Department of Plastic and Reconstructive Surgery, The University of Tokyo Hospital, Tokyo, Japan.
Lymphology. 2012 Jun;45(2):63-70.
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.
特发性淋巴水肿是一种原因不明的淋巴在皮下组织积聚的疾病。同样，特发性门静脉高压症是一种综合征，其特征为肝内周围门静脉分支阻塞引起门静脉高压，而无明确病因。我们遇到一位 37 岁男性，同时患有特发性淋巴水肿和特发性门静脉高压症。他自幼出现右下肢水肿和上腹部静脉曲张，受累肢体反复发生蜂窝织炎。靛氰绿和淋巴闪烁显像观察到右下肢淋巴积聚和侧支淋巴通路扩张，MRI 观察到蛇形胸导管。诊断为特发性门静脉高压症和特发性淋巴水肿，并进行外周淋巴管静脉吻合术治疗淋巴水肿。肢体周长改善，蜂窝织炎发作频率降低。推测在淋巴管分化之前胚胎心静脉的异常可能是两种病理情况的可能机制。
