Congenital Generalized Fibromatosis; A Case Limited to Osseous Lesions

Abstract

A rare case of congenital generalized fibromatosis, apparently limited to the skeleton, has been described. A presenting fracture through a distal femoral lesion healed rapidly and uneventfully in Bryant’s traction. The subsequent course was that of complete regression of all lesions within six to nine months.

Case Details

Personal Characteristics

A white boy, b. C., was born on september 10, 1967, and was first seen by us on january 17, 1968. He was born after a full-term uncomplicated pregnancy and delivery, and his birth weight was 2.89 kilograms. The neonatal period was normal and the child thrived. Subsequent inquiry as to maternal illness, use of drugs during pregnancy, or unusual environmental stresses was negative. The child was breast fed and later had normal diet and vitamin administration.

Clinical Characteristics

On january 17, 1968, the patient fell 60.9 centimeters from a couch and had immediate pain and deformity of the right femur. Roentgenograms revealed a fracture through a cystic lesion in the distal part of the right femur. Multiple similar lesions were discovered throughout the skeleton.

Remission Characteristics

The subsequent course was that of complete regression of all lesions within six to nine months.

Treatment & Mechanisms

Proposed Remission Mechanisms

Not discussed

Clinical Treatment

The child was placed in bryant’s traction. On the day following admission, january 18, 1968, a biopsy of the proximal lesion of the left tibia was performed under general anesthesia. On february 16, 1968, we curetted the lesions in the proximal and distal parts of the left femur and tibia and packed them with bank bone (frozen irradiated cancellous homograft).

Additional Notes

Over 100 separate bone lesions were easily identified. The initial impression on review of the pathological sections was that the diagnosis was neurofibroma. This has been the common diagnosis in other reported cases of this disorder. However, the fracture healed rapidly and the case was thought to be clinically inconsistent with neurofibromatosis. After considerable discussion, it was decided to attempt to ablate the major lesions of the lower extremity to try to avoid major deformities that might occur if there were repeated fractures, as expected, through the obviously weakened segments.