IDIOPATHIC PORTAL HYPERTENSION AND LOWER LIMB LYMPHEDEMA

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

Abstract


Idiopathic lymphedema is a conditionwhere lymph accumulates in subcutaneoustissue without a clear cause. Likewise,idiopathic portal hypertension is a syndromewhere intrahepatic peripheral portal branchobstruction causes portal hypertensionwithout a clear cause. We encountered a37-year-old man with both idiopathic lymphedemaand idiopathic portal hypertension.He had a history of right lower limb edemaand epigastric varices since childhood withrepeated cellulitis in the affected limb. Lymphaccumulation and dilation of collateral lymphpathways in the right lower limb were observedby indocyanine green and lymphoscintigraphy,and a serpentine thoracic duct was observedusing MRI. Idiopathic portal hypertensionand idiopathic lymphedema were diagnosed,and peripheral lymphaticovenous anastomosiswas performed for treatment of lymphedema.The limb circumference improved, and thefrequency of cellulitis decreased. It is postulatedthat an abnormality in the embryoniccardinal vein before lymph vessel differentiationcould be a possible mechanism of thedual pathologic conditions.

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