Current paediatric orthopaedic practice in hereditary

SICOT-J 2018, 4, 10© The Authors, published by EDP Sciences, 2018https://doi.org/10.1051/sicotj/2018002

Available online at:www.sicot-j.org

REVIEW ARTICLE

Current paediatric orthopaedic practice in hereditary multipleosteochondromas of the forearm: a systematic reviewTamer A. EL-Sobky1,*, Shady Samir1, Ahmed Naeem Atiyya2, Shady Mahmoud1, Ahmad S. Aly1,and Ramy Soliman2

1 Division of Paediatric Orthopaedics, Department of Orthopaedic Surgery, Faculty of Medicine, Ain-Shams University,Abbasia, Cairo, Egypt

2 Division of Hand Surgery, Department of Orthopaedic Surgery, Faculty of Medicine, Ain-Shams University, Abbasia,Cairo, Egypt

Received 25 October 2017, Accepted 29 December 20

*Correspon

This is anO

17, Published online 21 March 2018

Abstract -- Introduction: This systematic review aims to answer three research questions concerning themanagement of hereditarymultiple osteochondromas of forearm in children:What is the best available evidencefor the currently employed surgical procedures? What patient characteristics are associated with betterprognosis? What disease characteristics are associated with better prognosis?Methods: We searched the literature using three major databases with no publication date restrictions. Toenhance search sensitivity and maintain precision we used keywords/subject terms correlating with patientpopulation, problem and interventions.We used strict inclusion/exclusion criteria to improve validity evidence.Results: The search process yielded 34 eligible studies with a total of 282 patients (315 forearms). Wecomprehensively analysed study and patient demographics and interventions and outcomes. Eleven studies(32%) had a long-term follow-up and 31 studies (91%)were retrospective. Of the total number of forearms, ulnarlengthening +/� associated procedures was used in 210 forearms (66.7%), isolated osteochondroma excision in65 forearms (20.6%) and isolated distal radius hemiepiphysiodesis in 15 forearms (4.7%) among others.Discussion: Ulnar lengthening can restore radiologic anatomy, improve appearance and to a lesser extentobjective clinical parameters like joint range of motion on the short/intermediate term. Isolatedosteochondroma excision can relief pain and satisfy cosmetic concerns occasionally. There is poor evidenceto suggest that surgery improves quality of life or function. Predictors of surgical success in regard to patient anddisease characteristics remain elusive. Natural history and prospective randomized control studies where thecontrol group receives no treatment should be rethought. They have the potential for bias control andidentification of the ideal surgical candidate. The complex interplay between the confounding variables hasundermined the capability of most studies to provide well-grounded evidence to support and generalize theirconclusions. Valid quality of life scales should supplement objective outcome measures.

Key words: Children, Hereditary multiple exostoses, Multiple cartilaginous exostoses, Diaphyseal aclasis,Benign forearm tumours, Skeletal dysplasia, Ulna lengthening.

Introduction

Hereditary multiple osteochondromas (HMO) areuncommon benign bone tumors. They are usuallydiagnosed in early childhood. HMO are inherited in anautosomal dominant manner. The two genes in whichpathogenic variants are known to cause HMO are EXT1and EXT2 [1,2]. The pathogenesis is linked a criticalreduction in heparan sulfate chain elongation [2]. Con-trastingly, solitary osteochondromas are common benign

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penAccess article distributed under the terms of the CreativeComwhich permits unrestricted use, distribution, and reproduction i

bone tumours. The growth pattern of a solitary osteo-chondromas is comparable to that of HMO. The diagnosisof HMO is based upon a distinct clinical and radiographicappearance. A fundamental clinical feature is multiplefirm swellings erupting from the ends of long bones or fromthe surface of flat bones usually symmetrical [1].Radiologically they present as multiple juxtaphysealcartilage capped bony growths with undisturbed courseof cortex and medullary bone from the normal bone intothe osteochondroma [1,2]. Masada and colleagues [3]classified HMO into three main groups based upon thepathologic anatomy. Patients with HMO can exhibit limb

