Evaluation of the clinical value of capsule endoscopy in patients with unexplained abdominal pain / 西安交通大学学报(医学版)

【Objective】 To evaluate the clinical value of capsule endoscope in the diagnosis of unexplained abdominal pain. 【Methods】 We made a retrospective analysis of 191 patients with unexplained abdominal pain who sought medical help in our hospital and 25 normal controls. Capsule endoscopy was performed in both groups, small bowel lesions were detected, and clinical data were collected for further analysis. 【Results】 The total small bowel lesion detection rate was 52.87% (101/191) in abdominal pain (AP) patients and 20% (5/25) in the control group, respectively. The detection rate of significant findings (ulcers, erosions, polyps, diverticula, parasites, and neoplastic organisms) was only 16.23% (31/191) in AP patients. In the non-significant findings, no statistical difference in the detection rates for vascular malformation, capillary dilation, and lymphoid follicular hyperplasia were found between the two groups, while the detection rate of intestinal lymphangiectasia was significantly higher in the AP patients (23.56% vs. 4%, P<0.05, OR=7.089). 【Conclusion】 Capsule endoscopy can be an optional choice for diagnosis of unexplained abdominal pain, while the relationship between positive findings and abdominal pain should be further investigated.

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)

360° Circumferential Hemorrhagic Lymphangiectasia of the Conjunctivae

PURPOSE: We report a case of hemorrhagic lymphangiectasia of the conjunctiva with a 360° connected circumference, which recovered spontaneously.CASE SUMMARY: A 44-year-old female patient presented with congestion of the right eye 1 day prior to her visit. There was no history of any systemic disease or trauma, but she had experienced relapses of the same episode three times before the visit. There were no accompanying symptoms such as decreased vision or pain. Blood analysis, orbital computed tomography, and angiographic findings showed no remarkable finding. Slit lamp examination showed circumferential lymphatic dilatation extending 360° under the conjunctiva of the eye at a distance of about 6 mm behind the limbus of the right eye, which was accompanied by intralymphatic bleeding. Irregular local lymphatic dilatations were observed on the bulbar conjunctiva at 4 and 8 o'clock of the left eye. The bleeding spontaneously resolved in about 2 weeks, but the translucent enlarged lymphatic vessels were still observed on slit lamp examination and anterior segment optical coherence tomography.CONCLUSIONS: This is the first reported case of hemorrhagic lymphangiectasia involving 360° of the bulbar conjunctiva in Republic of Korea.

Intestinal lymphangiectasia: case report

Primary intestinal lymphangiectasia (PIL) was first described by Waldmann et al, in 1961. PIL is a rare disease with several hundred reported cases. It is rarely reported in adults because it is presumably a congenital disorder and when present in adults it typically produces a long duration of manifestation such as diarrhea, abdominal distention from ascites, and peripheral edema. This disorder is characterized by markedly dilated intestinal lymphatics, hypoproteinemia, generalized edema, lymphocytopenia hypogammaglobinemia, and immunologic anomalies. The loss of protein into the from dilated intestinal lymphatics leads to the development of hypoproteinemia in these patients and its demonstration is important in the diagnosis of intestinal lymphangiectasia. The disease can be secondary to congenital, secondary or idiopathic defects in the formation of the lymphatic ducts. In the present report, we describe a case of 15 years old female presented to our hospital with history of generalized edema, bilateral hand spasm, and diarrhea. Endoscopy of the patient revealed White spots (dilated lacteals), white nodules, and submucosal elevations were observed. Changes suggestive of the disease includes White villi and/or spots (dilated lacteals), white nodules, and submucosal elevations are observed. Xanthomata’s plaques are often visualized, there are no specific treatments for patients with PIL. treatment of patients with primary intestinal lymphangiectasia involves control of symptoms with the use of dietary, pharmaceutical, and behavioral modifications.

