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1.
Arch. argent. pediatr ; 119(3): e264-e268, Junio 2021. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1248231

ABSTRACT

La linfangiomatosis pulmonar difusa es una enfermedad rara caracterizada por una marcada proliferación y dilatación de los vasos linfáticos en los pulmones, la pleura y el mediastino. Se desconoce la prevalencia, y la etiología no se comprende completamente.Una niña de 22 meses ingresó por poliserositis, con derrame pericárdico y pleural. Requirió pericardiocentesis y avenamiento pleural, y presentó drenaje de quilo (1,5-4 litros/día) sin respuesta al tratamiento médico (ayuno, nutrición parenteral y octreotide). Se realizó biopsia pulmonar. La anatomía patológica mostró hallazgos compatibles con linfangiomatosis difusa pulmonar. Comenzó tratamiento con sirolimus y propanolol, que disminuyeron las pérdidas por el drenaje pleural a la semana. Presentó buena evolución; suspendió aporte de oxígeno y se retiró el drenaje pleural. Se externó al cuarto mes de internación. El diagnóstico temprano de la linfangiomatosis pulmonar difusa es difícil de lograr, pero permite aplicar terapéuticas que evitan la progresión de enfermedad y disminuir la morbimortalida


Diffuse pulmonary lymphangiomatosis is a rare disease characterized by marked proliferation and dilation of lymphatic vessels in the lungs, pleura, and mediastinum. The prevalence is unknown and the etiology is not fully understood.A 22-month-old girl was admitted for polyserositis, with pericardial and pleural effusion. She required pericardiocentesis and pleural drainage, presenting chyle drainage (1.5-4 liters/day) without response to medical treatment (fasting, parenteral nutrition and octreotide). A lung biopsy was performed. The pathological anatomy showed findings compatible with diffuse pulmonary lymphangiomatosis. Treatment with sirolimus and propanolol began, decreasing losses due to pleural drainage one week after treatment. She progressed well, discontinued oxygen supply and pleural drainage was removed, leaving the patient after the fourth month of hospitalization.Early diagnosis of diffuse pulmonary lymphangiomatosis is difficult to achieve, but it allows the application of therapies that prevent disease progression, reducing morbidity and mortality.


Subject(s)
Humans , Female , Infant , Lung Diseases/congenital , Lymphangiectasis/congenital , Pleural Effusion , Propranolol/therapeutic use , Biopsy , Sirolimus/therapeutic use , Lung Diseases/pathology , Lung Diseases/diagnostic imaging , Lymphangiectasis/pathology , Lymphangiectasis/diagnostic imaging
2.
Rev. cuba. med. mil ; 49(4): e603, graf
Article in Spanish | LILACS, CUMED | ID: biblio-1156524

ABSTRACT

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)


Subject(s)
Humans , Female , Middle Aged , Congenital Abnormalities , Tomography, X-Ray Computed/methods , Kidney/abnormalities , Lymphangiectasis/diagnostic imaging , Lymphangioma/diagnosis
5.
Rev. chil. pediatr ; 89(4): 516-520, ago. 2018. graf
Article in Spanish | LILACS | ID: biblio-959555

ABSTRACT

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.


Subject(s)
Humans , Male , Infant, Newborn , Infant, Premature, Diseases/diagnosis , Lung Diseases/congenital , Lymphangiectasis/congenital , Infant, Premature , Lung Diseases/diagnosis , Lymphangiectasis/diagnosis
6.
Acta méd. colomb ; 40(2): 158-161, abr.-jun. 2015. ilus, tab
Article in Spanish | LILACS, COLNAL | ID: lil-762703

ABSTRACT

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).


Subject(s)
Humans , Male , Middle Aged , Lymphangiectasis , Capsule Endoscopes , Double-Balloon Enteroscopy , Hemorrhage , Intestine, Small
7.
Journal of Korean Medical Science ; : 609-613, 2014.
Article in English | WPRIM | ID: wpr-65537

ABSTRACT

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.