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2 T.A. EL-Sobky et al.: SICOT-J 2018, 4, 10

length discrepancy, angular deformities around the knee,ankle and forearm, short stature, painful joint range ofmotion, joint subluxation and neurovascular compression[1,2]. Up to 70% of patients with HMOmanifest in forearmdeformities [4]. Most forearm and hand deformities areclinically pronounced [4,5]. Unbalanced physeal growthbetween the radius and ulna can result in forearm bowing,relative shortening of radius or ulna, carpal instability,and radial head dislocation with subsequent limitation offorearm rotation [1,4]. Forearm HMO has been managedby one or more of the following procedures: isolatedexcision of osteochondroma [6–12], acute [7,12,13], and/orgradual [6,14–26] ulnar lengthening, combined ulnar andradial lengthening [27], distal radial hemiepiphysiodesis[3,12,28,29], corrective radial osteotomy [7,13,30], crea-tion of one-bone forearm [31–33], radial head relocation[8,10,22], reconstruction of the distal ulnar epiphysis byvascularized proximal fibula epiphysis [34] and Sauve-Kapandji procedure [10]. Nevertheless, the optimalmanagement of HMO of the forearm is greatly disputable.The best evidence for each of the practiced surgicalprocedures, the optimal timing for intervention andpredictors for surgical success are alike unsettled disputes.Some authors cast fresh doubts about the value of surgeryin regard to improving function [7,35]. This topic has notbeen critically appraised before in the literature. Thissystematic review aims to resolve the above-mentioneddisputes. In consequence we formulated the followingresearch questions relating to the management of HMO offorearm in children: 1)What is the best available evidencefor surgical procedures used to manage HMO? 2) Whatpatient characteristics are associated with better progno-sis? 3) What disease characteristics are associated withbetter prognosis?

MethodsSearch approach

This article does not contain any studies with humanparticipants or animals performed by any of the authors.All authors shared in the study selection and dataextraction process relating to the surgical managementof HMO of the forearm in children.We conducted a searchfor English language publications before July 2017employing the following electronic databases: PubMed,Google scholar andEmbase.We checked the reference listsof the captured articles and review articles for additionaleligible publications. We also screened articles that citedthe captured articles. We discarded non-peer reviewedliterature that was not published in scientific journals andsecondary research such as review articles, letter to theeditor and commentaries. We conducted the initial searchon May 2017. We performed an additional search prior tomanuscript submission to make certain the extractedliterature is updated. To expand the recapture of relevantstudies our search strategy comprised both keywords andindexwords in accordancewithMedical SubjectHeadings.

We used Boolean operators properly to optimize searchresults quantitatively and qualitatively. The three mainBoolean operators are AND, OR, and NOT. Booleanoperators are used to narrow, broaden or restrict thesearch results. We aimed at avoiding biased inclusionterms. Hence, the selection of search terminology wassubdivided according to (a) patient population, (b)problem and (c) intervention terms.We retrieved relevantstudies using the following patient population andproblem terms: children, paediatric, hereditary multipleosteochondromas, hereditary multiple exostoses, multiplecartilaginous exostoses, diaphyseal aclasis, forearmtumors, ulnar shortening. Additionally, we used thefollowing intervention terms: osteochondroma excision,radius osteotomy, ulnar lengthening, distraction osteo-genesis. We did not impose limiting terms with regard tostudy design types. The collected studies were excluded asfollows; (a) descriptive studies reporting the clinical and/or radiologic features, (b) studies reporting the naturalhistory, (c) studies reporting on adults, (d) studiesreporting exclusively on solitary osteochondromas, (e)studies reporting solely on pathologies other than HMO,(f) studies with follow-up< one year, (g)HMOmanaged inthe context of malignant transformation and (f) studiesreporting solely on HMO of the hand. We includedprospective and retrospective studies. We also includedcase series and case reports. If studies were heterogenousfor age population and pathologic disorder, only skeletallyimmature patients with HMO were selected. Disputes inregard to study selection were settled with face-to-facemeetings. A schematic representation of the literatureextraction process together with exclusions is provided(Figure 1).

