Document Detail

Idiopathic portal hypertension and lower limb lymphedema.
MedLine Citation:
PMID:  23057151     Owner:  NLM     Status:  In-Process    
Idiopathic lymphedema is a condition where lymph accumulates in subcutaneous tissue without a clear cause. Likewise, idiopathic portal hypertension is a syndrome where intrahepatic peripheral portal branch obstruction causes portal hypertension without a clear cause. We encountered a 37-year-old man with both idiopathic lymphedema and idiopathic portal hypertension. He had a history of right lower limb edema and epigastric varices since childhood with repeated cellulitis in the affected limb. Lymph accumulation and dilation of collateral lymph pathways in the right lower limb were observed by indocyanine green and lymphoscintigraphy, and a serpentine thoracic duct was observed using MRI. Idiopathic portal hypertension and idiopathic lymphedema were diagnosed, and peripheral lymphaticovenous anastomosis was performed for treatment of lymphedema. The limb circumference improved, and the frequency of cellulitis decreased. It is postulated that an abnormality in the embryonic cardinal vein before lymph vessel differentiation could be a possible mechanism of the dual pathologic conditions.
H Hara; M Mihara; M Narushima; T Iida; T Todokoro; T Yamamoto; I Okuda; I Koshima
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Lymphology     Volume:  45     ISSN:  0024-7766     ISO Abbreviation:  Lymphology     Publication Date:  2012 Jun 
Date Detail:
Created Date:  2012-10-12     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0155112     Medline TA:  Lymphology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  63-70     Citation Subset:  IM    
Department of Plastic and Reconstructive Surgery, The University of Tokyo Hospital, Tokyo, Japan.
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