CONGENITAL

PSEUDOARTHOSIS OF
TIBIA, FIBULA AND
CLAVICLE.

Presenter :: Dr . BN PAVAN KUMAR

Moderator :: Dr. SUNDARESH
Associate professor
History
 1891 – paget was the first to describe a case of
CPT.
 1937--Ducroquet and cottard describe
association of CPT with neurofibromatosis.
 1951—MC farland suggested bone bypass graft
 1956– charnely treated CPT with intra
medullary nail
 1985– pho et al describes efficiency of
vascularised fibular graft.
Introduction
 Congenital pseudoarthrosis of tibia and fibula
(CPT) was first described by paget in 1891 and
is characterised by a spontaneous fracture that
heals poorly with routine treatment.
 Anterolateral bowing of the tibia , the precursor
of CPT, is present at birth ,although the fracture
and the ensuing pseudoarthrosis are rerely
seen at this time .although the term congenital
pseudoarthrosis is thus a misnomer , an
alternative name for the condition has not been
forthcoming.
 Pseudoarthrosis :: this is defined as a
fracture that has undergone nonunion and
cleft develops between the bone ends
resembling joint formation with end
becoming convex and the other concave.
 The cleft or cavity gets filled with fluid and is
lined by a membrane and pseudocapsule .
The structure is hyper mobile and has no
restraints .radiologically ,the characteristic
appearance is that of ‘mortar and pestle’.
 Congenital pseudoarthrosis of tibia refers to
nonunion of a tibial fracture that develops
spontaneously or after trivial trauma in a
dysplastic bone segment of the tibial diaphysis.
 The pseudoarthrosis usually develops during
the first 2 years of life.
 Normally CPT is unilateral , located at the
junction of the middle and distal thirds of the
tibial segment with no predominance for sex or
side .
Is congenital pseudoarthrosis really
congenital.
 Congenital pseudoarthrosis of tibia is a
misnomer it is often not a congenital
one,the nonunion develops usually after
birth due to congenital ‘defect’ in tibia.
 It is revealed by either pseudoarthrosis
at birth or by a pathological fracture
presenting in bone with modifications
such as bowing ,narrowing of the
medullary canal or a cyst.
Epidemiology
 1:250000 live births
 50- 90 % associated with
neurofibromatosis
 Fibrous dysplasia is seen upto 15%of
patients
 Usually left
 Bilateral rare.
Etiology
 The exact cause of CPT is not known .
Inheritant inability of the bone to form
callus at the site of fracture.
 Aplasia/dysplasia of portion of tibial
shaft – nutritional disturbance.
 Increased intrauterine pressure
 Amniotic bands
 Associated with neurofibromatosis.
Types
 Severe neonatal forms or primary
pseudoarthrosis ,in which are present at
birth.
 Secondary pseudoarthrosis ,which is
revealed by a pathological fracture when
the child begins walking.
Pathology
 Paley et al theorised that pathology of
pseudoarthrosis is not bony but rather its
periosteal in origin.

This theory is supported by

 Thickening with hamartomatous transformation
of periosteum
 Appearance of strangulation of bone with
atrophic changes followed by avascular changes
 Failure of remodelling leading to stress fractures
 Pathology
 There is a failure of normal bone formation in the distal half of
the tibia ,resulting in segmental defect of bone, anterolateral
angulation and pathological fracture.

