Linfangiomatosis pulmonar difusa con compromiso pleural y pericárdico. Reporte de un caso pediátrico / Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report

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.

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)

Linfangiomatosis kaposiforme: reporte de un caso y revisión de la literatura / Kaposiform lymphangiomatosis: a case report and literature review

La linfangiomatosis es una patología benigna, de etiología desconocida, que afecta especialmente a población pediátrica, caracterizándose por desarrollo de masas subcutáneas que involucran uno o más órganos. Cuando se asocia con coagulopatía y derrames quilosos, recibe el nombre de linfangiomatosis kaposiforme (LK), cuya evidencia disponible es escasa. El objetivo de este manuscrito es reportar un caso de LK pediátrico atendido en el Instituto del Cáncer SOLCA, Cuenca, Ecuador. Paciente femenina de 1 año 7 meses, con hernia inguinal bilaterales y alteración de los tiempos de coagulación. En los estudios de imagen se evidenció una masa mediastínica asociada a presencia de líquido en espacio pleural y cavidad abdomino-pélvica. Además, se evidenció la existencia de múltiples imágenes hipogénicas en el bazo. Se practicó timectomía mínima, toma de biopsia de linfonodos mediastínicos para establecer el diagnóstico, y se instaló un dren en el tórax para tratar el derrame pleural antes señalado. Se descartaron patologías similares (linfomas, lupus, inmunológicas, etc.). Dado el continúo deterioro respiratorio, coagulopatía, lesiones hipogénicas esplénicas y drenaje abundante del quilotórax por el tubo torácico, se planteó el diagnóstico de LK. Se inició apoyo nutricional vía parenteral (sin lípidos); se indicó vincristina, y se realizó una pleurodesis con bleomicina sin buena respuesta. La paciente falleció a los 16 días de su ingreso. Se trata de un caso de mal pronóstico, con evolución rápidamente progresiva hacia un desenlace fatal.

Lymphangiomatosis is a benign pathology, of unknown etiology. Affects especially to pediatric population and is characterized by development of subcutaneous masses that involve one or more organs. When it presents with coagulopathy and chylous effusions, it is called kaposiform lymphangiomatosis (KL). The available evidence of KL is scarce. The objective of this manuscript was to report a case of pediatric KL treated at SOLCA Cancer Institute, Cuenca, Ecuador. Female patient of 1 year and 7 months age. She had a bilateral inguinal hernia and altered coagulation times. Imaging studies revealed a mediastinal mass associated with fluid in the pleural space and in the abdominalpelvic cavity. Furthermore, the existence of multiple hypogenic images in the spleen was evidenced. Minimal thymectomy, biopsy of mediastinal lymph nodes was performed to establish the diagnosis, and a drain was installed in the chest to treat the aforementioned pleural effusion. Other pathologies like lymphomas, lupus, etc. were ruled out. Given the continuous respiratory deterioration, coagulopathy, splenic hypogenic lesions and abundant drainage of the chylothorax through the chest tube, the diagnosis of KL was raised. Parenteral nutrition (lipid-free) was started; Vincristine was indicated, and a pleurodesis with bleomycin was performed without a good response. The patient died 16 days after her admission. It is a case of very bad prognosis, with a rapidly progressive evolution towards a fatal outcome.

A Case of Gorham-Stout Disease with Progressive Cutaneous Involvement / 대한피부과학회지

Gorham-Stout disease is a rare bone disorder that is characterized by angiomatous proliferation and results in destruction and resorption of the osseous matrix. It has variable presentations and a number of different sites may be affected, including the dermis, soft tissue, and viscera. The involved cutaneous and soft tissue lesions typically present with sponge-like, soft, brown or purple lesions. The clinical course is generally prolonged, with eventual stabilization of the affected bone; however, it is rarely fatal. There is no standard treatment defined for this disease, and variable therapies such as medical, surgical, and radiation therapy have been used. We report a case of 24-year-old man with Gorham disease who presented with progressive cutaneous lesions and severe lymphedema adjacent to the diseased bone.

A rare case of pulmonary lymphangiomatosis from the tribal zone of central India.

Lymphangiomatosis, a rare disease, occurs in individuals of any age, regardless of gender, but is predominantly seen in younger individuals. It often presents with pulmonary involvement, although, the bones, spleen and liver can also be affected. Histologically, pulmonary involvement includes proliferation, complex anastomoses and secondary dilatation of the lymphatic vessels. Clinically presentation is often variable but pulmonary involvement is affected more common than other involvements. Diagnosis is made histologically but radiologically can suggest diseases. Treatment is only supportive and symptomatic.