Subject(s)
Female , Humans , Infant, Newborn , Gestational Age , Lung/pathology , Lung Diseases/congenital , Lymphangiectasis/congenital , Lymphatic Vessels/pathology , Tomography, X-Ray Computed
8.
Journal of the Korean Ophthalmological Society ; : 1165-1174, 2013.
Article in Korean | WPRIM | ID: wpr-112416

ABSTRACT

PURPOSE: In this study we investigated the histopathologic characteristics of conjunctivochalasis and its association with lymphangiectasis using impression cytology and conjunctival excisional biopsy in patients with conjunctivochalasis. METHODS: This prospective study included 14 outpatients diagnosed with conjunctivochalasis, and who had excisional biopsies performed between March 1, 2012 and November 30, 2012. Preoperative slit-lamp examination, dry eye diagnostic test and impression cytology were also performed. For histological analysis, conjunctival sections were stained with hematoxylin and eosin (H&E), Verhoeff-van Gieson (VVG) elastic staining, and monoclonal antibody D2-40, a lymphatic endothelial marker. RESULTS: Tear break-up time and Schirmer's value were decreased in patients with conjunctivochalasis. Impression cytology showed a decrease in goblet cell density and an increase in nucleoplasmic/cytoplasmic ratio. The patients' conjunctival tissues exhibited an infiltration increase of chronic inflammatory cells on H&E stain, and a decrease in collagen density with degeneration of elastic fibers on VVG stain compared to tissues from the control subjects. D2-40 immunohistochemistry revealed the dilated subconjunctival lymphatics in patients with conjunctivochalasis. CONCLUSIONS: This study suggests that the characteristic histopathological features of conjunctivochalasis include dilated lymphatic vessels as well as decreased goblet cell and collagen densities with degeneration of elastic fibers.


Subject(s)
Humans , Antibodies, Monoclonal, Murine-Derived , Biopsy , Collagen , Diagnostic Tests, Routine , Elastic Tissue , Eosine Yellowish-(YS) , Eye , Goblet Cells , Hematoxylin , Immunohistochemistry , Lymphangiectasis , Lymphatic Vessels , Outpatients , Prospective Studies
9.
Rev. méd. Chile ; 140(10): 1312-1325, oct. 2012. ilus
Article in Spanish | LILACS | ID: lil-668705

ABSTRACT

Background: Renal lymphangiectasia is a rare disorder characterized by the presence of cystic masses in renal parenchyma, renal sinus or perinephric space. This condition, congenital or acquired, is probably caused by an alteration of renal lymphatic drainage to retroperitoneal lymph ducts. We report a 48-year-old woman, who consulted in the emergency room due to left flank pain and microscopic hematuria. Blood pressure and renal function were normal. A CT scan showed a multilocular cystic mass in the left renal sinus without alterations in the renal parenchyma. Renal lymphangiomatosis was diagnosed. The pain subsided with analgesics and did not recur. Annual clinical and ultrasound follow-up was suggested.


Subject(s)
Female , Humans , Middle Aged , Kidney Diseases , Lymphangiectasis , Diagnosis, Differential , Follow-Up Studies , Hematuria/etiology , Incidental Findings , Tomography, X-Ray Computed
10.
Radiol. bras ; 45(3): 178-180, maio-jun. 2012. ilus
Article in Portuguese | LILACS | ID: lil-640286

ABSTRACT

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.


Subject(s)
Aged , Cysts , Diagnosis, Differential , Kidney , Lymphatic System , Lymphangiectasis/diagnosis , Thorax , Dyspnea , Tomography, X-Ray Computed , Ultrasonography
11.
Rev. colomb. radiol ; 22(3): 3272-3280, sept. 2011.
Article in Spanish | LILACS | ID: lil-619399

ABSTRACT

La linfangectasia renal es una entidad rara de los linfáticos renales que se presenta tanto en niños como en adultos, puede ser unilateral o bilateral y no tiene predilección por sexo. Se ha conocido con otros nombres, como linfangiomatosis renal, linfangioma renal, linfangectasia peripélvica, enfermedad poliquística del seno renal e higroma renal. Esta entidad es poco frecuente, por lo cual es común que sea erróneamente interpretada en las imágenes como quistes parapiélicos, quistes renales o hidronefrosis. Por lo tanto es importante que el radiólogo esté familiarizado con sus características imaginológicas.