 The site of pseudoarthrosis is usually surrounded by a

thickened periosteum and a thick, heavy cuff of poorly
perfused hamartomatous fibrous tissue. They are said to
prevent union by a mechanical effect of interposition and be
responsible for defective vascularisation in the bone. the
periosteum could create a fibrous band causing an increase
in local pressure around the bone resulting in reduced
vascularisation as well as bone atrophy. Reduced
vascularisation could also be secondary to thickening of the
vessel walls in the area of pseudoarthrosis.
Clinical features
 Inspection ::
Anterolateral or anterior angulation
/bowing of leg usually at middle and
distal third
 Signs of neurofibromatosis 1
 Foot deformity
 May be associated with Skin dimple
over angulation ,limb shortning, Ankle
valgus
Palpation
 Thinning of tibia at angulation
 Tenderness
 Abnormal mobility
 Wasting of calf muscles
 Contracted and prominent tendoachilles
Associated problems
 Leg length discrepancy
 Multilevel multidirectional tibial deformity
 Proximal migration of the fibula
 Fibular nonunion
 Ankle mortise valgus
 Ankle joint dorsiflexion
 Valgus contracture
 Cavovalgus foot deformity
 Persistent dorsiflexion contracture before
surgery.
Radiology (AP and lateral views)
 The radiological appearance varies from
bowing of the tibia with failure of formation of
a normal medullary cavity ,cystic lesions, and
an associated incomplete or complete
fracture of the tibia with atrophy and tapering
at the site of fracture and cupped proximal
fragment and pointed distal fragment
depending on stage.
 Lesions associated with
neurofibromatosis often show a typical
appearance in which a segment of
tibia , or tibia and fibula show hourglass
thinning ,sclerosis and loss of the
medullary cavity followed by a fracture
through the dysplastic sites.
 In lesions associated with fibrous
dysplasia , there is cyst formation with
expansion of the shaft of the bone
,surrounded by sclerosis, ground glass
appearance and other feature of fibrous
dysplasia
MRI
 To know the extent of disease
 Preoperative planning in that the
borders for resection can be defined
precisely
 The area of the pseudoarthrosis is hyper
intense on fat suppressed and t2 images
and slightly hypaintens on t1 images
with contrast enhancement after
administration of gadolium.
Biopsy
 Dense cellular fibrous connective tissue
with areas of cartilage formation
 Fibroblasts rather than schwann cells or
perineural cells
 Rarely – neurofibromatosis tissue
 Hamartomatous tissue.
Classification congenital pseudoarthrosis
of tibia .
 Boyds and anderson classification
 Crawford
 paleys
Boyds classification (type 1-6)
 Type 1
Anterior bowing and defect in the tibia at birth.
 Type 2

Most common, Anterior bowing and hour glass

constriction at birth.
Tibia is tapered ,sclerotic, medullary canal
oblitered.
High risk tibia worst prognosis
Associated with neurofibromatosis
 Type 3
Congenital cyst middle & distal 1/3rd of tibia .
Anterior bowing may precede or follow fracture .
Excellent results after only one operation noted
 Type 4

Originates in the Sclerotic segment at mid &

distal 1/3rd shaft, medullary canal partially
/completely oblitered.
Stress fracture often occur and fails to heal
And eventually pseudoarthrosis occur
 Type 5
Pseudo arthrosis tibia with dysplastic fibula
Pseudo arthrosis of tibia /fibula / both
Prognosis is good if only fibula is involved.

 Type 6
Occurs as an Intraosseous neurofibroma
/schwannoma
very rare
Needs aggressive treatment
CRAWFORD
CLASSIFICATION
 Type 1
Anterolateral bowing of tibia
 Type 2

Anterolateral bowing
Increased cortical thickness
Narrow medullary canal
Tubular defect
 Type 3

cystic lesion
 Type 4

presence of fracture , a cyst or frank pseudoarthrosis

PALEY CLASSIFICATION
Management
 Aim is to achieve union
 Prevent refracture
 Correct limb length inequality
 Correct associated growth abnormalities
 Prevent ankle deformity and arthritis
Strategies to achieve union
 Microvascular free fibular transfer
 Ilizarov technique
 Bone grafting with intramedullary nailing

