Despite the poor prognosis of the disease, 5 years after the treatment the patient has not presented new episodes of pleuropericardial effusions, nor has he required hospitalization for other symptoms, currently presenting only exertional dyspnea. [archbronconeumol.org]

The present article discusses clinical, radiographic and pathological features, and treatment options for DPL. [mayoclinic.pure.elsevier.com]

In this paper, We would like to present an unusual case of Diffuse pulmonary lymphangiomatosis involving a 28-year-old young female, who presented with progressive chest pain and polypnea of one year duration, and CT showed much left hydrothorax. [atm.amegroups.com]

This 50-year-old nonsmoking Hispanic woman presented with a 20-year history of cough, hemoptysis, chyloptysis, and pleuritic chest pain. [atsjournals.org]

Polyposis-like presentation of multiple lymphangiomas of the sigmoid colon. Tumori 2004;90:625-7. [indianjcancer.com]

• Ascites

  Chest and abdominal radiograph showed massive right pleural effusion causing collapse of the right lung with contralateral mediastinal shift and ascites [Figure 2]. [ijamhrjournal.org]

  Signs and symptoms Lymphangiomatosis (LYMF) can present with pericardial, pleural effusion, ascites (fluid in the abdominal cavity), and internal bleeding. [bionity.com]

  Nós descrevemos um caso extremamente raro de linfoangiomatose sistêmica congênita, em um recém-nascido que apresentava ascite e insuficiência respiratória, desenvolvidos imediatamente após o nascimento. O óbito ocorreu nas primeiras horas de vida. [scielo.br]

  In some case reports, chylous ascites, protein wasting enteropathy, peripheral lymphoedema, hemihyperplasia and lymphopenia have been also described [ 1 ] ( figure 3 ). [err.ersjournals.com]

  However, induration of the cyst and infection often complicate these procedures. [17] Complications of undiagnosed/untreated cases include hematuria, ascites, hypertension, renal vein thrombosis, and impairment of renal function. [18], [19] Perinephric [urologyannals.com]

• Splenectomy

  For larger splenic cysts, splenectomy is advantageous, as the risk of rupture is very high 8. Splenectomy tends to result in relief of symptoms and in resolution of anaemia and thrombocytopenia in patients with hypersplenism. [ispub.com]

  Significant symptom relief was recorded in one patient with KLA who underwent splenectomy (patient #3). [ojrd.biomedcentral.com]

  We present the case of a patient with hygromas or diffuse lymphangiomatosis of the spleen, analyzing the etiopathogenesis and diagnostic and therapeutic options, and proposing total splenectomy as the surgical technique of choice in cases of diffuse lesions [elsevier.es]

  Results from a splenectomy and an open liver biopsy were consistent with lymphangioma. Two weeks later, she developed hemoptysis, chest pain, and hemothorax, which was managed with the placement of a chest tube for drainage. [jnccn.org]

  One child underwent a splenectomy; one child underwent therapeutic embolization. Eight patients remained asymptomatic with respect to the spleen 1-20 years later. [lymphedemapeople.com]

• Lymphedema

  Symptoms include shortness of breath, difficulty breathing in (inhaling), severe pain in the abdominal cavity and pelvic cavity, and lymphedema (swelling). [bionity.com]

  Syndrome of Humerus in an 18-year-old Female Reported in Pakistan. 38 Ali M...Lakho MT 31403018 2019 10 Gastrointestinal: Diffuse esophageal lymphangiomatosis manifesting as multiple submucosal masses. 38 Cao D...Guo L 31257637 2019 11 Severe Pulmonary Lymphedema [malacards.org]

  […] lymphatics commonly with chylous effusions, and may be associated with lytic bone lesions and mediastinal compression. 8 Other manifestations include cervical lymphangioma, pericardial effusions, chyloptysis, hemoptysis, protein-wasting enteropathy, lymphedema [ncbi.nlm.nih.gov]

  Kaposiform lymangiomatosis: a new entity distinct from generalized lymphatic anomaly, kaposiform hemangioendothelioma, lymphedema-associated angiosaroma, and lymphangioma-like kaposi sarcoma. [atsjournals.org]

• Severe Pain

  Symptoms include shortness of breath, difficulty breathing in (inhaling), severe pain in the abdominal cavity and pelvic cavity, and lymphedema (swelling). [bionity.com]

• Internal Bleeding

  Signs and symptoms Lymphangiomatosis (LYMF) can present with pericardial, pleural effusion, ascites (fluid in the abdominal cavity), and internal bleeding. [bionity.com]

• Cough

  Accepted on 19 Sep 2018 Submitted on 05 Jul 2018 Case Report A 20-year-old man complained of exertional dyspnea for two months, cough and asthenia. He had no fever, chest pain or lower limb edema. [jbsr.be]

  DPL commonly presents with chylous effusions and other non-specific respiratory symptoms such as cough, chest pain, chest tightness, shortness of breath, dyspnea and wheezing. [academic.oup.com]

  Symptoms include chest pain and pressure, coughing up blood and shortness of breath. Talk with your doctor to find the best treatment if you or your child has been diagnosed with pulmonary lymphangiomatosis. [resourcerepository.org]