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

Hennekam Syndrome: A Case Report

Hennekam syndrome is a rare autosomal recessive disorder resulting from malformation of the lymphatic system. The characteristic signs of Hennekam syndrome are lymphangiectasia, lymph edema, facial anomalies, and mental retardation. This is a case in which a patient presented with left-arm lymphedema, facial-feature anomalies, and multiple organ lymphangiectasia consistent with symptoms of Hennekam syndrome. There is no curative therapy at this time, but rehabilitative treatments including complete decongestive therapy for edema control appeared to be beneficial.

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)

A Case of Lymphangiectasia after Treatment for Breast Cancer / 대한피부과학회지

No abstract available.

Lymphangiectasia follows traumatic ulcer of the vulva – A rare coincidence: Case report.

A 40 years old woman, presented with vulvar itching associated with warty lesions appeared after a trauma to vulvar region few months back. She gave history of fall on a sharp object and developed those lesions while working as a labourer. She was diagnosed as a case of Lymphangiectasia of vulva. It was a rare coincidence, so we are reporting this case.

Linfangiectasias en yeyuno como causa de sangrado digestivo oscuro manifiesto / Clinical case of lymphangiectasia in jejunum as a cause of manifest obscure gastrointestinal bleeding / Clinical case of lymphangiectasia in jejunum as a cause of manifest obscure gastrointestinal bleeding

El sangrado digestivo oscuro constituye entre 10 y 20% de todas las causas de sangrado digestivo. La etiología está claramente relacionada con el grupo etario en que se presente. Las linfangiectasias del yeyuno son una causa rara de sangrado digestivo, pero puede llegar a ser de alta mortalidad en caso de que no se detecte tempranamente. Reportamos un caso de un paciente joven sin historia previa de enfermedad, con sangrado digestivo manifestado por melenas, con anemización severa, con estudios endoscópicos iniciales normales, en quien hacemos abordaje con estudios para intestino delgado (video cápsula endoscópica y enteroscopia anterógrada de doble balón), con los que hacemos el diagnóstico y es llevado a cirugía, con adecuada evolución clínica y curación de la enfermedad. (Acta Med Colomb 2015; 40: 158-161).

Obscure gastrointestinal bleeding is between 10 to 20% of all causes of gastrointestinal bleeding. The etiology is clearly related to the age group in which it is presented. Lymphangiectasia in jejunum is a rare cause of gastrointestinal bleeding, but can have high mortality if not detected early. A case of a young patient with no history of previous disease presenting gastrointestinal bleeding manifested by melaena, with severe anemia and normal initial endoscopic studies, is presented. The diagnosis was made by endoscopic video capsule and antegrade double-balloon enteroscopy and surgery was performed with adequate clinical evolution and cure of the disease. (Acta Med Colomb 2015; 40: 158-161).

Hennekam lymphangiectasia syndrome.

Hennekam lymphangiectasia syndrome is a rare autosomal recessive condition. Onset is usually in childhood. The prevalence is unknown but less than 50 cases have been reported in the literature. Incidence is about 1 in 100000 and occurs in all ethnic groups. The syndrome is characterized by the association of lymphedema, intestinal lymphangiectasia, intellectual deficit and facial dysmorphism. Here is a case presented with distension of abdomen with ascites, bilateral pedal oedema, macrocephaly, left half facial edema, left half hypertrophied tongue, dental anomalies, acanthosis nigricans, acrochordons and syndactyly consistent with a diagnosis of Hennekam syndrome. The diagnosis of Hennekam is suspected on the basis of clinical phenotypic features. This is one of the very few cases reported from India.

