puede aparecer en población adulta. Los síntomas más frecuentemente encontrados son la presencia de anasarca y dolor abdominal acompañado de malnutrición. El tratamiento es sintomático y se basa en la sustitución dietética de los triglicéridos de cadena larga por triglicéridos de cadena mediana con aumento del aporte proteico. En este trabajo se reporta el caso de una paciente femenina de 22 años de edad la cual presenta manifestación clínicas, imaginológicas y anatomopatológicas que permiten realizar el diagnóstico de una linfangiectasia intestinal primaria, caso extremadamente infrecuente y sobre todo a esta edad(AU)

Primary intestinal lymphangiectasia in an uncommon pathology caused by malformation of the intestinal lymphatic ducts. It is usually diagnosed before 3 years of age, but may appear in the adult population. The most frequent symptoms are the presence of anasarca and abdominal pain accompanied by malnutrition. The treatment is symptomatic and is based on the dietary substitution of long chain triglycerides by medium chain triglycerides with increased protein intake. This paper reports the case of a female patient of 22 years of age who presents clinical, imaging and anatomopathological manifestations that allow the diagnosis of primary intestinal lymphangiectasia, an extremely rare case, especially at this age(AU)

Behçet disease and protein-losing enteropathy due to intestinal lymphangiectasia.

We report an unusual case of a patient with Behçet's disease that developed protein-losing enteropathy due to intestinal lymphangiectasia.

[Primary intestinal lymphangiectasia: twenty years of experience at a Mexican tertiary care hospital]. / Linfangiectasia intestinal primaria: 20 años de experiencia en el Instituto Nacional de Pediatría.

BACKGROUND: Primary intestinal lymphangiectasia is a rare congenital disease described by Waldmann in 1961 that is a consequence of obstruction of the lymphatic drainage of the small bowel with secondary lymph vessel dilation. This distorts the architecture of the villi and causes a leakage of lymph into the intestinal lumen, resulting in protein-losing enteropathy and malabsorption. AIM: To describe the clinical, biochemical, radiologic, endoscopic, and histologic characteristics in children with primary intestinal lymphangiectasia. METHOD: A retrospective observational, descriptive, cross-sectional study was conducted that reviewed the case records of children diagnosed with primary intestinal lymphangiectasia that were seen at the Department of Gastroenterology and Nutrition of the Instituto Nacional de Pediatría within the time frame of January 1, 1992 to September 30, 2012. RESULTS: Four patients were found that presented with primary intestinal lymphangiectasia. Three of them had been diagnosed before 3 years of age. All the patients presented with chronic diarrhea, edema, lymphopenia, hypocalcemia, and hypogammaglobulinemia, and 3 patients presented with hypocholesterolemia. Bowel transit time, endoscopy, and intestinal biopsies were characteristic of this pathology. CONCLUSIONS: Intestinal lymphangiectasia should be suspected when there is a clinical picture of chronic diarrhea and protein-losing enteropathy accompanied with edema at any level, as well as hypoalbuminemia, hypocalcemia, lymphopenia, hypogammaglobulinemia, and hypocholesterolemia, which are the main biochemical findings of this pathology. All children presenting with intestinal lymphangiectasia should undergo an upper gastrointestinal series with bowel transit time and endoscopy with biopsies taken at the level of the duodenum. Treatment includes diet and the periodic administration of albumin and gamma globulin.

Linfangiectasia intestinal: Reporte de un caso / A case report of Intestinal Lymphangiectasia

A propósito del caso de un paciente de 7 meses de vida remitido desde Yopal a la ciudad de Bogotá, se revisa el tema de la linfagiectasia intestinal. Esta es una rara enfermedad que involucra los vasos linfáticos intestinales, y origina hipoproteinemia, edemas, ascitis y enteropatía perdedora de proteínas.

This is the case report of a 7 month old child from Yopal with intestinal lymphangiectasia who was sent to Bogota. We also review the issue of intestinal lymphangiectasia, a rare disease involving intestinal lymphatic vessels which caused hypoproteinemia, edema, ascites and protein-losing enteropathy.

Linfangiectasia intestinal asociada a hemihipertrofia: Reporte de caso / Intestinal Lymphangiectasia associated with Hemihypertrophy: A case report

La linfangiectasia intestinal primaria es una enfermedad caracterizada por dilatación de los vasos linfáticos del intestino, manifestándose como una enteropatía perdedora de proteínas. Presentamos un paciente con diarrea crónica, prolapso rectal recurrente y hemihipertrofia de miembro superior izquierdo, asociado a linfopenia e hipoalbuminemia. Por endoscopia digestiva superior y biopsia se diagnostica linfangiectasia intestinal primaria y se inicia tratamiento nutricional exitosamente.

