Clinical case of preiser disease, bilateral femoral head and humeral head osteonecrosis associated with chronic corticosteroid therapy

Abstract

Osteonecrosis is a pathologic process that is associated with numerous conditions and therapeutic interventions. Most commonly the hip is involved but almost any bone can develop osteonecrosis. It can occur in the femoral head, but also affect the femoral condyles, humeral heads, proximal tibia, vertebrae, and small bones of the hand and foot. The most frequent etiological factors are trauma, alcoholism, and chronic corticosteroid therapy; the latter causing the most devastating form of osteonecrosis. Glucocorticoid-induced osteonecrosis is a known toxicity in pediatric and young adult patients treated for Acute Lymphoblastic Leukemia (ALL) and Non-Hodgkin Lymphoma (NHL). Osteonecrosis of the shoulders and weight-bearing joints of the lower extremities has emerged as a major adverse effect of modern ALL therapy. Pain in the absence of activity (ie, rest pain) occurs in approximately two-thirds of patients, and nocturnal pain occurs in one-third. MRI continues to be the gold standard for diagnosis in symptomatic and asymptomatic patients, especially in early-stage disease. Respectively to Preiser disease, this condition is a rare disease of unknown etiology characterized by Avascular Necrosis (AVN), also known as osteonecrosis, of the scaphoid bone and the clinical manifestations include insidious and progressive pain lasting from months to years, edema, loss of strength and reduced range of motion in the wrist. The treatment of non-traumatic osteonecrosis remains one of the most controversial subjects in the orthopedic literature. The purpose of this case report is to bring to attention osteonecrosis as a potential side effect of corticosteroid use in the pediatric population, as a delayed or missed diagnosis can lead to several joints destruction.