  CLINICAL • Rare disease characterized by diffuse proliferation of abnormal, complex lymphatic channels – If localized (focal) lesion  lymphangioma • Typically presents in children and young adults – Similar picture of asthma (dyspnea, wheezing, cough [de.slideshare.net]

  Home supplemental oxygen therapy and symptomatic treatment of recurrent cough and wheeze are often necessary during childhood, sometimes associated with prolonged pleural drainage. [orpha.net]

• Abdominal Pain

  Abdominal pain, anemia, abnormal bowel habits, intussusception, and protein-losing enteropathy are the main symptoms of colonic lymphangiomatosis. [indianjcancer.com]

  Lungs – may cause a chronic cough, wheezing, shortness of breath, a collection of fluid around the lungs Heart – rapid heartbeat, chest pain Stomach, intestines – abdominal pain, nausea, vomiting, diarrhea Kidneys – pain, increased blood pressure Bones [verywellhealth.com]

  Case Reports CASE I: A l0-year old Indian boy with a previous history of measles and whooping cough was admitted with an eighteen-hour history of abdominal pain. The boy had been unwell and had lost weight over the previous three months. [pubs.rsna.org]

  Multiannual abdominal pain complicated by obstruction of the gastrointestinal tract in the course of cystic form of mesenteric lymphangioma of the small intestine. Pol JSurgery. 2015;87:185-188. 2. Handa R, Kale R, Harjai MM, Dutta V. [patientcareonline.com]

  The clinical signs and symptoms of colonic lymphangioma vary depending on the size and position of the lesion, and include abdominal discomfort such as abdominal pain and abdominal distension, colonic bleeding, acute abdomen and protein-losing enteropathy [spandidos-publications.com]

• Vascular Disease

  Category Vascular disease Brite Human diseases [BR: br08402 ] Cardiovascular diseases Vascular diseases H01735 Lymphangiomatosis Human diseases in ICD-11 classification [BR: br08403 ] 02 Neoplasms Benign neoplasms, except of lymphoid, haematopoietic, [genome.jp]

  Diagnosis and management of lymphatic vascular disease. J Am Coll Cardiol. 2008;52:799-806. http://www.ncbi.nlm.nih.gov/pubmed/18755341 Kinnier CV, Eu JPC, Davis RD, et al. Successful bilateral lung transplantation for lymphangiomatosis. [rarediseases.org]

• Fracture

  The choice of treatment for lymphangiomatosis depends on the severity and type of involvement; namely, treatment of fractures and surgical procedure to remove cysts compressing vital structures. 2,7 The severity of lymphangiomatosis is related to the [scielo.br]

  Symptoms of kaposiform lymphangiomatosis may include: Respiratory issues such as coughing, wheezing and shortness of breath Hemostatic abnormalities such as bleeding into the chest cavity Bone issues such as pain and fractures Cardiac issues such as a [chop.edu]

  Surgical intervention is necessary for certain pathological fractures and to correct symptomatic deformity. Fractures treated with non-surgical techniques can be expected to heal. [lymphedemapeople.com]

  […] shortness of breath, a collection of fluid around the lungs Heart – rapid heartbeat, chest pain Stomach, intestines – abdominal pain, nausea, vomiting, diarrhea Kidneys – pain, increased blood pressure Bones – pain, weakening of the bone leading to fracture [verywellhealth.com]

  Rib or thoracic vertebral bone involvement may present as chronic pain or acute pain in cases of pathological fracture. Bone involvement in the young may result in shortening or deformity of the affected bone. [adc.bmj.com]

• Angiomatosis

  Aviv RI McHugh K Hunt J : Angiomatosis of bone and soft tissue: a spectrum of disease from diffuse lymphangiomatosis to vanishing bone disease in young patients. [thejns.org]

  Diffuse pulmonary angiomatosis. Thorax 1991; 46:851-3 8 Faul JL, Berry GJ, Colby TV, et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 2000; 161:1037-46 9 Molitch, H. [e-immunohistochemistry.info]

  Several authors have considered lymphangiomatosis as a benign vascular tumor or part of a systemic cystic angiomatosis syndrome. 1,3 Lymphangiomatosis can be classified into three categories: 1. [scielo.br]

  Terminology Imaging Top Differential Diagnoses Clinical Issues Diagnostic Checklist Abbreviations Diffuse pulmonary lymphangiomatosis (DPL) Synonyms Diffuse pulmonary angiomatosis ; uncertain nature of vascular abnormalities Definitions Congenital disorder [app.statdx.com]

  Seckler denominó a esta enfermedad "angiomatosis quística sistémica". La linfangiomatosis quística limitada al bazo es extremadamente rara, existiendo pocos casos descritos en la bibliografía mundial. [elsevier.es]

Clinical Testing and Workup Pulmonary function tests may be used to help diagnose diffuse pulmonary lymphangiomatosis. This group of tests can evaluate how well the lungs are functioning. [rarediseases.org]

