Infantile malignant osteopetrosis (IMO) is a rare congenital disease that is characterized by an impaired function or differentiation of osteoclasts. IMO is the most severe type of osteopetrosis. Patients usually present various fatal manifestations soon after birth and die in infancy or childhood. Clinical features include bone marrow failure resulting in pancytopenia, hepatosplenomegaly, blin...

INTRODUCTION Osteopetrosis introduces technical limitations to the traditional treatment of fracture management that may be minimised with specific pre-operative planning. Extreme care and caution are required when drilling, reaming, or inserting implants in patients with osteopetrosis. Caution must be exercised throughout the postoperative course when these patients are at greatest risk for de...

Temporal bone osteodysplasia can produce many different symptoms, such as involvement restricted to the temporal bone or impairment of other bones. We consider, in this study two entities that are rare osteodysplasia cases, which are osteopetrosis and Camurati-Engelmann disease, the latter being extremely rare. We present two cases of benign form of osteopetrosis (Albers-Schulenburg's disease),...

Case 1. A full-term male child, weighing 6j lb., the product of a second pregnancy, was born on Aug. 3, 1945. The parents were unrelated. When three months old the child was thought to be blind, and two months later on x-ray examination a diagnosis of osteopetrosis was made. Mental retardation was noted at ten months. At two years two months he was admitted to the Royal Blind School; he was a w...

Osteopetrosis is an inherited bone disorder characterized by general skeletal sclerosis. The cortex and spongiosa are increased in thickness and density. and the bone marrow spaces are reduced in volume [1]. Histopathologically, excessive retention of chondroosseous tissue and failure of remodeling of bones are the result of retarded osteocytic osteolysis and osteocytic chondrolysis [2] . Signs...