Idiopathic portal hypertension and lower limb lymphedema.

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Idiopathic portal hypertension and lower limb lymphedema.

Postby patoco » Sat Dec 15, 2012 12:30 pm

Idiopathic portal hypertension and lower limb lymphedema.

Jun 2012

Hara H, Mihara M, Narushima M, Iida T, Todokoro T, Yamamoto T, Okuda I, Koshima I.


Department of Plastic and Reconstructive Surgery, The University of Tokyo Hospital, Tokyo, Japan.


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.

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