Health &medicine.

1. Radiological imaging of Osteopoikilosis.
Dr/ ABD ALLAH NAZEER. MD.

2. 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.
Epidemiology
The 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 Findings
Asymptomatic
Diagnosis is usually made incidentally.

3. Pathology
Histologically 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 associations
Gunal-Seber-Basaran syndrome: osteopoikilosis with dacrocystitis
mixed sclerosing bone dysplasia: concurrent osteopoikilosis,
osteopathia striata and melorheostosis.
scleroderma
tendency to keloid formation

4. Radiographic features
Plain film and CT
The 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.
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 if
metastatic disease is considered in the differential.
Osteopoikilosis is one of the skeletal “don’t touch” lesions

5. Differential diagnosis
When 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 dysplasias
sclerotic metastases (rarely involve epiphyses)
osteosarcoma
lymphoma
osteoid osteoma: only rarely multiple
chronic multifocal sclerosing osteomyelitis
calcium and phosphate metabolism abnormalities
Erdheim-Chester disease
previous instrumentation/fractures/avascular necrosis
Paget disease

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

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

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

9. 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).

10. 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.

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

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

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

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

15. Osteopoikilosis.

16. CT scan of pelvis in a patient with osteopoikilosis.

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

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

19. Osteopoikilosis - MRI features.

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

21. OSTEOPOIKILOSIS SHOULDER.

22. From the case osteopoikilosis.

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

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

25. Thank You.