Complication Of Osteopetrosis Research Articles

Alberg's Schoenberg Disease – A Review and Case report

Osteopetrosis is a rare hereditary bone disease characterized by a abnormal overgrowth of dense bone because of osteoclasts malfunction, impaired bone resorption, decrease in the rate of bone turnover and formation of immature bone. There are a number of clinical variants with significantly different prognosis and clinical behaviours that have been described in the literature. The diagnosis of Osteopetrosis is largely based on the clinical and radiographic evaluation, and confirmed by gene testing. Treatment for osteopetrosis is largely symptomatic, however haematopoietic stem cell transplantation is currently the best chance for the long term survival of the patient. Osteomyelitis is a known complication of osteopetrosis. A prompt diagnosis, appropriate treatment and a continuous follow up decreases the long-term sequelae of the disease. This article emphasis on the signs and symptoms, clinical and radiographic features of Osteopetrosis and gives an insight on a case diagnosed with Osteopetrosis in an eight year old female patient and about the treatment rendered.

Maxillary osteomyelitis in Albers-Schönberg disease: A rare presentation

Osteopetrosis (Albers-Schonberg disease) is a rare congenital disorder in which the bones become overly dense. This results from an imbalance between the formation of bone and the breakdown of bone. Osteomyelitis is well-documented as a complication of osteopetrosis. Decreased vascularity of the involved bones predisposes the patient to the development of osteomyelitis. Several case reports have been published of infectious complications affecting the mandible but maxillary infection is quite rare. This discusses a case of osteopetrosis with maxillary osteomyelitis as a complication in a 13-year-old boy.

Osteopetrosis with osteomyelitis of mandible: A case report

Osteopetrosis is a rare genetic disorder caused by congenital defect in the development or function of the osteoclasts, resulting in fragile bones which are sensitive to fracture and infection. Mandibular osteomyelitis is an important and well documented complication of osteopetrosis. This case report discusses a case of osteopetrosis with chronic osteomyelitis of the mandible and increased fetal haemoglobin in a 6-year old girl.

Osteopetrosis: Report of a Rare Case

Osteopetrosis is a rare genetic disorder that causes generalized sclerosis of bone due to a defect in bone resorption and remodeling. Osteomyelitis is well documented as a complication of osteopetrosis. This disease can be severe and difficult to treat in the osteopetrotic patient. Prevalence is about 0.005% in general population. There are relatively few cases reported of osteopetrosis and its actual cause is not known. Dental abnormalities that present radiographically are delayed eruption and missing teeth. The overall clinical and radiographic findings of this patient were sufficient to arrive at the diagnosis of osteopetrosis.

Computed tomography of intrathoracic extramedullary haematopoiesis occurring as a complication of osteopetrosis

Intrathoracic extramedullary haematopoiesis occurring as a complication of osteopetrosis is uncommon. The combination of classic plain film and CT findings should suggest the diagnosis, which can be non-invasively confirmed with bone marrow scintigraphy.

TOTAL HIP ARTHROPLASTY IN OSTEOPETROSIS – REPORT OF A CASE

Background. Authors introduced a case of a 27-year men with osteopetrosis with hip arthrosis. He has an autosomal – dominant form of disease and he needed total hip arthroplasty. There are seven cases described in literature with developed hip arthrosis by osteopetrosis. Methods. TEP implantation represents the greates surgical challenge in this patients, especially creation of intramedullary canal in femur and implantation of the femoral stem because of the very dense and brittle bones. This article describes the operative technique used in the case involved. It proved to be a very good solution. Results. The patient was released from the hospital ten days after implantation of total hip endoprosthesis. Three months after the operation the patient started to walk without aid of canes, he had non pain, and his range of motion was almost normal. Conclusions. Severe coxarthrosis is a rare complication of osteopetrosis. Great care must be taken with implantation of total hip endoprosthesis, especially with preparation of medular canal. It was recommended hand drilling under x-rays to exercise maximal control because reaming can cause false root of stem and greater probability of fracture.