Radiological imaging of Osteopoikilosis.

Dr/ ABD ALLAH NAZEER. MD.

Osteopoikilosis is a sclerosing bony dysplasia characterized by multiple benign enostosis. It is a rare inherited benign condition incidentally found on skeletal x-rays. Its importance is predominantly in correct diagnosis so that it is not mistaken for pathology.EpidemiologyThe bone islands of osteopoikilosis develop during childhood and do not regress and therefore are seen in all age groups. There is no gender predilection.Osteopoikilosis is inherited as an autosomal dominant disorder. Clinical FindingsAsymptomaticDiagnosis is usually made incidentally.

PathologyHistologically the bone islands found in osteopoikilosis and in sporadic enostosis are merely patches of dense cortical like bone complete with haversian canals located within the spongiosa, often just deep to the cortex.A closely related entity is Buschke-Ollendorff syndrome.Osteopoikilosis is often found concurrently with osteopathia striata, and melorheostosis, and it is thought by some that they represent a spectrum of the same condition. Indeed recent genetic evidence suggests that these conditions are related by a loss of function mutation of the LEMD3 gene.Other associationsGunal-Seber-Basaran syndrome: osteopoikilosis with dacrocystitismixed sclerosing bone dysplasia: concurrent osteopoikilosis, osteopathia striata and melorheostosis.sclerodermatendency to keloid formation

Radiographic featuresPlain film and CTThe bone islands of osteopoikilosis are typically clustered around joints and align themselves parallel to surrounding trabeculae (thus predominantly longitudinally in the epiphyses and metaphases). Most lesions are found in the appendicular skeleton and pelvis. The axial skeleton is largely spared. It is rare for the skull vault to be involved.The lesions vary in size, usually a 5-10 mm, but ranging from only 1-2 mm up to 1-2 cm. MRIAppearances 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 scintigraphyBone scan should not demonstrate any increase in uptake, useful if metastatic disease is considered in the differential.Osteopoikilosis is one of the skeletal “don’t touch” lesions

Differential diagnosisWhen seen throughout multiple bones with characteristic appearances, there is little differential. When only a few lesions are seen on an isolated film, the differential includes:incidental bone islands (enostoses) other sclerosing bone dysplasiassclerotic metastases (rarely involve epiphyses)osteosarcomalymphomaosteoid osteoma: only rarely multiplechronic multifocal sclerosing osteomyelitiscalcium and phosphate metabolism abnormalitiesErdheim-Chester diseaseprevious instrumentation/fractures/avascular necrosisPaget disease

Radiograph both wrists PA: Osteopoikilosis multiple, small sclerotic lesions in periarticular distribution involving the distal radius and ulna, carpals and the metacarpals noted.

Frontal radiograph of the right shoulder revealing Osteopoikilosis with multiple periarticular, small sclerotic lesions involving the humerus and scapula.

Osteopoikilosis. Black arrows point to numerous sclerotic bone islands surrounding the hip joints in a pattern characteristic of osteopoikilosis.

Radiographic findings diagnostic of osteopoikilosis include the appearance of numerous small, well-defined, spheroid sclerotic foci clustered in periarticular regions in the shoulder (A), ankle (B), and pelvis (C).

Ovoid, well-defined sclerotic foci , which are small and symmetrically distributed, are seen on the pelvic and hand radiographs. Appearance is in favor of osteopoikilosis.

Sclerotic changes in the hands, feet, and pelvis of the osteopoikilosis.

CT images of the same patient show the well-circumscribed lesions in the femurs and pelvis.

Unenhanced CT of the pelvis shows Osteopoikilosis with multiple, small, discrete, sclerotic lesions in symmetric periarticular distribution of hip and SI joints.

Computed tomography of the ankle: anteroposterior and lateral radiographs of the ankle. Sclerotic foci of variable size appear in the tibia and calcaneum

Osteopoikilosis.

CT scan of pelvis in a patient with osteopoikilosis.

AP plain film radiograph and coronal section MR sequence of the left elbow showing osteopoikilosis.

Osteopoikilosis MRI of the ankle for research of a posterior impingement.

Osteopoikilosis - MRI features.

There are numerous lesions within the marrow demonstrating decreased T1 and T2 signal. This is most likely thought to be osteopoikilosis.

OSTEOPOIKILOSIS SHOULDER.

From the case osteopoikilosis.

Osteopoikilosis, A) Normal whole body bone scintigraphy and B) Multiple, symmetric sclerotic lesions in iliac bones at pelvic SPECT-CT.

Innumerable Small Bony Nodular Sclerotic Lesions with Negative Findings on Both Bone Scintigraphy and F-18 FDG PET : Osteopoikilosis

Thank You.