(excision of the pseudoarthrosis should be

an integral part of the procedure)
Strategies for minimizing the risk of
refracture
 Splint the limd in an orthosis until
skeletel maturity
 Retain an intramedullary nail until
skeletal maturity
Strategies for dealing with shortening
of the limb
 Minimize the extent of shortening by
obtaining union of the pseudoarthrosis as
early as possible.
 Establised shortening can be addressed by
limb equalization procedures
Strategies for minimizing valgus deformity
of the ankle
 Ensure union of the fibular pseudoarthrosis
 Retaining an intramedullary device that
crosses ankle joint can also prevent ankle
deformity although the motion is lost.
Prophylaxis
 Once the diagnosis of non resolving
anterolateral bowing of the tibia has
been made the first step is to prevent
fracture if possible.\
 In an infant before walking age, no
specific treatment is needed other than
education of the caretakers.
 Once the child begins to bear weight
prophylactic bracing should be
attempted altough there is no
documentation that such a program can
prevent a fracture.
 Protection of the unfractured tibia should
be continued indefinitely till skeletal
maturity or until patient approaches
skeletal maturity.
Surgical options
 Vascularised fibular graft
 External fixation
 Intramedullary device
 BMP
 Electrical stimulation
Intramedullary fixation (CHARNLEY)
 The procedure of choice for the first
attempt to gain union entails resection of
pseudoarthrosis ,shortning and fixation
with an intramedullary rod and autogenous
bone grafting.
 The procedure can be performed at any
age and rates of union of around 85%
have been reported although solid long
lasting union with out deformity is another
matter.
 Fixation by an interamedullary rode eliminate an
angulatory strain while permitting beneficial axial
compressive forces desperate the criticism that is still
allows rotational stresses.
 rodding of the tibia by the transtarsal approach is the
most commonly used method of fixation
 it can be used alone with external plaster support ,but
generally autogeneous cancellous bone or osteo-
periosteal bone transplant are added fixation tarsus will
prevent nail from cutting of short distal segment of the
tibia before introducing the nail .
 Achilles tenotomy will overcome the bowing action that
prevents correction of deformity
 mcElvenny first called attention to the
heavy cuff of tissue surrounding the bone
at pseudoarthorosis and he reasoned the
presence of these tissues , whether
congenital or a result of a fracture , may
decrease bone production and
consequently healing .
 any operation for congenital
pseudoarthorosis should include complete
excision of this tissue.
Williams technique
 Williams conceived the novel technique of
threaded male and female components of the
rod that when joined ,can be placed antegrade
through the pseudoarthrosis site and out the
bottom of the foot.
 After retrograde insertion back in to the proximal
intramedullary of the tibia the male end is
unscrewed and removed from the bottom of the
foot with the female threaded rod left
intraosseously in the tibia or across the ankle in
talus/calcaneum.
 The undesirable effect of ankle
immobilisation by IM fixation is thought
ot be necessary evil to adequately
immobilise the small distal fragment.
 As the tibia grows the foot and ankle
may eventually grow off the distal end of
the IM rod and thus allow ankle to regain
motion
 • The possibility of ankle valgus is also
almost inevitable especially if there is a
fibular pseudoarthrosis despite the
acheivement of a solid union.
 Limb length discrepancy is yet another
untoward event with shortening at maturity
averaging as much as 5 cm.
 Weak and stiff ankle and subtalar joint
secondary to cross ankle fixation producing
a poorly functioning foot.
 • The need for fibular surgery remains
controversial. Researchers concluded
that it is crucial to resect a fibular pseud-
arthrosis or, if the fibula is intact, to
perform a fibular osteotomy in order to
achieve optimal limb alignment and
union.
MCFARLAND bypass operation
 the operation for prophylactic treatment of
an imminent fracture and pseudoarthorosis
a congenital angulation of the tibia
 a single graft is placed posteriorly so as to
span the pseudoarthorosis except and the
upper and lower sites of implantation the
transplant is entirely exta-periosteal
 The operation is also used preliminary to
curettage and bone grafting of a congenital
cyst pre-pseudoarthrotic lesion
Sofield’s multiple osteotomies with
internal fixation by medullary rods:

 Fragmentation
 Reversal of fragments
 realignment
 Vascularized fibular graft

• The procedure entails harvesting a long

segment of the opposite fibula along with
its vascular pedicle.
• This is transferred into the gap created
after radical excision of the pseudarthrotic
segment.
• The vessels of the transferred fibula are
anastomosed to the local vessels. The
transferred fibula is fixed securely to the
tibia.
 • Intra medullary fixation of the donated
fibula is contraindicated theoretically
because of the possibility of disturbance of
blood supply of the microvascular graft.
 Because the transfer brings tissue with its
own blood supply, free fibular vascularised
transfer has been recommended as
procedure of choice for gaps > 3cm after
resection of pseudoarthrosis
 • Success rate for free fibula transfer is
92%- 95% if union alone is considered.
 Refracture has been reported in a third
of the patients probably a direct result of
not being able to apply a permanent
intra medullary fixation and thus ignoring
a major principle in treatment.
 • Morbidity of the donor leg—
• The distal end of fibula must be
synostosed to the tibia(Langenskiold
procedure) or the fibula of the donor site
must be reconstructed with bone graft to
prevent ankle valgus.
• In addition weakness may ensue in the
donor leg due to resection of origins of
flexor muscles.
Bone morphogenic protein

