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.