Colonic Lymphangiomatosis Resolved after Excisional Biopsy

Lymphangioma is an uncommon malformation of the lymphatic system that involves a benign proliferation of the lymphatics, with no established treatment method. Multiple colonic lymphangioma, or colonic lymphangiomatosis, is an extremely rare condition. We report a case of colonic lymphangiomatosis that was detected during a colonoscopic examination conducted as part of a general health check-up. The lesion completely resolved after excisional biopsy.

A Rare Case of Diffuse Pulmonary Lymphangiomatosis in a Middle-Aged Woman

Diffuse pulmonary lymphangiomatosis (DPL) is a rare lymphatic disorder characterized by lymphatic channel proliferation. It is mostly reported in children and young adults. Here, we report a case involving a 52-year-old asymptomatic woman who presented with increased interstitial markings, as seen on a chest radiograph. Diffuse interstitial septal thickening was found on a serial follow-up chest computed tomography scan, and lymphangitic metastasis was the primary radiologic differential diagnosis. However, histologic sections of wedge resected lung revealed diffuse pleural and interlobular septal lymphatic proliferation characteristic of DPL.

Linfangiectasia renal unilateral: Caso clínico / Unilateral renal limphangiectasia: Report of one case

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.

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.

A Case of Pulmonary Lymphangiomatosis / 결핵및호흡기질환

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.

Lymphangiomatosis Involving the Inferior Vena Cava, Heart, Pulmonary Artery and Pelvic Cavity

A 38-year-old woman who had undergone pelvic lymphangioma resection two months previously presented with cough and dyspnea. Transthoracic echocardiography and CT demonstrated the presence of a mixed cystic/solid component tumor involving the inferior vena cava, heart and pulmonary artery. Complete resection of the cardiac tumor was performed and lymphangioma was confirmed based on histopathologic examination. To the best of our knowledge, this is the first report of lymphangiomatosis with cardiac and pelvic involvement in the published clinical literature.

Congenital lymphangiomatosis of the right lower limb / 대한산부인과학회지

Lymphangiomatosis is a condition of lymphatic tissue malformation with multiple or diffuse involvement of soft tissues, visceral organs. Congenital abnormalities of the lymphatic system are very rare, and reports of congenital lymphangiomatosis are even fewer. We experienced a case of congenital lymphangiomatosis detected as edema of the right limb by prenatal ultrasonography and then diagnosed by magnetic resonance imaging. We describe this case with a brief review of the literature.

A Case Report with Lymphangiomatosis of the Colon

The incidence of lymphangiomas in the gastrointestinal tract is low, particularly in the colon and rectum, and most cases are solitary. Lymphangiomatosis of the colon are encountered infrequently with only one report in the English literature, and polypectomy was performed for the diagnosis in that case report. However, trends in the diagnosis of lymphangiomatosis of colon have been changing since the development of endoscopic ultrasonography (EUS), and this case is the first in that lymphangiomatosis of the colon was diagnosed without invasive procedures. Here we describe the case of 31-yr-old woman with lymphangiomatosis of the colon with numerous polyposis-like appearing lesions diagnosed by endoscopic ultrasonography and a colonoscopy.

Generalized Lymphangiomatosis: A Case Report

Generalized lymphangiomatosis is a rare congenital malformation of the lymphatics. CT and MR scan have been used to evaluate lymphangiomas, which appear as large multicystic fluid-filled masses. CT and MR Imaging findings are often helpful in distinguishing lymphangiomas from various vascular disorders. We report the findings of CT, MRI and bone scan in a patient with generalized cystic lymphangiomatosis. Whole body 3.0-T MR scan using STIR sequence with a larger FOV could detect the additional lesions that were not seen at other imaging modalities. We believe that whole body 3.0 T MR imaging is a good modality to evaluate the extent of the disease and following up the patients with the generalized cystic lymphangiomatosis.

