Universidad Médica Pinareña (Sep 2018)
Fibrous dysplasia of the tibia in a patient with neurofibromatosis type 1
Abstract
Introduction: fibrous dysplasia is a metabolic bone disease characterized by an accelerated bone resorption, resulting in a bone with less mechanical resistance. It is considered a nonneoplasticdisorder that simulates a bone tumor. The origin of this disease remains uncertain, although genetic or environmental factors are implicated in its etiology. It is among the rarest onset of neurofibromatosis type 1. Case report:a 7-year-old schoolgirl with a history of neurofibromatosis type 1, who attended the Orthopedics Emergency Room at Pepe Portilla Provincial Pediatric Hospital in Pinar del Río, with a pathological fracture of the left tibia. A diagnosis of fibrous bone dysplasia secondary to neurofibromatosis type 1 was completed and immobilization treatment was initiated. Conclusions:fibrous bone dysplasia is a rare indicator of neurofibromatosis type 1. This condition has several types of presentation, which depends on the prognosis and the evolution of patients. Its treatment includes several objectives and the alternative of its management depends on several factors.