Yaghmai I, McKowne F, Alizadeh A
South Med J. 1976 Dec;69(12):1565-8. doi: 10.1097/00007611-197612000-00015.
The clinical, pathologic, and roentgenographic features of macrodactylia fibrolipomatosis described are based on 40 cases from the literature and five cases seen by the authors. This condition previously has been described as macrodactyly, megalodactyly, macrodystrophia lipomatosa, dactylomegaly, and local gigantism. We believe macrodactylia fibrolipomatosis is the best term because it describes the characteristics which separate this condition from other forms of macrodactyly. Macrodactylia fibrolipomatosis is a form of macrodactyly noticed soon after birth. Usually affected are multiple adjacent digits on the lateral aspect of the hand or the medial aspect of the foot, although a single digit may be involved. Roentgenograms show bony enlargement, often with medial deviation in the hand and lateral deviation in the foot. The soft tissues ventral to the affected bones are diffusely enlarged and are predominantly of fat density, commonly mixed with bands of denser connective tissue. The cause of this rare condition is obscure but it may be related to fibrofatty tumors, often found at surgery, impinging on nerves supplying the enlarged digits.
本文所描述的纤维脂肪性巨指症的临床、病理及影像学特征基于文献报道的40例病例以及作者所诊治的5例病例。这种病症以前曾被描述为巨指（趾）症、巨趾（指）畸形、脂肪性巨营养障碍、巨指（趾）、局部巨大症。我们认为纤维脂肪性巨指症是最恰当的术语，因为它描述了该病症与其他形式的巨指（趾）症相区别的特征。纤维脂肪性巨指症是一种出生后不久即被发现的巨指（趾）症形式。通常受累的是手外侧或足内侧的多个相邻手指或足趾，不过也可能仅单个手指或足趾受累。X线片显示骨骼增大，手部常向内侧偏斜，足部则向外侧偏斜。受累骨骼腹侧的软组织弥漫性增大，主要呈脂肪密度，通常与较致密的结缔组织条带混合存在。这种罕见病症的病因尚不清楚，但可能与纤维脂肪性肿瘤有关，手术时经常发现此类肿瘤压迫供应增大手指或足趾的神经。
