• Rare autosomal dominant or sporadic osteosclerotic dysplasia• Multiple punctate sclerotic lesions representing "bone islands"or foci of compact bone located in cancellous bone• Asymptomatic• Occurs in the epiphyses and metaphyses with predilection for• Tubular bones of the hands and feet• Carpals• Tarsals• Pelvis• Scapula• Ribs, clavicles, spine, and skull are rarely involved• Usually clustered around joints• Males and females affected equally
• Buschke-Ollendorff syndrome(comprises ofosteopoikilosis associated with disseminated connectivetissue and cutaneous yellowish naevi, predominantly onthe extremities and trunk).• Gunal-Seber-Basaran syndrome : osteopoilkilosis withdacrocystitis• Mixed sclerosing bone dysplasia• Tendency to keloid formation, dwarfism, spinal stenosis,dystocia, tuberous sclerosis and scleroderma It may berelated to osteopathia striata and melorheostosis
Plain film and CT• Multiple small radiopacities found scattered in epiphyseal andmetaphyseal regions are generally pathognomonic• The bone islands of osteopoikilosis are typically clustered aroundjoints and align themselves parallel to surrounding trabeculae (thuspredominantly longitudinally in in the metaphyses). Most lesionsare found in the appendicular skeleton and pelvis. The axialskeleton is largely spared. It is rare for the skull vault to beinvolved.• The lesions vary in size, usually a 5 - 10mm, but ranging from only1 - 2mm up to 1 - 2cm.MRI• Appearances on MRI are the same as individual bone islands.Each lesion is small and dark on both T1 and T2 weighted images,as it is composed of mature dense bone.Bone scintigraphy• Bone scan should not demonstrate any increase in uptake, useful ifmetastatic disease is considered in the differential.
INVOLVEMENTOF THE HANDSAND FEET.• A. Wrist. Notethe smallroundishradiopacitiesresemblingbone islands inthe carpal andproximalmetacarpalbones.• B. Foot.Observe thatall of thevisualizedbones of thefoot showroundishradiopacities.
• PELVIC INVOLVEMENT. A. AP Pelvis. Note the diffuseinvolvement of osteopoikilosis, with radiopacitiesscattered throughout the pelvis and proximal femora. B.CT, Mid-Pelvis. Observe the small densities located in themedullary portion of the innominates
SPINALINVOLVEMENT.• A. LateralLumbar Spine.• B. Lumbar CT.Note the diffuseinvolvement ofthe lumbarvertebrae.• C. APShoulder.Observe theclusteredopacities nearthe joints in thehumerus andscapula.
When seen throughout multiple bones with characteristic appearances,there is little differential. When only a few lesions are seen on an isolatedfilm, the differential includes:• Incidental bone islands (enostoses)• Other sclerosing bone dysplasias• Sclerotic metastases• Osteosarcoma• Lymphoma• Osteoid osteoma : only rarely multiple• Chronic multifocal sclerosing osteomyelitis• Calcium and phosphate metabolism abnormalities• Erdheim-Chester disease (non-Langerhans cell, non-familialgranulomatosis)• Previous instrumentation / fractures / avascular necrosis• Pagets disease• Tuberous sclerosis• Mastocytosis