Goldman A B, Kaye J J
AJR Am J Roentgenol. 1977 Jan;128(1):101-5. doi: 10.2214/ajr.128.1.101.
Macrodystrophia lipomatosa is a rare form of localized gigantism characterized by progressive overgrowth of all the mesenchymal elements with a disaproportionate increase in the fibroadipose tissues. This congenital abnormality occurs most frequently in the distribution of the median nerve in the upper extremity and in the distribution of the planter nerves in the lower extremity. It is usually recognized at birth or in the neonatal period. As the patient grows, the deformity begins to mechanically interfere with joint function, vascular supply, and innervation. Six cases are described with emphasis on the radiographic findings which differentiate this entity from other forms of local gigantism. These findings include a predominately distal involvement, enlargement of the phalanges and soft tissue elements of the digit, and lucencies within the soft tissues.
巨脂性营养障碍是一种罕见的局限性巨人症，其特征是所有间充质成分进行性过度生长，纤维脂肪组织不成比例地增加。这种先天性异常最常发生在上肢正中神经分布区和下肢足底神经分布区。通常在出生时或新生儿期被发现。随着患者成长，畸形开始对关节功能、血管供应和神经支配产生机械性干扰。本文描述了6例病例，重点强调了将该病症与其他形式的局部巨人症相鉴别的影像学表现。这些表现包括主要累及远端、指骨和手指软组织成分增大以及软组织内透亮区。