Primary intestinal lymphangiectasia is a disease characterized by dilated intestinal lymph vessels which manifests as a protein losing enteropathy. We present a patient with chronic diarrhea, recurrent rectal prolapse and left upper limb hemihypertrophy associated with lymphopenia and hypoalbuminemia. Primary intestinal lymphangiectasia was diagnosed with upper endoscopy and biopsy. Nutritional treatment was successfully begun.

Linfangiectasia renal: achado incidental em tomografia computadorizada multicorte e revisão da literatura / Renal lymphangiectasia: incidental finding at multislice computed tomography and literature review

Linfangiectasia renal é uma condição rara, caracterizada por coleções parapiélicas e perirrenais, que pode progredir de assintomática para insuficiência renal crônica. É apresentado um caso de achado incidental em tomografia computadorizada de linfangiectasia renal bilateral em paciente assintomático, com descrição dos principais achados à luz dos métodos de imagem e ampla revisão da literatura.

Renal lymphangiectasia is a rare condition characterized by parapyelic and perirenal fluid collections, which may progress from asymptomatic condition to chronic renal failure. The present report describes a case of incidental computed tomography finding of bilateral lymphangiectasia in an asymptomatic patient, as well as the main imaging findings with a comprehensive literature review.

Malformación linfangiomatosa intestinal con tejido pancreático ectópico: a propósito de un caso y revisión de la literatura / Intestinal lymphangiomatosis with ectopic pancreatic tissue: a case report and literature review

Intestinal lymphangiomatosis is a rare malformation typically affecting children. Its etiology is unknown, although several hypotheses have been proposed. We present a case of intestinal lymphangiomatosis with ectopic pancreatic tissue of the mesentery, an association not previously described in literature. We emphasize the fact that this entity has sometimes been associated with other intra-abdominal malformations, even conditions undescribed in scientific literature. It must be considered the first differential diagnosis for pediatric patients with radiological evidence of mesenteric cystic lesions, subsequently confirmed by pathological examination.

La malformación linfangiomatosa intestinal es una entidad poco frecuente, típica de la edad pediátrica, cuya etiopatogenia es desconocida, aunque se barajan varias hipótesis. Presentamos un caso de linfangiomatosis intestinal con tejido pancreático ectópico mesentérico, una asociación no descrita anteriormente en la literatura. Destacamos la importancia de conocer que esta entidad, en ocasiones, se asocia a otro tipo de malformaciones intrabdominales, incluso no descritas previamente en la literatura. Se debe plantear como el primer diagnóstico diferencial durante la edad pediátrica, ante el hallazgo radiológico de lesiones quísticas mesentéricas, confirmando posteriormente mediante un estudio anatomopatológico de las lesiones.

Doença celíaca associada à tireoidite de Hashimoto e síndrome de Noonan / Celiac disease associated with Hashimoto's thyroiditis and Noonan syndrome

OBJETIVO: Relatar o caso clínico de uma criança portadora de doença celíaca, tireoidite de Hashimoto e síndrome de Noonan. DESCRIÇÃO DE CASO: Menina de dez anos e seis meses, branca, apresentando história de diarreia líquida há cinco meses e "aumento da barriga". Ao exame, mostrava peso de 20.580g (p<3), estatura de 114cm (p<3), hidratada, descorada 2+/4+ e consciente. Presença de fácies triangular, com hipertelorismo ocular aparente, posição antimongoloide das fendas palpebrais, orelhas em abano de baixa implantação, micrognatia, pescoço curto e pectus excavatum. O abdome mostrava-se globoso, flácido, indolor, com hérnia umbilical, fígado a 2cm do rebordo costal direito, linfedema em membro superior direito e edema de membros inferiores. Nos exames subsidiários, havia anemia microcítica e hipocrômica, déficit de proteínas totais, tireoidite de Hashimoto e atraso de cinco anos na idade óssea. Na ultrassonografia abdominal, as alças intestinais estavam levemente dilatadas. Devido ao linfedema e à diarreia crônica, a hipótese inicial foi de linfangiectasia intestinal, confirmada pela biópsia jejunal, que ainda mostrou padrão compatível de doença celíaca. O cariótipo foi 46XX com diagnóstico clínico de síndrome de Noonan. COMENTÁRIOS: As doenças autoimunes se associam; no caso apresentado, a doença celíaca se associou à tireoidite de Hashimoto, possivelmente pela presença de antígenos do sistema HLA. Já a associação de doença celíaca à síndrome de Noonan é muito rara, sendo este o terceiro relato na literatura.

