ABSTRACT
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.
Subject(s)
Humans , Male , Middle Aged , Lymphatic Vessels/physiopathology , Intestine, Small/physiopathology , Lymphangiectasis, Intestinal/physiopathology , Rare Diseases , Lymphangiectasis, Intestinal/diagnosis , Lymphangiectasis, Intestinal/diet therapyABSTRACT
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.
Subject(s)
Humans , Infant , Male , Lymphangiectasis, Intestinal/diagnosis , Peru/epidemiology , Venezuela/ethnology , Dietary Fats/therapeutic use , Dietary Proteins/therapeutic use , Adrenal Cortex Hormones/therapeutic use , Combined Modality Therapy , Diarrhea/etiology , Diuretics/therapeutic use , Edema/etiology , Hemodynamics , Hypoproteinemia/diet therapy , Hypoproteinemia/etiology , Lymphangiectasis, Intestinal/complications , Lymphangiectasis, Intestinal/therapy , Lymphangiectasis, Intestinal/epidemiologyABSTRACT
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.
Subject(s)
Child , Lymphangioma/surgery , Lymphangioma/diagnosis , Intestinal Neoplasms/surgery , Intestinal Neoplasms/diagnosis , Choristoma , Abdominal Pain/etiology , Lymphangiectasis, Intestinal/surgery , Lymphangiectasis, Intestinal/diagnosis , Magnetic Resonance Imaging , Mesentery , Pancreas/pathology , Tomography, X-Ray ComputedABSTRACT
Infantile systemic hyalinosis [ISH] is a rare autosomal recessive disease. Typically, ISH patients present with progressive painful joint contractures, intractable diarrhea, hyperpigmented skin lesions, and perianal fleshy nodules. We report a case of a 19-month-old male child with atypical ISH presentation. His main clinical finding was protein-losing enteropathy due to intestinal lymphangectasia. This report is intended to enhance awareness about the gastrointestinal tract presentation of ISH
Subject(s)
Humans , Male , Infant , Hyalin/metabolism , Protein-Losing Enteropathies/etiology , Diarrhea/etiology , Diarrhea/diagnosis , Syndrome , Lymphangiectasis, Intestinal/diagnosisABSTRACT
Congenital intestinal lymphangiectasia is a rare protein-losing enteropathy that usually affects children and young adults. Major symptoms include peripheral edema, mild non-bloody diarrhea, and chylous effusions that may develop during the course of the disease. In this disorder intestinal lymphatic vessels show fibrous occlusions that lead to pressure elevation of the lymphatic flow and rupture of the small lymphatic vessels. Transudation of lymph fluid into the different layers of the intestinal wall and lumen then occurs
Subject(s)
Humans , Male , Lymphangiectasis, Intestinal/diagnosis , Lymphangiectasis, Intestinal/complications , Lymphatic Vessels/abnormalities , Tomography, X-Ray ComputedABSTRACT
Chronic diarrhea and steatorrhea occur frequently in patients with autoimmune polyglandular syndrome (APS) type I. Intestinal lymphangiectasia has been reported earlier as a cause of steatorrhea in a young girl with APS Type I. We describe 2 patients with APS Type I who were found to have intestinal lymphangiectasia, one of whom had symptomatic protein-losing enteropathy.
Subject(s)
Adult , Diagnosis, Differential , Fatal Outcome , Female , Humans , Lymphangiectasis, Intestinal/diagnosis , Male , Polyendocrinopathies, Autoimmune/complicationsSubject(s)
Child, Preschool , Diagnosis, Differential , Humans , Lymphangiectasis, Intestinal/diagnosis , MaleABSTRACT
Paciente de sexo femenino quien a los 6 meses presenta diarrea persistente, asociada a edemas y falla en el crecimiento. Los paraclínicos mostraron: hipoalbuminemia, linfopenia, hipocalcemia, sudan (++) en materia fecal y carotinemia baja. La biopsia intestinal reveló linfangiectasia intestinal primaria
Subject(s)
Humans , Female , Infant , Lymphangiectasis, Intestinal/congenital , Lymphangiectasis, Intestinal/diagnosis , Lymphangiectasis, Intestinal/epidemiology , Lymphangiectasis, Intestinal/etiology , Lymphangiectasis, Intestinal/pathology , Lymphangiectasis, Intestinal/physiopathology , Lymphangiectasis, Intestinal/rehabilitation , Lymphangiectasis, Intestinal/therapyABSTRACT
We report a woman with intestinal lymphangiectasia whose symptoms were wrongly attributed to pregnancy; the diagnosis was made in the postpartum period. She also developed alopecia and herpes zoster.