 BMP2 is useful to speed up the union

rates in CPT
 BMP 7 seems to be ineffective in the
absence of an actively differentiating
osteoblastic cell line
Electrical stimulation

 Electrical stimulation doesnot correct

existing deformities and thus its
appplication is probably limited to the
earlier phases of pseudoarthrosis
treatment when union is the primary
goal.
Amputation

 • The final function in a patient who has

undergone multiple operations but must
still protect the leg in an orthosis may
well be worse than if an earlier
amputaion and prosthetic fitting had
been performed.
Complications

 1. Refracture
 2. Malalignment of the tibia
 3. Limb length discrepancy
 4. Ankle valgus
 5. Ankle stiffness
1. Refracture.

 • 14% to 60%.
 • Anatomic alignment of the tibia and
fibula minimize the risk of re-fracture.
 • Intramedullary rod and external
bracing must be continued as effective
protection against re-fractures.
2. Malalignment of the tibia

 • Diaphyseal malalignment of the tibia

(procurvatum or valgus deformity) are
progressive and do not remodel
3. Limb length discrepancy

 • Residual limb length discrepancy following

successful union is a major problem.
 • Growth abnormalities of the tibia, fibula,
and the ipsilateral femur abnormalities are
also noted with CPT
 • which include inclination of the proximal
tibial physis, posterior bowing of the
proximal third of the tibial diaphysis,
proximal migration of the lateral malleolus.
4. Ankle valgus
 • Compromises functional outcome.
 • Progressive ankle valgus is a problematic
postoperative donor-site morbidity of a
vascularized fibular graft in children.
 • Tibiofibular metaphyseal synostosis (the
Langenskiöld procedure)
5. Ankle stiffness

 • Progressively regresses once

intramedullary rod is removed from
ankle.
 • Pain secondary to degenerative
changes of the ankle can be treated with
limitation of activity and shoe
modification.
 • Severe pain may require ankle
arthrodesis
Follow up.

 • till skeletal maturity to identify and

rectify residual problems.
Congenital Pseudoarthrosis of Clavicle
 It is the failure of the fusion of the medial and
lateral ossification centers of the clavicle.
 Diagnosis can be made with radiograph of the
clavicle showing a rounded sclerotic bone at
pseudoarthrosis site in the absence of trauma.
 Treatment is observation in the case of
minimal symptoms and cosmetic deformity
.surgical management is indicated in the event
of persistent pain or functional limitations.
Epidemiology
 Rare condition
 Predominantely most common on right
side .
 When left side is involved look for
dextrocardia.
 Bilateral in <10%
Etiology
 Pathophysiology :: postulated to be due
to extrinsic compression by the
subclavian artery.
 No clear genetic involvement.
 Not associated with neurofibromatosis
 Symptoms

 Usually asymptomatic
 On inspection

Painless nontender mass on clavicle

May be associated with winging of scapula
Radiographs

 Recommended AP view of clavicle

 Show rounded sclerotic bone at
pseudoarthrosis site.
 Lack of callous formation ( rule out
clavicle fracture)
Treatment :
 Non operative --
Observation (minimal symptoms and cosmatic
deformity)
 Operative

ORIF with iliac crest bone grafting at age 3-6 yrs

(avoid bone graft substitute )
Indications (pain, functional impairment,
cosmesis,)
 Take Home message
 • Rare.
 • The natural history of the disease is extremely unfavorable and
 • Little or no tendency for the lesion to heal spontaneously. • challenging to
treat effectively
 • Aims to obtain a long term bone union, to prevent limb length
discrepancies, to avoid mechanical axis deviation, soft tissue lesions, nearby
joint stiffness, and pathological fracture.
 • The key to get primary union is to excise hamartomatous tissue and
pathological periosteum
 . • Age at surgery, status of fibula, associated shortening, and deformities of
leg and ankle play significant role in primary union and residual challenges
after primary healing.
 • Surgical options such as intramedullary nailing, vascularized fibula graft,
and external fixator, have shown equivocal success rate in achieving primary
union • Amputation must be reserved for failed reconstruction, severe limb
length discrepancy and gross deformities of leg and ankle..