congenital pseudoarthrosis of the tibia

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JOURNAL READING

Epidemiology and Treatment Outcome ofCongenital Peudart!roi

of t!e Ti"ia

Joachim Horn • Harald Steen • Terje Terjesen

O##a Prima Ad!i!arta$% & '%% & ()*$

+uper,ior - dr& .inu /urti0 +p&OT



Epidemiological data are limited and

treatment of the condition is challenging. The

purpose of our study was to gain

epidemiological data on the incidence of CPT

in Norway and to evaluate the treatment

outcome of the disease.

ABSTRACT

Congenital pseudarthrosis ofthe tibia (CPT)



ABSTRACT

11 boys, 11 girls; mean age 15 years, age range !"# years.

Primary surgical treatment was the $li%aro& method in 15

patients, intramedullary nailing in three patients, and plate

osteosynthesis in two patients

22 patients with CPT

Primary surgical treatment



INTRODUCTION

Congenital pseudarthrosis ofthe tibia *CPT+ is a rare disease

with a &ariable history and

appearance. Pseudarthrosis in

most cases of CPT are not present

at birth. Therefore the term

pseudarthrosis-- might be

somewhat inaccurate, and

dysplasia would be the preferred

term.



55( of the cases ofanterolateral bowing and

thera are pseudarthrosis

associated withneurofibromatosis and 5.(

of patients with

neurofibromatosis type 1 ha&ethe deformity.

INTRODUCTION



Congenital pseudarthrosis of tibia refers to

nonunion of tibial fracture that de&elops

spontaneously or after tri&al trauma in a dysplastic

bone segment of tibia diaphysis. CPT is rare /

0sually de&elops in first " yrs of life. There is a

strong association of CPT with neurofibromatosis

type 1.

INTRODUCTION



2ssociated with anterolateral bowing of tibia.

3owing usually occurs at the

4unction of middle / distalthird.

eformity may be associated

with s6in dimple, limbshortening, dysplasia of fibula /an6le &algus.

0sually unilateral.

INTRODUCTION



N781 occurs due to mutation on the gene codingfor NE09)7$39):$N on chromosome 1.

Neurofibromin is epressed in a broad range of

cells / tissue type. $t negati&ely regulates 9as

acti&ity * cell proliferation / function+. $t-s

deficiency leads to increased 9as acti&ity. :2P<* mitogen acti&ated protein 6inase+ acti&ity which

is essential for osteoclast function .

INTRODUCTION



BOYD CLASSIICATIONType 1 =8

Pseudarthrosis occurs with anterior

bowing.

2 defect in tibia present at birth.

)ther congenital deformities may be

present which may affect the management

of pseudarthrosis.



Type " =8

Pseudarthrosis occur with anterior

bowing / a hourglass constriction of the

tibia is present at birth.

>pontaneous fractures or after minor

trauma.

2lso 6nown as ?$@? 9$>< T$3$2 .

Tibia is tapered, rounded, sclerotic /

obliteration of medullary canal.

:ost common type.

2ssociated with N781

Poorest prognosis.

BOYD CLASSIICATION



Type A =8

Pseudarthrosis de&elops in a congenital cyst

usually near the 4unction of middle / distal

third of tibia.

2nterior bowing may precede or follow the

de&elopment of fracture.

9ecurrance of fracture is less common after

treatment.

BOYD CLASSIICATION



Type # =8

)riginates in a sclerotic segment of

bone.

Bithout narrowing of tibia.

:edullary canal is partially or

completely obliterated.

2n insufficiency or stress fracture

de&elops in the corte of tibia /

gradually etends through the sclerotic

bone.

Prognosis is good.

BOYD CLASSIICATION



Type 5 =8

Pseudarthrosis of tibia occurs with a dysplastic fibula.

Pseudarthrosis of both bone may de&elop.

Prognosis is good if the lesion is confined to fibula.$f the lesion progress to tibia then the natural ho usually

resembles type ".

Type ' =8

)ccurs as an intraosseous neurofibroma or schwannoma

Etremely rare.

BOYD CLASSIICATION



CRA!ORD CLASSIICATION

i&ided broadly di&ided into " types=8

Non8ysplastic 2nterolateral bowing with increased density /

sclerosis of medullary canal.

ysplastic

2nterolateral bowing with failure of tubulari%ation.Cystic changes.7ran6 pseudarthrosis.



$ 2nterolateral bowing with increased density /

sclerosis of medullary

canal.$$ 2nterolateral bowing

with failure of

tubulari%ation.

$$$ Cystic changes.

$D 7ran6 pseudarthrosis.

CRA!ORD CLASSIICATION



CLASSIICATION BY "AL#Y



#"ID#$IOLO%Y

9egister in the time period 1!"FF'.