Subject(s)
Kidney , Lymphangiectasis , Magnetic Resonance Imaging , Tomography
12.
Chinese Medical Journal ; (24): 797-800, 2011.
Article in English | WPRIM | ID: wpr-321414

ABSTRACT

Diffuse pulmonary lymphangiomatosis (DPL) is a rare disease that is characterized by diffuse proliferation of abnormal pulmonary lymphatic channels. DPL occurs mostly in children and young adults and often undergoes a progressive clinical course, eventually causing deterioration of the lung. Both the clinical diagnosis and treatment of DPL remain a challenge. Here, we report a case of DPL in a 53-year-old Chinese woman with comprehensive investigations including pulmonary function tests, computer tomography (CT), bronchoscopy and histological examination of the lung biopsy, and review the literature.


Subject(s)
Female , Humans , Middle Aged , Bronchoscopy , Lung Diseases , Diagnosis , Diagnostic Imaging , Metabolism , Lymphangiectasis , Diagnosis , Diagnostic Imaging , Metabolism , Tomography, X-Ray Computed
13.
Tuberculosis and Respiratory Diseases ; : 428-432, 2011.
Article in Korean | WPRIM | ID: wpr-181451

ABSTRACT

Pulmonary lymphangiomatosis is a rare disorder involving the entire intrathoracic lymphatic system from the mediastinum to the pleura. Pulmonary lymphangiomatosis mostly occurs in children and young adults without gender predilection. Although it is pathologically benign, it shows a progressive and fatal course with variable initial presentation. We now report a case of pulmonary lymphangiomatosis in a 35-year-old man. He presented with hemoptysis 6 months previously. Chest x-ray and a chest computed tomography scan showed diffuse interstitial thickening with left pleural effusion. Chylothorax was confirmed by thoracentesis. Lymphangiography showed dilated and tortuous lymphatic channels. Surgical lung biopsy revealed proliferation of complex anastomosing lymphatic channels. He was diagnosed with pulmonary lymophangiomatosis. Closed thoracostomy and chemical pleurodesis were done and the dyspnea was reduced.


Subject(s)
Adult , Child , Humans , Young Adult , Biopsy , Chylothorax , Dyspnea , Hemoptysis , Lung , Lung Diseases , Lung Diseases, Interstitial , Lymphangiectasis , Lymphatic System , Lymphography , Mediastinum , Pleura , Pleural Effusion , Pleurodesis , Thoracostomy , Thorax
15.
Indian J Dermatol Venereol Leprol ; 2007 Sep-Oct; 73(5): 353-4
Article in English | IMSEAR | ID: sea-53199
16.
Journal of Korean Medical Science ; : 740-745, 2007.
Article in English | WPRIM | ID: wpr-169938

ABSTRACT

Congenital pulmonary lymphangiectasis (CPL) is a rare, poorly documented disease, characterized by abnormal dilatation of pulmonary lymphatics without lymphatic proliferation. This disease is seen almost exclusively in infancy and early childhood. It can usually be divided into primary (congenital) and secondary forms. The primary form presents in neonates, and the patients mostly die due to the respiratory distress, shortly after birth. The authors experienced two cases of primary CPL in a 13-day-old male neonate and a one-day-old male neonate, showing prominent lymphatic dilatation in the septal, subpleural, and peri-bronchial tissue throughout both lungs. The latter case was associated with congenital cardiac anomaly including single ventricle. These are unique cases of CPL in Korea of which the diagnosis was established through post-mortem examination. Therefore, the authors report these two cases with primary CPL with a review of the literature.


Subject(s)
Humans , Infant, Newborn , Male , Lung/pathology , Lung Diseases/congenital , Lymphangiectasis/congenital , Lymphatic System/pathology
17.
Korean Journal of Dermatology ; : 1044-1050, 2006.
Article in Korean | WPRIM | ID: wpr-189430

