2004 Texas Radiological Society UNKNOWN CASES Pediatric

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2004 Texas Radiological SocietyUNKNOWN
CASESPediatric

• Justin Q. Ly, MD
• Douglas P. Beall, MD
• Wilford Hall Medical Center
• San Antonio, TX

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PEDIATRIC

• 9-month-old male
• Fever
• CXR to r/o pneumonia

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2004 Texas Radiological SocietyDiagnosis
forPediatrics Case

• Justin Q. Ly, MD
• Douglas P. Beall, MD
• Wilford Hall Medical Center
• San Antonio, TX

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Imaging Findings

• Metaphyseal/dipah notching or radiolucent columns
  
• Sparing of epiphyses and physes
• Hand, feet, pelvis involvement
• Diffuse rib end enlargement
• Fan-like notching of iliac crests

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DX Olliers Disease

• DDX
• Metaphyseal dysostosis (pelvis spared, symmetric
  involvement)
• Maffucis syndrome (phlebs/soft tissue masses)
• Fibrous dysplasia (does not typically affect
  hands/feet)
• Mets (older pts, more random distribution)
• Langerhans Cell Histiocytosis (occurs in older
  children)

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Same pt 1.5 yrs later

• Notched lucencies involving iliac crest creating
  fanlike pattern pathognomonic

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Olliers Disease

• Aka Mult Enchondromatosis
• Nonheredit, congenital
• Cause derangement of cartilagenous growth
  resulting in migration of cartilagenous rests
  from epiphyseal plate into metaphysis where they
  prolif
• Presents in early childhood
• Predom unilateral/monomelic
• Diaphysis / metaphysis of tubular or long bones
• A/w limb shortening (impaired of epiphyseal
  fusion), bowing deformity long bones, hand/ft
  deformity
• Complications
• Spontaneous fxs
• Risk malig transformation to chondrosarc/osteosarc
  (25-30)

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Olliers Disease

• XR
• Well-demarcated rounded radiolucencies / columnar
  streaks of decreased density from epiphyseal
  plate into diaphysis of long bones (cartilagenous
  rests)
• Exspansile remodeling of affected bone

Bulbous rib ends believed to be due to
proliferation of cartilagenous rests
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2004 Texas Radiological SocietyUNKNOWN CASES

• Justin Q. Ly, MD
• Douglas P. Beall, MD
• Wilford Hall Medical Center
• San Antonio, TX

This case has been previously reported Ly JQ,
Beal DP. A rare case of infantile Ollier's
disease demonstrating bilaterally symmetric
extremity involvement. Skeletal Radiol 2003
Apr32(4)227-30.
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