"" patients with CPT were registered during thesame time, of which "F were ethnic Norwegian,one was the child of immigrants from 2sia, andone was from the :iddle East. The incidence of

CPT based on the time period 1!"FF' was1='F,FFF for ethnic Norwegian. 1# patients *'A

(+ had definite signs of neurofibromatosis.



?erring-s classification relies on two simplecriteria= *1+ the presence or absence of fracture

and *"+ the age at which fracture first occurs

*early onset-- before # years of age, delayed

onset-- after # years+.

#"ID#$IOLO%Y



There were 18 patient early8onset cases

since they occurred before the age of # years

*mean age 1' months, age range F!A' months+.2 patient late8onset cases occurred at the age of

5 and 1A years. 2 patients showed characteristic

bowing of the tibia but ne&er de&eloped a

fracture.

#"ID#$IOLO%Y



3ased on the classification of Crawford and

3agamery, we found three type ! one type !

four type , and nine type " cases. #ive

cases could not be classified according to

the Crawford and 3agamery system since

radiographs before primary surgery were

not available

#"ID#$IOLO%Y



uring the period 1!"FF' *"F8year period+.

?au6eland 0ni&ersity ?ospital in 3ergen

reported two cases, whereas "F patients had

been treated at our institution *)slo 0ni&ersity

?ospital+.

"ATI#NTS AND $#THOD

nformation on all cases of CPT



15 patients primary surgical treatment waspseudarthrosis resection and a proimal metaphyseal

osteotomy by use of the $li%aro& method

' patients were treated with segmental transport

patients with acute compression and subseGuent

proimal tibial lengthening. $n these nine patients with

proimal lengthening of the tibia, an osteotomy was also

performed in the middle third of the fibula.

"ATI#NTS AND $#THOD



" patients a resection of the pseudarthrosis

was done combined with bone grafting and

periostal transplantation from the iliac crest.

"ATI#NTS AND $#THOD

$ean age at first surgery! including all

operated patients in this study! was %.&(range '1') years.



Patients who were treated with an $li%aro& frame recei&ed a

plaster8cast for the first '! wee6s after frame remo&al.

Eamined on standard anterior!posterior and lateral

radiographs, was defined as radiological healing of thepseudarthrosis

No refracture within at least " months following frame

remo&al.

"ATI#NTS AND $#THOD

Criteria ealing



P9$:29H T9E2T:ENT 9E>0IT> $n the $li%aro&group primary healing was achie&ed in 1F patients *''

(+, which means that they showed apparent healing

before frame remo&al and did not refracture within thefirst " months after frame remo&al. 5 patients

refractured within " months after frame remo&al.

?owe&er, all patients successfully with the $li%aro& frame

refractured within # years *mean refracture time

months, range '!# months+

R#SULT



The two patients who primarily were treated

with a telescopic nail and grafting of bone and

periosteum from the iliac crest achie&ed primary

healing

Primary treatment with pseudarthrosis resectionand plate fiation combined with bone grafting did

not lead to healing.

R#SULT



709T?E9 T9E2T:ENT and long8term results

Bithin 5 years after initial treatment all patients but

three reGuired further surgery due to refracture at

the pseudarthrosis site. Eight patients refractured

more than one time *mean no. of refractures at the

pseudarthrosis ", range 1!5+. Two of these patients

recei&ed a &asculari%ed fibula graft, one patient was

operated on with an intramedullary nail.

R#SULT



Two patients recei&ed an $li%aro& procedure

combined with 7assier!u&al telescopic nails

and one patient recei&ed an $li%aro& procedure

combined with a rush8pin. 2 total of "lengthening procedures were performed in 1

patients of our study population; mean

lengthening was #" mm *range !,5 cm+

R#SULT



>e&en patients fractured at the lengtheningsite, of whom one patient refractured two times

and one patient refractured three times. Nine

patients reGuired an a&erage of two *range 1!#+

additional surgical procedures for lengthening or

ais correction.

R#SULT



9E>$02I E7)9:$T$E>79)NT2I PI2NE 2I$@N:ENT

The alignment and 4oint orientation of the affected limb

in the frontal plane were compared with the patient-s own

healthy contralateral side. :al8alignment in the frontal plane

based on the mechanical ais de&iation *:2+ was .5 mm to

lateral *range " lateral!#F medial+, whereas 1A patients had a

&algus deformity with a mean :2 of 1A *range A!"+ mm to

the lateral. ' patients had a neutral mechanical ais and 1

patient a &arus deformity with a :2 of #F mm to the medial.

R#SULT



2nalysis of 4oint orientation of the 6nee 4oint

in the frontal plane showed that 1# patients had

a mI72 within the normal range, whereas fi&e

patients had a mild &algus deformity in the

femur with a mean of #." *range "!+ &algus and

one patient had of &arus

R#SULT



3ased on the :PT2 measurements, twopatients had a &arus deformity of A and #,

respecti&ely.

