Congenital Psudoarthosis Of Radius,ulna,clavicle,congenital Dislocation Radial Head

Congenital Pseudarthosis of Radius and Ulna

Dr. Abdul Karim FCPS II Trainee PGMI/LGH, Lahore

Definition

Pseudarthosis is a false joint associated with abnormal movement at the site.



Congenital pseudarthrosis refers to a spontaneous fracture which progresses to non union.



This is rarely presented at birth but commonly developed during first 18 months of life.

Commonly affected Bone 

Tibia is the most frequently affected bone



Associated with congenital anterolateral tibial bowing in neurofibromatosis which progresses to psudoarthrosis.

Rarely affected Bones 

Fibula



Radius, Ulna



Clavicle



Femur

Radius 

Congenital pseudarthrosis of the radius/ulna is an extremely rare condition.

Genetics  



 

Autosomal dominant disorder. Neurofibromatosis type 1 (von Recklinghausen’s disease). Mutation of the NF1 gene on chromosome 17. The NF1 gene is a tumour suppressor gene; It encodes a protein, neurofibromin which modulates signal transduction through the ras GTPase pathway.



In all cases reported, Pseudarthosis of the radius occurred in the distal third.

Features 

Deformity is the most common complaint



Anterior or Posterior bowing of Radius/Ulna.



Pathological fractures.

Gender 

Male /female ratio is 8 / 1

Diagnosis 

X-Rays 

AP and Lateral view of the radius, ulna is sufficient to make accurate diagnosis.

Treatment 

Numerous treatment options have been explored with varying degrees of success and the reports have demonstrated successful healing.

Boyd  

Dual onlay bone grafting Advantages:  





Restores length Viselike grip on the osteoporotic distal fragment Increases size of distal end of proximal fragment.

Resulting in satisfactory union.

Kameyama and Ogawa 

  



Complete resection of involved radius. Periosteum and soft tissue removal. Free Vascularized fibular transfer. Operation is delayed untill skeletal maturity. Forearm brace until surgery is performed.

Ulna 

Congenital pseudarthrosis of the ulna in neurofibromatosis is extremely rare.

Literature 

Only 18 patients with NF and isolated ulna Pseudarthosis have been reported in literature.

Features 

The ulnar pseudoarthrosis produces 

Angulation of radius



Forearm shortening



Dislocation of radial head

Diagnosis 

X-Rays 

AP/lat of the radius, ulna is sufficient to make accurate diagnosis.

Treatment 

Bone grafting with or without internal fixation



Creation of one-bone forearm



Free vascularized fibular grating



Ilizarov compression-distraction technique



Radial osteotomy for correction of bowing

 

Bone grafting usually fails Significant radial bowing develops in very young children, so early surgery is indicated.

Associated Radial head dislocation 

Excision of radial head



Synostosis (one-bone forearm)

Congenital Pseudarthosis Clavicle

Congenital Pseudarthosis Clavicle    

Rare anomaly Present at birth Usually involves middle third Right side more common (80%)

Features 

Palpable and visible prominence at birth



No history of trauma



No signs and symptoms of fracture



Each end is separately mobile



Hyper mobility of the shoulder

Features 

Dropped shoulder, arm closer to midline



May be painful or painless



The main complaint is deformity



Usually right clavicle



10% bilateral, rarely left

Ethiology 

Familial (AD) transmission reported



No association with NF



Two theories

Ist Theory 

Intrinsic failure of development 

Clavical develops in two seperate masses by medial & lateral ossification centers.



Faliure of ossification of the precartilageneous bridge.

2nd Theory 

Extrinsic pressure on the clavicle 



Right side predominance result of higher subclavian artery on that side Cervical rib; pseudarthrosis at site where artery crosses the rib

Radiographic Features 

Defect in central 1/3



Bone ends usually hypertrophic



No evidence of healing or periosteal reaction

Differential Diagnosis 

Cleidocranial dysostosis



Non union after fracture clavicle.

Treatment  

Mainly operative Indication:  



Cosmetic appearance Pain

Timing of surgery 

3-6 year of age

Treatment 

Remove atrophic, sclerotic bone ends 





Suturing periosteum of ends together ORIF with plates/screws/graft

High union rate, unlike tibia

Tricortical Bone Graft 

Fracture is reduced after debriding fibrous nonunion tissue and removing atrophic, sclerotic bone ends. Resultant defect is measured, and appropriately sized tricorticocancellous bone graft is obtained from iliac crest

Congenital dislocation of radial head 



 

Congenital dislocation of radial head is rare. It should be suspected that radial head has been dislocated for a long time. Ulna fracture should be ruled out. Abnormally small and misshapen Radial head.

Etiology 

Congenital dislocation of the radial head may be familial, especially on the paternal side .



May be associated with chondroosteodystrophy.

Types 

Anterior dislocation of radial head. 

Less common



Posterior dislocation of radial head.



more common

Clinical Feature 

May be painful or painless.



Mild loss of extension/flexion.



Loss of supination.

X-ray 

The radial shaft is abnormally long.



Ulna usually is abnormally bowed.



The radial head dislocated .frequently posteriorly but some time anteriorly.



The capitellum may be small.



Radial head may be small or absent.

Treatment 

< 1 / 2yrs 

Lengthening of the ulna or shorting of the radius.



Reconstruction of the annular ligament.

Treatment



>2yrs 

Resection of the radial head.