Quality appraisal instruments

The reported items of this review were in concordancewith the Preferred Reporting Items for SystematicReviews and Meta-Analyses statement [36]. We usedthe systematic review critical appraisal worksheet fromthe University of Oxford Centre for Evidence-basedMedicine www.cebm.net/critical-appraisal to checkquality through all phases of this systematic review[37]. We identified factors that may reflect a significantresearch bias before, during and after the conduction ofincluded studies with respect to patient selection,outcome measure assessment, statistics and confoundingvariables. We employed a valid instrument designed toevaluate the methodological quality of non-randomizedsurgical studies, whether comparative or non-compara-tive (MINORS) [38]. We selected MINORS [38] evalua-tion instrument as case series observational studies werethe key of the primary studies. We conducted acomprehensive comparison between the aggregatedtreatment outcomes and tried to identify patient anddisease features linked to better prognosis. To avoid biasin favour of reporting positive findings only we decided tofinalize our systematic review protocol in advance of anydata extraction.

Figure 1. A schematic representation of literature extraction process.

T.A. EL-Sobky et al.: SICOT-J 2018, 4, 10 3

ResultsStudy demographics

The literature extraction process involved 4 phases: (a)identification, (b) screening, (c) eligibility, and (d)inclusion. There were only three prospective case seriesstudies [20,27,39] (OCEBM type II) versus 31 retrospec-tive studies (OCEBM type III). These prospective studieshad small sample sizes [39] or were simply case reports[20,27]. The characteristics of the 34 final studies includedin this systematic review are presented in Table 1. Elevenstudies (32%) had a follow-up> 5 years, five of which wererelatively sizable with study participants ≥ 10. Of the 34included studies, 32 were published in recognized ortho-paedic society journals. The publication years ranged from2016 to 1984. Eight were multicentre studies [8,11,13,15–17,19,23]. According to MINORS the methodologicquality all but three prospective studies scored 5 out of 8.

Patient characteristics

The summed number of patients enrolled in the includedstudies was 304. One study with 22 patients was excludedfrom analysis due to poor demographic reporting but itsimplicationswere discussed [29].Therefore, thefinal number

of patients enrolled was 282 patients with 315 forearms. Themean age of patients for individual studies ranged from (5–13.5) years. One study provided a separate mean age foreach of the three surgical procedures conducted [10] andanother provided a separatemean age for boys and girls [28].Gender distribution was provided for 222 patients (79%) ofthe 282 patients finally enrolled in the review. There were118 (53%) males and 104 (47%) females. Six studies did notprovide the gender distribution [7,10,11,16,28,29]. Onestudy did not provide a mean age or follow-up and provideda range instead [29]. For publications that did not directlyreport the Masada types the information was extrapolatedfrom descriptive clinical picture and/or radiographs.Interestingly, in one study [26] neither Masada types norosteochondroma excisionwere reported.Of the 257 forearmsthat “reported” Masada types, 166 (64.6%) were type I, 25(9.7%) were type IIA, 48 (18.7%) were type IIB and 18 (7%)were type III. Two studies [22,25] referencedMasada but didnot provide details of patients’ radiographic grading.

Interventions and outcomes

A detailed reporting of the interventions, outcomes,and complications at final follow-up of the included studiesin this review is presented (Table 2). The indications of

Tab

le1.

Stud

ydemog

raph

ics.

Autho

r:Year

[Reference]

Patients

(N)

Forearm

s(N

)Male:Fem

ale

(N)

Mean

age(Y

)Mean

follo

w-up

(Y.M

)

Fam

ilyhistory(N

)Su

rgical

indication

sMasad

asubtyp

e

Cho

:201

4[19]

33

0:3

7.2

2.1

NR

Restrictedrotation

&cosm

etic

IIB

(3)

D’A

mbrosi:2

016[15]

1515

8:7

10.1

6.4

NR

Restrictedrotation

orda

ilyactivities,ulna

rshortening

≥1.5cm

IIA

(6),IIB

(8),III

(1)