"F patients for J8rays were a&ailable, 1' had

a &algus deformity in the an6le 4oint on the

affected side, with a mean IT2 of 1'. *range

5!A#+

R#SULT



>2@$TT2I PI2NE 2I$@N:ENT$n the sagittal plane, normal &alues, as described

by Paley, were used for e&aluation. 2nalysis of P72

showed that si patients had a mild recur&atumdeformity in the distal femur on the affected side,

with mean of A. *"!'+ recur&atum, one patient

showed a procur&atum of A, whereas all otherpatients with a&ailable J8rays had normal sagittal

alignment in the distal femur.

R#SULT



IEN@T? :E2>09E:ENT>:ean leg length discrepancy *II+ in our

patient group was 1A *range F!'F+ mm, and the

affected etremity was shorter in all patients, but

one. )f the 1 measured patients, 11 showed a

femoral o&ergrowth of K1F mm *mean 1" mm, range

11!" mm+ on the affected side. :ean length

discrepancy in the tibia was 1 *range #!'"+ mm.

R#SULT



$n 1# of 1 measured cases the affected tibia

was shorter. $n 11 patients, foot height, from the

le&el of the floor to the top of the talus, was

reduced on the affected side compared to the

healthy side *mean mm, range #!AF mm+,

whereas se&en patients showed no difference in

foot height.

R#SULT



DISCUSSION

3ased on a "F8year period

)nly two patients fulfilling the criteria

for late8onset-- cases

The incidence of CPT of1*&+!+++

erring nor the Crawford and,agamery classification could

provide guidance tomanagement



DISCUSSION

-ith the liarov method! healing in primary

treatment was achieved in more than half of the

patients. owever! all patients but onerefractured within % years and re/uired multiple

surgeries in order to achieve permanent healing.



3ecause of mal8alignment and complications

related to lengthening

There was no correlation between the length of

the lengthening and the ris6 of refracture

DISCUSSION

0ditional surgeries

Correlation between the lengthof the lengthening and the ris

of refracture



2nalysis of residual deformities in our

patients showed that the ma4ority of the patients

had a &algus deformity in the affected etremity

with a lateral :2. The source of the frontal

plane &algus malalignment affecting the

mechanical ais was mainly locali%ed on the

tibial segment.

DISCUSSION



>upramalleolar distal tibia osteotomy to

correct an6le &algus is considered to be the least

attracti&e treatment option, since recurrent

pseudarthrosis may de&elop in up to 5F ( of the

patients

DISCUSSION



DISCUSSION

Permanent intramedullaryfiation to maintain alignment

and to provide internal bracing for a united tibia

might be considered.

-eaness of the study is that did notinclude the functional outcome of

the patients. 3ue to the compleityof the disease the studyconcentrated on the radiographicoutcome.



DISCUSSION

ncidence of CPT in 4orway

liarov method! healing can be achieved in more than half

of the patients



DISCUSSION

liarov frame combined witha permanent intramedullary

nail

0voidance of ecessiveresection of thepseudarthrosis

5engtheningprocedures



TITL#

6pidemiology and Treatment 7utcomeof Congenital Pseudarthrosis

of the Tibia

8 There are 11 words

8 The title had describe the content of the 4ournal

8 The title can described all the &ariable



ID#NTITY O JOURNAL 2n )riginal Clinical 2rticle

L. ?orn. ?. >teen. T. Ter4esenepartment of Children-s )rthopaedics and

9econstructi&e >urgery

Publish )nline = 1 Lanuary "F1



ABSTRACTConsist of # part

$nformati&eIess than "5F word *"#' word+



C9$T$C2I

2PP92$>2I



Population patients 22 patients with CPT wereregistered in university hospital in

4orway

ntervention Patient with liarov method

Comparator control Patient with segmental transport !acute compression andsubse/uent proimal tibiallengthening

outcome 9ate of healing in primarytreatmen



R#SULT

The main result described in sentenceand use a table

The comparison between the &ariable

was described in sentence and paragraph



DISCUSSION& CONCLUSION& AND

R##R#NC#S The discussion and conclusion didn-t di&ided

in another point

iscussion and conclusion had describe withclear and complete

escribe for do another trial with a differentarea and method



Criteria

:ethod of the study 9etrospecti&e

Time and place escribed

$nclusion and eclusion criteria escribed

The &alue of sample escribed

:ethod of sampling Consecuti&e

>tatistical analy%e The study used statistical analy%e

3lind no



Than6 Hou Dery :uch, >ir.

congenital pseudoarthrosis of the tibia

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