الموقع الرسمي للدكتور أحمد رفاعي

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Case : 5

1/10/2013

7 Comments

 
Picture







Technique :

Axial CT study for the brain with IV contrast administration

 Findings :
 Calcified subependymal tubers are seen confirming the diagnosis
of tuberous sclerosis

 Diagnosis : tuberous sclerosis

 Teaching points :
·    One of the phacomatosis( neuroectodermal disorders ) characterized by hamartomatous tumors + malformations occurring in brain, eyes, bone,  kidneys, lung, skin, heart and visceral involvement .
 ·    Classic clinical triad ( in only 30%) : seizures, mental retardation, and adenoma sebaceum
·      Brain involvemet: subependymal tubers ( pathognomonic finding ), giant cell astrocytoma at the region of foramen of Monro, cortical and subcortical tubers and gray matter heterotopia.
·      Renal involvement: angiomyolipoma ( 40-90%) , simple cysts ( 15%),and renal cell carcinoma (1-3%)
 ·      Ocular involvement: retinal hamartoma, optic nerve glioma
 ·      Lung involvement:interstitial lung disease, lymphangioleiomyomatosis, pneumothorax, and chylothorax
 ·      Heart involvement: cardiomyopathy, subendocardial rhabdomyoma 
·      Bone involvement: bone islands in pelvic brim, vertebrae, long bones,sclerotic calvarial patches and bone cysts.
·     Visceral involvement: multiple lipomas of liver, adenomas of pancreas and tumors of spleen.


7 Comments
Ahmad
1/9/2013 05:48:06 pm

Very interesting case, thank you Dr Ahmad

Reply
M A Farooqui
1/9/2013 09:50:11 pm

Thanks for a nice case. Can the liver lesions be biliary hamartomas ?

Reply
javed
1/11/2013 07:09:05 pm

excellent exercise ,,,,,, thank you Dr.Ahmed

Reply
Rania
1/12/2013 01:20:31 pm

Thanks dr Ahmed very nice case, but the sclerotic lesions in skeleton of what

Reply
Rania
1/12/2013 01:22:02 pm

Oh thanks I got it it s bone islands thanks again

Reply
shereen anwer
1/16/2013 07:18:34 am

very nice and interesting case dr ahmed , thank you very much.

Reply
Hamada
2/28/2013 01:31:52 pm

very nice and interesting case Dr Ahmed, thank you very much.

Reply



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