congenital psudoarthrosis of clavicle

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Page 1: Congenital Psudoarthrosis of  clavicle

WELCOME TO

DAILY TOPIC PRESENTATION

Page 2: Congenital Psudoarthrosis of  clavicle

Dr. Maftun Ahmed Resident NITOR

Congenital Psudoarthrosis Of

Clavicle

Daily Topic On CPC

Page 3: Congenital Psudoarthrosis of  clavicle

Daily Topic On CPC

Introduction:

• Congenital pseudarthrosis of the clavicle (CPC) is a rare condition of unknown etiopathogenesis, not associated with any H/O birth injury.

• First reported by Fitzwilliams in 1910, since then about 200 cases have been reported in literature in last hundred years.

Page 4: Congenital Psudoarthrosis of  clavicle

Daily Topic On CPC

Introduction: (cont)

• usually unilateral.• Right side is mostly affected.• Left sided unilateral lesions: associated with

Dextrocardia.• During childhood, function is unaffected and

less symptoms • A relative female predominance (70%) was

also observed.

Page 5: Congenital Psudoarthrosis of  clavicle

Daily Topic On CPC

Etiology:

• Two major theories are• Intrinsic failure of devolopment related to the

embryogenesis of the clavicle (Fawcett 1913)

• Extrinsic pressure on Clavicle :Most authors believe that the lesion is caused by pressure exerted upon the developing clavicle by the pulsating subclavian artery.

• Unknown

Page 6: Congenital Psudoarthrosis of  clavicle

Daily Topic On CPC

Etiology: (cont.)

Intrinsic:• 2 primary ossification centres are

formed, one medial and the other lateral, in the 5th and 6th gestational weeks and fuse during 7th week of fetal development,

• Interrupted formation of the two primary ossification centres and failure of these to coalesce at 7th week could be responsible for clavicular pseudarthrosis.

Page 7: Congenital Psudoarthrosis of  clavicle

Daily Topic On CPC

Extrinsic:• Lloyd-Roberts observed that Right

sided predominance may be result of higher Subclavian artery on right side

• This causing extrinsic pressure on the budding clavicle by the adjacent pulsatile subclavian artery during fetal devolopment.

• Others: Cervical ribs

Page 8: Congenital Psudoarthrosis of  clavicle

Daily Topic On CPC

Clinical Features:

• Painless prominence in the middle of the clavicle at birth or in early neonatal life.

• End of the clavicular segments are enlarged at the site of the pseudarthrosis and there is some degree of motion between them.

• The skin above the prominence may become atrophic

Page 9: Congenital Psudoarthrosis of  clavicle

Daily Topic On CPC

Radiographic Features:

Page 10: Congenital Psudoarthrosis of  clavicle

Daily Topic On CPC

Differential Diagnosis:

• Cleidocranial dysostosis: which is characterised by hypoplasia / aplasia of the

lateral clavicular ends, retarded cranial ossification, supernumerary teeth and short stature.

• Dissimilar location (lateral involvement), tapering of both bone ends and co-existing structural abnormalities distinguish itself from congenital pseudarthrosis of Clavicle.

Page 11: Congenital Psudoarthrosis of  clavicle

Daily Topic On CPC

Differentials: cont.

• Post-traumatic pseudarthrosis: Usually a history of trauma or birth injury, Always painful and tender on pressure with motion

between fragments. The radiograph shows exuberant callus formation.

• Neurofibromatosis. There are no cafe au lait spots Ends of the segmems are seen to be enlarged (bulbous) in cases of congenital pseudarthrosis whereas

in cases of neurofibromatosis they are tapered.

Page 12: Congenital Psudoarthrosis of  clavicle

Daily Topic On CPC

Treatment:• In most series the preferred treatment has been

surgery.• Undertaken either at the parents' request or in order

to strengthen the shoulder or remove the lump.

• Surgery should be delayed until the patient is at least of preschool age.

• Excision of the pseudarthrosis combined with cancellocortical onlay grafts is favoured.

Page 13: Congenital Psudoarthrosis of  clavicle

Daily Topic On CPC

Treatment: (cont.)

• Using an External fixator is another possible technique.

• Since aesthetics are the major concern, the surgery is performed during childhood with an external fixator, yielding better cosmetic results with smaller postoperative scars

• avoiding the need for a second surgical procedure to remove the implants.

Page 14: Congenital Psudoarthrosis of  clavicle

Daily Topic On CPC

References:

• Lovell & Winter’s Paediatric Orthopaedics, 4th Edition,

• Sharrard’s Paediatric Orthopedics and Fracture, Vol.1, 2nd Edition

• Internet.

Page 15: Congenital Psudoarthrosis of  clavicle

Daily Topic On CPC

THANK YOU