1. Dr. Nikhil Murkey

2. • 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

3. • Buschke-Ollendorff syndrome(comprises of
osteopoikilosis associated with disseminated connective
tissue and cutaneous yellowish naevi, predominantly on
the extremities and trunk).
• Gunal-Seber-Basaran syndrome : osteopoilkilosis with
dacrocystitis
• Mixed sclerosing bone dysplasia
• Tendency to keloid formation, dwarfism, spinal stenosis,
dystocia, tuberous sclerosis and scleroderma It may be
related to osteopathia striata and melorheostosis

5. Plain film and CT
• Multiple small radiopacities found scattered in epiphyseal and
metaphyseal regions are generally pathognomonic
• The bone islands of osteopoikilosis are typically clustered around
joints and align themselves parallel to surrounding trabeculae (thus
predominantly longitudinally in in the metaphyses). 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 - 10mm, but ranging from only
1 - 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 if
metastatic disease is considered in the differential.

6. INVOLVEMENT
OF THE HANDS
AND FEET.
• A. Wrist. Note
the small
roundish
radiopacities
resembling
bone islands in
the carpal and
proximal
metacarpal
bones.
• B. Foot.
Observe that
all of the
visualized
bones of the
foot show
roundish
radiopacities.

7. • PELVIC INVOLVEMENT. A. AP Pelvis. Note the diffuse
involvement of osteopoikilosis, with radiopacities
scattered throughout the pelvis and proximal femora. B.
CT, Mid-Pelvis. Observe the small densities located in the
medullary portion of the innominates

8. SPINAL
INVOLVEMENT.
• A. Lateral
Lumbar Spine.
• B. Lumbar CT.
Note the diffuse
involvement of
the lumbar
vertebrae.
• C. AP
Shoulder.
Observe the
clustered
opacities near
the joints in the
humerus and
scapula.

13. 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 dysplasia's
• Sclerotic metastases
• Osteosarcoma
• Lymphoma
• Osteoid osteoma : only rarely multiple
• Chronic multifocal sclerosing osteomyelitis
• Calcium and phosphate metabolism abnormalities
• Erdheim-Chester disease (non-Langerhan's cell, non-familial
granulomatosis)
• Previous instrumentation / fractures / avascular necrosis
• Paget's disease
• Tuberous sclerosis
• Mastocytosis