Subject(s)
Adolescent , Alopecia/diagnosis , Biopsy , Diagnostic Errors , Duodenoscopy , Duodenum/pathology , Female , Fetal Death/pathology , Herpes Zoster/diagnosis , Humans , Infant, Newborn , Intestinal Mucosa/pathology , Lymphangiectasis, Intestinal/diagnosis , Lymphatic System/pathology , Pregnancy , Pregnancy Complications/diagnosisABSTRACT
Informamos la observación de una niña de 18 meses con una enteropatía perdedora de proteínas con linfangiectasia intestinal, que presentó intenso edema generalizado, severa anemia, hipoalbuminemia e hipogamaglobulinemia, sin manifestaciones clínicas de malabsorción digestiva. La poca frecuencia de la entidad, la forma atípica de presentación y la excelente evolución lograda motivaron la presente comunicación
Subject(s)
Humans , Female , Infant , Protein-Losing Enteropathies/etiology , Lymphangiectasis, Intestinal/diagnosis , Anemia/complications , Protein-Losing Enteropathies/diagnosis , Protein-Losing Enteropathies/diet therapy , /drug therapy , /therapy , Lymphangiectasis, Intestinal/diet therapy , Lymphangiectasis, Intestinal/pathology , Triglycerides/therapeutic useABSTRACT
Se reportan tres casos de Linfangiectasia Intestinal Primaria que reportaron la triada común de diarrea, edema e hipoalbuminemia, en los cuales pudemos observar las variaciones clínicas que puede presentar esta entidad. Se demostró la importancia de la biopsia intestinal para lograr un diagnóstico preciso, así como, lo indisopensable del uso de los triglicéridos de cadena media con la restricción de las grasas comunes, para recuperar a estos pacientes
Subject(s)
Infant , Child , Humans , Male , Female , Biopsy/statistics & numerical data , Child , Lymphangiectasis, Intestinal/diagnosisABSTRACT
A gastroenteropatia perdedora de proteínas consequente à cirurgia cardíaca de Fontan-Kreutzer é complicaçäo pouco frequente e de difícil diagnóstico. Descreve-se o caso de criança com dez anos e oito meses de idade com cardiopatia congênita complexa operada com essa técnica, que desenvolveu edema por perda de proteíca quatro anos após a cirurgia. Após a exclusäo de patologias renais e hepáticas que justificassem a hipoproteinemia, procedeu-se o diagnóstico etiológico e topográfico da perda proteíca. O diagnóstico endoscópico e histológico nessa patologia é ressaltado
Subject(s)
Humans , Female , Child , Heart Defects, Congenital/surgery , Lymphangiectasis, Intestinal/etiology , Postoperative Complications , Duodenum/pathology , Lymphangiectasis, Intestinal/diagnosis , Lymphangiectasis, Intestinal/pathology , Lymphangiectasis, Intestinal/surgery , Fontan Procedure/adverse effectsABSTRACT
Linfangiectasia intestinal é uma patologia decorrente de bloqueio linfático a qual se caracteriza por dilataçäo cística dos canais linfáticos na lâmina própria da mucosa intestinal que determina distorçäo e aumento de tamanho das vilosidades, sem atrofia. Os autores relatam dois casos de linfangiectasia intestinal primária em duas crianças do sexo feminino com 2 anos e 7 meses e 1 ano e 7 meses de idade, respectivamente. Em ambos os casos as manifestaçöes da doença foram diarréia crônica e edema hipoproteinêmico. A avaliaçäo da funçäo intestinal mostrou malabsorçäo de gorduras nos dois casos e a biópsia de intestino delgado evidenciou dilataçäo cística dos canais linfáticos no córion com distorçäo das vilosidades e preservaçäo do epitélio cilíndrico. Ambas as pacientes tiveram ensaio terapêutico inicial com dieta hipogordurosa adicionada de triglicerídeos de cadeia média sem sucesso, requerendo a instalaçäo de nutriçäo parenteral. A partir daí uma das crianças evoluiu mal, com distúrbios metabólicos graves e incontroláveis e a outra se recuperou, voltando a receber a dieta incial
Subject(s)
Infant , Child, Preschool , Humans , Female , Lymphangiectasis, Intestinal/diagnosis , Parenteral Nutrition , Diarrhea , Edema , Perna , Lymphangiectasis, Intestinal/diet therapy , Lymphangiectasis, Intestinal/physiopathology , Diagnosis, DifferentialABSTRACT
Se presenta un enfermo con linfangiectasia intestinal secundaria a pericarditis constrictiva que consultó por retraso del peso, edema generalizado asimétrico, hipoproteinemia, linfopemia e hipogamaglobulinemia. El diagnóstico se confirmó por la presencia de linfáticos dilatados en las vellosidades intestinales en la biopsia de yeyuno y por el aumento de la excreción fecal de proteínas marcadas con Cr51. La existencia de injurgitación yugular discreta y reflujo hepato yugular positivo insinuaron el diagnóstico de pericarditis constrictiva. Este fue confirmado por la ecocardiografía modo M y bidimensional. Con el tratamiento quirúrgico se logró una rápida remisión clínica, de laboratorio y de la morfología de la mucosa yeyunal