OBJECTIVE: To describe the clinical case of a child with celiac disease, Hashimoto's thyroiditis and Noonan syndrome. CASE DESCRIPTION: A Caucasian girl aged ten years and six months had liquid diarrhea for five months, and a "distended belly". At the physical exam: weight of 20,580g (p<3), length of 114cm (p<3), hydrated, anemic 2+/4+ and conscious. The patient presented triangular facies, apparent ocular hypertelorism, antimongoloid position of the palpebral fissures, ears with low implantation, micrognathia, short neck and pectus excavatum. The abdomen was globular, flaccid and painless; the liver was 2cm below the right costal margin. Lymphedema in right upper limb and lower limb edema was also noted. Laboratory exams showed microcytic and hypochromic anemia, deficit of total proteins, Hashimoto's thyroiditis and a 5-year delay in bone age. Abdominal ultrasonography showed the bowel slightly dilated. Due to lymphedema and chronic diarrhea, the initial hypothesis was intestinal lymphangiectasis, which was confirmed by a jejunal biopsy, which also showed celiac disease. The genetic evaluation revealed a 46XX karyotype and a clinical diagnosis of Noonan syndrome. COMMENTS: Different autoimmune diseases can be associated. In this case, the celiac disease and the Hashimoto's thyroiditis are possibly related to the presence of HLA system antigens. However, the association of the celiac disease with the Noonan syndrome is very rare, and this is the third report in the literature.

Manejo nutricional e digestibilidade no quilotórax canino / Nutritional therapy and digestibility in canine chylotorax

O quilotórax corresponde ao acúmulo de fluido linfático em um ou em ambos os espaços pleurais, resultante do extravasamento de linfa do ducto torácico ou de uma de suas divisões principais. Este pode estar associado à ascite quilosa em pacientes com distúrbios linfáticos sistêmicos, como na linfangiectasia, em decorrência de defeitos no transporte linfático-venoso do quilo da cavidade torácica para a circulação sistêmica. A linfangiectasia intestinal é um distúrbio obstrutivo que envolve o sistema linfático do órgão e revela-se como a causa mais comum de enteropatias associadas à má absorção e perda de proteínas em cães. São apresentados no presente relato o controle clínico com o uso de dieta caseira com baixa gordura e a quantificação da função digestiva, por meio da determinação da digestibilidade do alimento, em um cão acometido por quilotórax secundário à linfangiectasia intestinal. A dieta caseira incluiu arroz, peito de frango, cenoura, carbonato de cálcio, levedura de cerveja, suplemento vitamínico e mineral e sal. O ensaio de digestibilidade foi conduzido pelo método de coleta total de fezes. A dieta caseira foi eficaz em interromper o acúmulo de efusão quilosa torácica, restabelecer as concentrações de proteína total e albumina e promover ganho de peso no paciente. Os resultados da digestibilidade demonstraram menor aproveitamento de todos os nutrientes no cão com linfangiectasia em relação ao controle sadio.

Chylothorax corresponds to accumulation of lymphatic fluid in one or both pleural spaces, resulting from the leak of thoracic duct or of one of their main divisions. This can be associated to chylous ascites in patients with systemic lymphatic disturbances, as in lymphangiectasia, due to defects in the lymphatic-veined transport of chylo from thoracic cavity to systemic circulation. Intestinal lymphangiectasia is an obstructive disturbance that involves lymphatic system of gastrintestinal tract and it’s the more common cause of enteropathies associated to reduced-absorption and protein loss in dogs. A clinical management of a dog assaulted by chylothorax secondary to intestinal lymphangiectasia is described. A homemade low fat diet was used, and the quantification of digestive function, through the determination of diet’s digestibility, was performed. The homemade diet included rice, chicken chest, carrot, calcium carbonate, yeast, vitamin and mineral supplement and salt. The digestibility assay was carried out by total feces collection method. Homemade diet was efficient in stop recurrence of chylous thoracic effusion accumulation, recovery of serum concentrations of total protein and albumin, and promotes weight gain. Compared to the healthy control dog, animal with lymphangiectasia showed reduced digestibility of all analyzed nutrients.