SPINAL-CANAL INVOLVEMENT IN INFANTILE MYOFIBROMATOSIS - CASE-REPORT AND REVIEW OF THE LITERATURE

Purpose: An unusual case of infantile myofibromatosis with spinal cana l involvement is reported and the literature is reviewed. Patient and Methods: A female neonate had bladder and bowel dysfunction and paresi s of the lower extremities. Results: Intrapelvic and paravertebral mas ses with extension into the spinal canal were detected by imaging stud ies. In addition, radiologic examination showed multiple metaphyseal r adiolucent lesions of the long bones and pathologic fracture of the le ft femur. The histopathologic diagnosis of the paravertebral tumor and bone lesions was infantile myofibromatosis. Surgical resection of the paravertebral and intrapelvic masses was performed to improve her neu rologic impairments. Paresis of the lower limbs gradually improved, wh ereas bladder and bowel dysfunction remained unchanged. Conclusions: O nly six cases of infantile myofibromatosis associated with spinal cana l involvement have been reported. Three patients with flaccid paresis of extremities and respiratory distress died in the newborn period. Th e other three patients showed improvement of the paresis. The prognosi s of infantile myofibromatosis without visceral complication is genera lly good, but neurologic impairment may occur at birth if the spinal c ord is compressed.