Wolfgang Hirsch, R. Finke (Halle)  

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Generalized diseases  

neoplastic  

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  

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.  

<- 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%)



<- view CT 1

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



<- view CT 2

CT 2: Abdomen




<- view MRI 1

MRI 1: Transversal



<- view MRI 2

MRI 2: T2 with FS - Transversal



<- view MRI 3

MRI 3: T2 with FS - Transversal



<- view MRI 4

MRI 4: T1 native - Coronal






<- 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.





 

Surgery / Histo  

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

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

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.  

N/A  

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

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

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