ABSTRACT

BACKGROUND: Lymphangioma is an uncommon, benign hamartomatous malformation that consists of dilated lymphatic channels lined by normal, single-cell lymphatic endothelia. The diagnosis of lymphangioma is usually made on the basis of clinical and histopathological characteristics. The lymphangiomas are divided into lymphangioma circumscriptum, cavernous lymphangioma, cystic higroma, acquired progressive lymphangioma and lymphangiectasis. OBJECTIVE: The purpose of this study was to investigate clinical and histopathological features of lymphangioma, especially focusing on the pathologic classification. METHODS: We retrospectively reviewed the medical records and 52 biopsy specimens of 51 patients who had been diagnosed with lymphangioma between January 1991 to September 2005 in 8 Hospitals under the charge of the Catholic Medical Center. RESULTS: We compiled clinical data and histologic information on the 52 cases of 51 patients of lymphangioma as follows: The mean age at development was 16.5 years, and 56.9% of the patients were under 10 years old. Lymphangioma developed predominantly in females, at a sex ratio of 1:1.55. The disease duration exceeded 10 years in 45.1% of patients. Most patients were asymptomatic, but 13.7%, 9.8% and 2.0% of patients complained of tenderness, pruritus and pain, respectively. The lesion was usually solitary. Lymphangioma occurred on the trunk, extremities, head and neck, and genitalia in 43.6%, 30.9%, 20.0% and 5.5% of 52 cases respectively. The legs, back, abdomen and lip were also found to be commonly involved. Histopathologically, lymphangiomas were divided into lymphangioma circumscriptum, cavernous lymphangioma, cystic higroma, acquired progressive lymphangioma and lymphangiectasis in 69.2%, 17.3%, 1.9%, 5.8% and 5.8% of the 52 cases respectively. CONCLUSION: Lymphangioma is un uncommon, benign hamartomatous malformation of lymphatic endothelial cells and occurs in several forms that differ in clinical and histological characteristics and prognosis. Therefore it is important to make a diagnosis on the basis of clinical and histopathologic findings and to understand the characteristics of the subtypes of lymphangioma.


Subject(s)
Child , Female , Humans , Abdomen , Biopsy , Classification , Diagnosis , Endothelial Cells , Extremities , Genitalia , Head , Leg , Lip , Lymphangiectasis , Lymphangioma , Medical Records , Neck , Prognosis , Pruritus , Retrospective Studies , Sex Ratio , United Nations
18.
Diagnóstico (Perú) ; 43(1): 37-39, ene.-feb. 2004. ilus
Article in Spanish | LILACS, LIPECS | ID: lil-409511

ABSTRACT

Se reporta un caso de Elefantiasis Nostras Verrucosa del miembro inferior izquierdo tratado por el Departamento de Senos y Tumores Mixtos del Instituto de Enfermedades Neoplásicas. Los datos de su incidencia en la población no han sido reportados debido a la rareza de esta patología. Es un desorden crónico y deformante, caracterizado por hiperqueratosis y papilomatosis de la epidermis, con fibrosis de la dermis y del tejido celular subcutáneo. Sin el tratamiento adecuado los cambios cutáneos son lentamente progresivos culminando con el crecimiento grotesco de la región del cuerpo afectada, tal como se reporta en esta ocasión. Describimos el caso de una mujer de 42 años, con historia de tumoración gigante en el muslo izquierdo que la mantenía postrada en cama y que fue tratada quirúrgicamente con conservación del miembro inferior, dada de alta con resultados excelentes.


Subject(s)
Humans , Adult , Female , Elephantiasis , Lymphangiectasis
19.
The Journal of the Korean Rheumatism Association ; : 61-65, 2004.
Article in Korean | WPRIM | ID: wpr-81385

ABSTRACT

Protein-losing enteropathy is a rare complication of autoimmune diseases. We report a case of primary Sjogren's syndrome with protein-losing enteropathy in 50-year-old female who complained of generalized edema. Protein-losing enteropathy of the small intestine was demonstrated by Tc-99m labeled albumin abdominal scintigraphy. Duodenal biopsies showed chronic inflammatory cell infiltration without lymphangiectasis or vasculitis. The patient received oral prednisolone (60 mg/day) for 4 weeks with subsequent clinical improvement. Primary Sjogren's syndrome or other autoimmune diseases should be considered in cases of protein-losing enteropathy.


Subject(s)
Female , Humans , Middle Aged , Autoimmune Diseases , Biopsy , Edema , Intestine, Small , Lymphangiectasis , Prednisolone , Protein-Losing Enteropathies , Radionuclide Imaging , Sjogren's Syndrome , Vasculitis
20.
Journal of the Korean Association of Maxillofacial Plastic and Reconstructive Surgeons ; : 520-523, 2002.
Article in Korean | WPRIM | ID: wpr-784434
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