, Volume 16, Issue 5, pp 652-655
Date: 07 May 2011

Bipolar hip arthroplasty for subtrochanteric femoral nonunion in an adult with autosomal dominant osteopetrosis type II

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Introduction

Osteopetrosis or marble bone disease is a disease of osteoclasts that results in a failure of bone remodeling [1]. It is a generalized skeletal disorder characterized by osteosclerosis, obliteration of the medullary cavity and fragility of the bones [2]. This malignant sex-linked recessive condition is associated with childhood problems and early death. However, the autosomal dominant form is compatible with a normal life span, and as many as 40% of patients may remain asymptomatic [3, 4].

Autosomal dominant osteopetrosis is divided into two subtypes (I and II) [3]. Type I is characterized by increased thickness of the cranial vault, diffuse osteosclerosis of the lumbar spine and pelvis, and symmetrical, long-bone involvement. Type II shows more basal skull involvement, a ‘Rugger-Jersey’ spine and ‘endobones’ within the pelvis. Type II is important from an orthopedic perspective because it is associated with an increased rate of fractures, which are difficult to treat [2, 5] ...