A Case of Lymphangiomatosis Arising in the Colon / 대한소화기내시경학회지

Lymphangioma is a benign vascular lesion that shows the characteristics of subepithelial tumor, which can proliferate in the lymphatic system. Only a few cases of multiple lymphangimas of the colon, the so called "colonic lymphanigomatosis", have been currently reported on the medical literature. Because lymphangioma is absolutely a benign tumor, it does not require any specific treatment, except for rare disease-related symptoms or complications such as anemia, intussusception and protein-losing enteropathy. Endoscopic resection for this tumor has sometimes been performed for both diagnostic and therapeutic purposes. We recently experienced a case of multiple colonic lymphangomas that arose in the ascending colon of a 46-year-old male patient and these lesions were found during performance of colonoscopy. The final diagnosis was confirmed by a pathologic examination of the specimen that was obtained via endoscopic resection. Follow-up colonoscopy at 1 year after the initial examination showed complete resolution of the previously noted lesions without any specific treatment. We report here on a very rare case of colonic lymphangiomatosis along with a brief review of the relevant literature.

Metachronous Bilateral Renal Lymphangiomatosis Mimicking as a Simple Renal Cyst / 대한비뇨기과학회지

Renal lymphangiomatosis is a rare developmental malformation of the perirenal lymphatic system, and this can result in the development of cystic masses in the peri-pelvic or peri-renal areas. This is difficult to differentiate from other cystic renal disease on imaging studies. We present here a case of metachronous bilateral renal lymphangiomatosis that was falsely diagnosed as a simple renal cyst. A 47 year-old man was referred for a large amount of drainage after right renal cyst marsupialization. The abdominal computed tomography(CT) findings and fluid analysis were compatible with a lymphangiomatosis. After 6 month, he presented with flank pain on his left side and CT showed intracystic hemorrhage on the left renal lymphangiomatosis. Percutaneus drainage and conservative management were done.

Metachronous Bilateral Renal Lymphangiomatosis Mimicking as a Simple Renal Cyst / 대한비뇨기과학회지

Renal lymphangiomatosis is a rare developmental malformation of the perirenal lymphatic system, and this can result in the development of cystic masses in the peri-pelvic or peri-renal areas. This is difficult to differentiate from other cystic renal disease on imaging studies. We present here a case of metachronous bilateral renal lymphangiomatosis that was falsely diagnosed as a simple renal cyst. A 47 year-old man was referred for a large amount of drainage after right renal cyst marsupialization. The abdominal computed tomography(CT) findings and fluid analysis were compatible with a lymphangiomatosis. After 6 month, he presented with flank pain on his left side and CT showed intracystic hemorrhage on the left renal lymphangiomatosis. Percutaneus drainage and conservative management were done.

Colonic Lymphangiomatosis with Normal Colonoscopic Finding in an Adult

Lymphangiomatosis of the colon is an extremely rare disease. We report here a case of a 40 year old woman with colonic lymphangiomatosis who was treated by surgical resection. Computed tomography of the abdomen and pelvis showed a multiple cystic mass encasing the descending colon. However, although colonoscopic examination did not reveal any abnormalities, a laparotomy revealed cystic masses on the entire colon; therefore a near total colectomy was performed. There was no sign of recurrence after 14 months of follow up. Histology revealed lymphangiomatosis of the colon. Colonic lymphangiomatosis is an uncommon disorder, and the occurrence of a case of lymphangiomatosis that cannot be detected by colonoscopy is extremely rare. Therefore, this report should be helpful for the management of patients with lymphangiomatosis.

Lymphangiomatosis of Bone and Visceral Parenchyma : Report of Two Cases / 대한정형외과학회잡지

Lymphangiomatosis is a rare disorder that occurs mainly in children or during the first two decades of life. It is characterized by a diffuse proliferation of lymphatic channels involving the bones, visceral parenchyma, and soft tissue. Most cases of lymphangiomatosis have bone and visceral involvement and usually present with chylothorax, chylous ascites, chylous pericardial effusion, or acute symptoms that are related to the affected organs. The authors experienced two cases that presented with chylothorax and multiple lytic bone lesions. Chest drainage and chemical pleurodesis were performed for treatment of the chylothorax. In one case, lytic bone lesions were found only in the right scapula and bone lengthening with an Ilizarov frame was performed for growth arrest in the right humerus. In the other case, lytic bone lesions were found in both femurs and both humeri, the right tibia, and the right scapula; and were particularly severe in the right tibia and femur. The lytic bone lesion, osteosclerosis, pathologic fracture, and pseudoarthrosis were so severe that weight-bearing was impossible. Internal fixation was performed with an intramedullary nail in the left femur.