Report a case of congenital pulmonary lymphangiectasia with fetal pleural effusions / 临床儿科杂志

ObjectiveTo summarize and review the clinical characteristics of congenital pulmonary lymphangiectasia with fetal bilateral pleural effusions.MethodsThe clinical data of a newborn diagnosed as congenital pulmonary lymphangiec-tasia with bilateral pleural effusions in Obstetrics and Gynecology Hospital, was summarized. The clinical, radiographic features, treatment and prognosis of this case are discussed in the context of the literature review.ResultsThe premature infants present-ed with rapid progression bilateral pleural effusions, respiratory distress, chylothorax, hypoalbuminemia and persistent pulmo-nary hypertension at birth. The pulmonary surfactant was given and mechanical ventilation was used for respiratory support. NO was inhaled, high-frequency mechanical ventilation was applied and albumin was repeatedly administered. After treatment for 3 months in the NICU, the patient was discharged. After 10 days, the patient was administered to the PICU with severe pneumo-nia, chronic lung disease, mechanical ventilation and anti-infection treatments were applied for 2 months. After living for six months, the baby died.ConclusionsCongenital pulmonary lymphangiectasia is extremely rare and prenatal diagnosis was dif-ifcult. The disease should be considered in patients presented with progressive dyspnea, interstitial emphysema after birth. Lung tissue biopsy and radioisotope scanning should be performed in time to get diagnosis.

Pneumonectomy Case in a Newborn with Congenital Pulmonary Lymphangiectasia

Congenital pulmonary lymphangiectasia (CPL) is a rare lymphatic pulmonary abnormality. CPL with respiratory distress has a poor prognosis, and is frequently fatal in neonates. We report a case of pneumonectomy for CPL in a newborn. An infant girl, born at 39 weeks' after an uncomplicated pregnancy, exhibited respiratory distress 1 hr after birth, which necessitated intubation and aggressive ventilator care. Right pneumonectomy was performed after her symptoms worsened. Histologic examination indicated CPL. She is currently 12 months old and developing normally. Pneumonectomy can be considered for treating respiratory symptoms for improving chances of survival in cases with unilateral CPL.

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.

Primary intestinal lymphangiectasia in 8 infants / 中华实用儿科临床杂志

Objective To improve the diagnosis and treatment level of primary intestinal lymphangiectasia (PIL) in infants.Methods Clinical,laboratory,gastroscopy imaging,lymph radionuclide imagining,and therapeutic intervention data in eight patients admitted with PIL in Beijing Children's Hospital from Jan.2007 to Feb.2012 were reviewed.Results Their ages ranged from 4 to 8 months old.The common complaints were edema(8/8 cases),diarrhea (8/8 cases),infection (8/8 cases),and ascites (7/8 cases).Other symptoms included vomiting (5/8 cases),low body weight (2/8 cases),and convulsions (2/8 cases).Infections involving respiratory tract,blood,and gastrointestinal system were prominent.The causal pathogens for the infections were bacteria,virus,fungi,and ectosarc.The laboratory abnormalities included lymphocytopenia (8/8 cases),hypoalbuminemia (8/8 cases),and hypogammaglobulinemia (8/8 cases).On immunologic evaluation,the CD4 cell counts and serum IgG levels were significantly decreased(7/7 cases) while B cell and NK cell counts were normal.The gastroscopy revealed nodular lesions in duodenal that appeared white opaque spots(8/8 cases).Further pathological examinations indicated dilated lymphatic channel in mucosal and submucosal(5/8 cases).Lymph radionuclide imaging discovered abnormalities consistent with PIL in 6 out of 8 patients,including 3 cases that were negative for pathological examination.A multidisciplinary approach was taken to treat each patient.The low-fat and medium chain triglycerides-rich diet was introduced with supplements of albumin and globulin.Infection control and edema alleviation were well managed through medication.However,diarrhea remained a problem.Only 2 out of 8 patients had recovered from hypoalbuminemia and lymphocytopenia.One case had a complete remission after surgical therapy withno relapse.Conclusions PIL in infant has common clinical presentations with an exception of more severe infection.Lymph radionuclide imaging is a sensitive diagnostic method.Medication treatment for infant PIL is not satisfactory.Surgical management is recommended if the locus of pathology is confined.