[…] histological features of lung specimens from nine patients in whom no extrathoracic lesions were identified, which they termed “diffuse pulmonary lymphangiomatosis” (DPL). 3 Recognition of the disease requires a high index of suspicion and an extensive workup [lgdalliance.org]

MRI showed obvious tumor shrinkage at 6 ( f ), 12 ( g ), and 24 ( h ) months after the start of sirolimus treatment Pathological findings All patients with KLA underwent incisional biopsy during the diagnostic workup (Fig. 1 d). [ojrd.biomedcentral.com]

• Pericardial Effusion

  The major symptoms are directly related to pleural and/or pericardial chylous effusions. [jbsr.be]

  Bronchovascular bundles, parietal and visceral pleura, pericardial effusion, bilateral pleural effusion, and extrapleural soft-tissue thickening are also seen. [ajronline.org]

  There were moderate pericardial and bilateral pleural effusions. [ijri.org]

  This often leads to pleural effusion (fluid on the lungs) or pericardial effusion (fluid around the heart). KLA may also interfere with blood clotting, which can lead to life-threatening hemorrhage. [chop.edu]

It may be erroneously diagnosed as asthma responding to bronchodilator treatment, which was the reason for the late diagnosis in our case, previously diagnosed with COPD with bronchodilator treatment. [archbronconeumol.org]

The present article discusses clinical, radiographic and pathological features, and treatment options for DPL. [mayoclinic.pure.elsevier.com]

Treatment with sirolimus and prednisolone was then initiated. [ojrd.biomedcentral.com]

Talk with your doctor to find the best treatment if you or your child has been diagnosed with pulmonary lymphangiomatosis. [resourcerepository.org]

It has a favorable prognosis when it is localized, and a poor prognosis when vital organs, including vertebral medulla, are involved. 3 When the disease is diffuse and extensive in a particular organ, as in the lungs in our case, the prognosis is also [scielo.br]

Although it is a benign disease of unknown etiology and pathogeny, it presents a high rate of relapse and a poor prognosis. [archbronconeumol.org]

[…] the prognosis is generally poor. [3] There is no evidence of visceral involvement in the present cases. [cancerjournal.net]

[…] often in thoracic cavity, also bone, extremities, soft tissue, viscera Clinical features Rare, idiopathic, occurs mostly in children Cases with visceral involvement are often fatal ( Am J Surg Pathol 1993;17:329 ), but cases involving limbs have good prognosis [pathologyoutlines.com]

The systemic form which was noted in this case above differs from the localised forms of the disease – which are isolated to a single anatomic location, classically bone, and have a good prognosis. [ghrnet.org]

Etiology Most reported cases are sporadic and the etiology is not completely understood. It has been suggested that PL lymphatic channels of the fetal lung do not undergo the normal regression process at 20 weeks of gestation. [orpha.net]

The disease can be considered a failure of normal lymphatic development, but the etiology remains poorly understood. Treatment has been limited to surgical resection or drainage procedures because there is no accepted effective systemic therapy. [genome.jp]

Pulmonary lymphangioleiomyomatosis (LAM) is a benign, uncommon disorder of unknown etiology affecting women of child-bearing age ( 7 ). [atm.amegroups.com]

The proliferative lymphatic aspect suggests a neoplastic etiology, and the structural disorganization indicates a hamartomatous origin. [scielo.br]

Although it is a benign disease of unknown etiology and pathogeny, it presents a high rate of relapse and a poor prognosis. [archbronconeumol.org]

Summary Epidemiology The prevalence is unknown. [orpha.net]

Epidemiology Lymphangiomatosis may present at any age but is predominantly a condition of children and young adults, with no gender predilection 8. Clinical features Patients present with "asthma", dyspnea or haemoptysis 8 ,12. [e-immunohistochemistry.info]

There is limited information available on the pathophysiology, treatment responses, and long-term outcomes of these disorders. [atsjournals.org]

It highlights the need for further research into the pathophysiology and management of lymphangiomatosis as current treatment options are limited. http://www.liebertonline.com/doi/abs/10.1089/lrb.2011.0021 .................... [lymphedemapeople.com]

Treatment Treatment Options: No effective preventative treatment exists but individual lesions can be surgically removed when indicated. [disorders.eyes.arizona.edu]

In the case of recurring chylothorax, drainage and pleurodesis are indicated, with the option of thoracic duct ligation 3 to prevent complications such as malnutrition, pulmonary fibrosis and deteriorated respiratory function. [archbronconeumol.org]

Transplantation Substance Use and Addiction Surgery Surgical Innovation Surgical Pearls Teachable Moment Technology and Finance The Rational Clinical Examination Tobacco and e-Cigarettes Toxicology Trauma and Injury Treatment Adherence United States Preventive [jamanetwork.com]

The biologic effect of imatinib, when prescribed at higher concentrations, has been shown to inhibit angiogenesis by blocking the activity of c-kit tyrosine kinase cell surface receptor and PDGFR kinase, 22 and through this mechanism, is perhaps preventing [jnccn.org]

This procedure thereby obliterates the space between the pleura and prevents the reaccumulation of fluid. [lymphedemapeople.com]