Kozanoglu Erkan, Koc Filiz, Goncu Kamil
Department of Physical Medicine, Cukurova University Medical School, Adana, Turkey.
Int J Neurosci. 2008 Apr;118(4):545-53. doi: 10.1080/00207450601050071.
Macrodystrophia lipomatosa is a rare nonhereditary congenital malformation that mainly affects mesenchymal structures. The pathology is associated with hypertrophic fibro-adipose tissues. One or more of the digits of the extremities are affected. This condition is previously described as macrodactyly, megalodactyly, or localized gigantism. This article describes a 48-year-old male patient who presented with the enlargement of unilateral (right) lower limb, especially of the first toe and tarsal tunnel syndrome. Although there is no clinically significant involvement of the upper extremities, bilateral cubital and unilateral carpal tunnel syndromes were also detected and macrodystrophia lipomatosa with multiple entrapment neuropathies was diagnosed in the patient.
巨指（趾）脂肪营养不良是一种罕见的非遗传性先天性畸形，主要影响间充质结构。其病理与肥厚性纤维脂肪组织有关。四肢的一个或多个手指或脚趾会受到影响。这种情况以前被描述为巨指（趾）、巨趾症或局限性巨人症。本文描述了一名48岁男性患者，其表现为单侧（右侧）下肢肿大，尤其是第一趾以及跗管综合征。虽然上肢无明显临床受累，但也检测到双侧肘管综合征和单侧腕管综合征，该患者被诊断为伴有多发性卡压性神经病变的巨指（趾）脂肪营养不良。
