Delafargue H, Michel J, Medelsi N, Jobard P
J Radiol Electrol Med Nucl. 1977 Jun-Jul;58(6-7):465-9.
The authors report a new case of generalized congenital fibromatosis in a young white girl who presented at birth a sub-cutaneous frontal localization with osteolytic lesions. At three and a half months of age, a complete auriculo-ventricular block was discovered secondary to a myocardial localization and she died of acute respiratory failure without showing early anormal pulmonary radiological lesions. In reviewing ten cases of this generalized congenital form of the osteolytic lesions encountered, and stress the fact that the discrete radiological pulmonary signs that condition the prognosis. The then discuss the utility of distinguishing diffus and generalized forms of congenital fibromatosis.
作者报告了一例年轻白人女孩的全身性先天性纤维瘤病新病例，该女孩出生时前额皮下有溶骨性病变。在3个半月大时，因心肌受累发现了完全性房室传导阻滞，她死于急性呼吸衰竭，早期未出现肺部放射学异常病变。在回顾所遇到的10例这种伴有溶骨性病变的全身性先天性疾病病例时，强调了影响预后的离散性肺部放射学征象这一事实。然后讨论了区分先天性纤维瘤病弥漫性和全身性形式的实用性。
