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    Go to the top of the page   ID: 20011028095053 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    Lymphangioleiomyomatosis
    Wolfgang Hirsch, R Finke. Lymphangioleiomyomatosis. PedRad [serial online] vol 1, no. 10.
    URL: www.PedRad.info/?search=20011028095053


     
     Pediatric Radiology CasesImages to this case: There are Ultrasound-images available for this case. [ Ultrasound ] There are CT-images available for this case. [ CT ] There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    Wolfgang Hirsch, R. Finke (Halle)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    N/A  

     
     Pediatric Radiology CasesGender:

    N/A  

     
     Pediatric Radiology CasesRegion-Organ:

    Generalized diseases  

     
     Pediatric Radiology CasesMost likely etiology:

    neoplastic  

     
     Pediatric Radiology CasesHistory:

    Often by premenopausal patients, but also (rarer) in adolescence.

    The pulmonary manifestation is most common: pneumothorax, dyspnea.
    Abdominal symptoms include chronic complaints, abdominal swelling.
    Extremities: Swollon legs (vessel compression)
    Brain: Seizures (tuberous brain sclerosis)
    Skin: See phakomatoses (tuberous brain sclerosis)
    Kidney: See Angiomyolipomatosis  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    Proliferation of smooth muscle, particularly in the lung and in the lymphatic system.

    Lung: Cysts, chylo-pleural effusion, pneumothorax

    Abdomen: Lymphangioma (large, cystic, without relation to the organ), also in the liver and (as in this case) in the spleen. Sometimes vertebral lesions.

    Brain: Tuberous brain sclerosis is common.

    Kidney: Angiomyolipomatosis is common.  

     
     Pediatric Radiology CasesRadiological findings:


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Abdomen: In the abdominal space there is a poorly echogenic tumor (16%), the vessels are widened, cystic lesions in the liver as well (4%) and spleen . Ascites (10%).

    Retroperitoneum:
    Niere: (US) multiple, echogenic TM (54%).
    Retroperitoneal LKS: (US: poorly echogenic nodules in 31%)



    CT 1 <- view CT 1

    CT 1: Thorax - Pumonary window. Small or larger bullae.



    CT 2 <- view CT 2

    CT 2: Abdomen




    MRI 1 <- view MRI 1

    MRI 1: Transversal



    MRI 2 <- view MRI 2

    MRI 2: T2 with FS - Transversal



    MRI 3 <- view MRI 3

    MRI 3: T2 with FS - Transversal



    MRI 4 <- view MRI 4

    MRI 4: T1 native - Coronal






    MRI 5 <- view MRI 5

    MRI 5: Coronal

    In T2-weighted image, very high signals, T1: some contrast uptake.

    X-Ray Lungs: reticular Lung markings, bullae, effusion

    Tuberous Brain sclerosis: see there.





     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    Surgery / Histo  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    Lung cysts of any cause, also Langerhans cell histiocytosis
    Lymphangioma
    Lymphoma
    Isolated angiomyolipoma
    Isolated tuberous brain sclerosis  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    A radical, curative surgery is usually not possible. A progression of the findings is, although, often not able to be proven.  

     
     Pediatric Radiology CasesComments of the author about the case:

    We saw in adolescence (14 years of age) as of yet only one case with abdominal involvement and histologic proof.

    Pumonary involvement with cysts is probably more common, since there is no histological assurance when there is proof of cysts.  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    1. Medline: Medline
    Avila et al.
    Lymphangioleiomyomatosis: abdominopelvic CT and US findings
    Radiology 216 (147-153) (2000)
     

     
     Pediatric Radiology CasesKeywords:

    Lymphangioleiomyomatosis, smooth muscle proliferation, lung cysts, chylo-pleural effusion, chylothorax, pneumothorax, Lymphangioma, vertebal lesions, renal angiomyolipomatosis, brain tuberous sclerosis , child, childhood, pediatric radiology  

     
     Pediatric Radiology Cases Cite this article:

    Wolfgang Hirsch, R Finke. Lymphangioleiomyomatosis. PedRad [serial online] vol 1, no. 10.
    URL: www.PedRad.info/?search=20011028095053  

     
     Pediatric Radiology Cases Read similar articles: with corresponding keywords
    in the same field: Generalized diseases
    or in the region: Generalized diseases
    or in the tissue/organ:
    or with the etiology: neoplastic
     
     Pediatric Radiology CasesImages to this case: There are Ultrasound-images available for this case. [ Ultrasound ] There are CT-images available for this case. [ CT ] There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     
    Lymphangioleiomyomatosis
    Wolfgang Hirsch, R Finke. Lymphangioleiomyomatosis. PedRad [serial online] vol 1, no. 10.
    URL: www.PedRad.info/?search=20011028095053


     

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    Lymphangioleiomyomatosis
    Other cases by these authors:

    Search Wolfgang Hirsch in Medline Wolfgang Hirsch (17)   
    Search R. Finke in Medline R. Finke (1)   

    Lymphangioleiomyomatosis  
     
    Lymphangioleiomyomatosis
    Wolfgang Hirsch, R Finke. Lymphangioleiomyomatosis. PedRad [serial online] vol 1, no. 10.
    URL: www.PedRad.info/?search=20011028095053


     
     
    Lymphangioleiomyomatosis
    Wolfgang Hirsch, R Finke. Lymphangioleiomyomatosis. PedRad [serial online] vol 1, no. 10.
    URL: www.PedRad.info/?search=20011028095053


     

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    Lymphangioleiomyomatosis
    Wolfgang Hirsch, R Finke. Lymphangioleiomyomatosis. PedRad [serial online] vol 1, no. 10.
    URL: www.PedRad.info/?search=20011028095053


     




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