A Case of Macrodystrophia Lipomatosa of the Lower Extremity: An Effective Measuring of the Dynamic Plantar Pressure for Severe Congenital Deformity.

Kenta Ikushima, Yoshiro Abe, Hiroyuki Yamasaki, Yutaro Yamashita, Shinji Nagasaka, Masahiro Takaiwa, Ichiro Hashimoto
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Abstract

Macrodystrophia lipomatosa is a rare, congenital, non-hereditary form of localized gigantism characterized by abnormal fibroadipose tissue proliferation. Here, we report a case of a 39-year-old male with macrodystrophia lipomatosa of the whole left extremity with refractory foot ulcer. Imaging showed fibroadipose tissue proliferation and severe giant deformities of the second and third toes. The plantar pressure measurements during gait revealed that both peak pressure and pressure-time integral around the ulcer were increased; therefore, the forefoot deformities might have caused the ulceration. To decrease and redistribute the plantar pressure, debulking the forefoot soft tissue, involving the second and third toe amputation, and cutting the flexor tendon of the fifth toe of severe flexion contracture were performed. No recurrences were found 6 months after surgery. The plantar pressure measurements helped in planning surgical strategies, and we evaluated the loading condition of the foot sole despite a severe congenital deformity.

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下肢巨营养不良性脂肪瘤1例:重度先天性畸形动态足底压力的有效测量。
巨营养不良性脂肪瘤是一种罕见的、先天性的、非遗传性的局部巨人症,其特征是异常的纤维脂肪组织增生。在此,我们报告一位39岁男性,患有整个左下肢巨营养不良性脂肪瘤,并伴有难治性足部溃疡。影像显示纤维脂肪组织增生,第二和第三趾严重畸形。步态时足底压力测量显示,溃疡周围的峰值压力和压力-时间积分均增加;因此,前足畸形可能导致溃疡。为了减少和重新分配足底压力,对前足软组织进行减压,包括第二和第三趾截肢,并切断严重屈曲挛缩的第五趾屈肌腱。术后6个月无复发。足底压力测量有助于制定手术策略,我们评估了严重先天性畸形的足底负荷状况。
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