congenital psudoarthrosis of clavicle

WELCOME TO

DAILY TOPIC PRESENTATION

Dr. Maftun Ahmed Resident NITOR

Congenital Psudoarthrosis Of

Clavicle

Daily Topic On CPC

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.

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.

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

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.

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

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

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Radiographic Features:

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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.

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.

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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.

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.

Daily Topic On CPC

References:

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

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

• Internet.

Daily Topic On CPC

THANK YOU