Dem

ir:201

1[39]

66

2:4

124.2

NR

Restrictedrotation

,pa

in,neurov

ascularcompression

&cosm

etic

IIA

(3),IIB

(3)

Vog

t:20

11[21]

1212

3:9

9.8

2NR

Ulnar

shortening

≥1cm

,prog

ressivedeform

ity,

function

alim

pairment

I(7),IIA

(1),IIB

(4)

Tan

g:20

13[41]

1414

8:6

9.2

3.6

NR

Restrictedda

ilyactivities,Ulnar

shortening

≥1.5cm

,&

cosm

etic

I(14)

Masad

a:19

89[3]

1113

5:6

10.3

2.6

7Restrictedda

ilyactivities

&ROM

I(9),IIA

(1),IIB

(2),

III(1)

Beutel:2

014[20]

11

1:0

112

Non

ePain&

restricted

ROM

inelbo

wI(1)

Hill:201

1[22]

#4

52:2

8.8

2.2

NR

Wrist,forearm

orelbo

wdeform

itywithulna

ror

radial

shortening

>2cm

,pa

rticularly

youn

gchild

ren,

restricted

daily

activities

NR

Litzelm

ann:20

12[7]

1415

NR*

11.1

9.8

NR

Cosmetic

orfunction

al(painor

limited

mob

ility)

basedup

onsurgeonpreference

I(7),IIA

(1),IIB

(4),

III(3)

Jiya

:199

7[11]

1012

NR

13.3

6.3

NR

Restrictedda

ilyactivities,grip

streng

thI(9),III(3)

Shin:200

6[10]

2222

NR

9.2,

8.8,

11.1

†3.6

NR

Pain,

function

alloss

ofmov

ementof

theforearm

&cosm

esis.

I(11),Ian

d/or

IIB

(11)

Rasoo

l:200

8[31]

22

0:2

51.3

NR

Elbow

pain

&deform

ity

IIA

(1),IIB

(1)

Pritchett:198

6[26]

810

3:5

11.4

3.1

NR

Ulnar

shortening

≥1.5,

carpal

slip

≥50

%,

symptom

atic

radial

head

instab

ility,restricted

daily

activities

&cosm

etic.

NR

Massobrio:201

5[27]

11

0:1

920

NR

Fun

ctiona

lrestrictionof

ROM

inelbo

w&

wrist

I(1)

Kelly:201

6[28]

1315

NR

Boy

s:10

.3&

Girls:11

.55

NR

NR

NR

Ton

ogai:201

5[18]

23

1:1

9.5

3.7

NR

RestrictedROM

I(2),III(1)

Refslan

d:20

16[14]

1717

11:6

74.6

NR

RestrictedROM

I(10),IIB

(7)

Bau

er:201

7[17]

33

NR*

15NR

NR

RestrictedROM

NR

Yan

g:20

13[34]

22

1:1

6.5

Boy

:1&

Girl:8

1Deformed

wrist

I(1),IIB

(1)

Bilen:20

09[23]

78

3:4

103.3

NR

NR

I(5),IIB

(3)

Mad

er:200

3[40]

24

1:1

8.8

2NR

Fun

ctiona

lrestricted

ofROM,ulna

r/radial

shortening

≥2cm

,carpal

slip

≥50

%&

RAA

≥40°

I(4)

Peterson:20

08[32]

11

1:0

9.2

15NR

Elbow

deform

ity&

unstab

le,forearm

leng

thdiscrepa

ncy,

restricted

motion

IIB

(1)

Eralp:201

6[16]

34

NR

105.8

NR

Deformity&

restricted

daily

lifeactivities

I(4)

Waters:19

97[13]

17NR

11:11*

10.7

*3

NR

Progressive

forearm/w

rist

deform

ity,

limited/p

ainful

ROM,radial

head

sublux

ation&

basedon

definite

radiog

raph

iccriteria

I(12),IIA

(4),IIB

(1)


Tab

le1.

(con

tinu

ed).

Autho

r:Year

[Reference]

Patients

(N)

Forearm

s(N

)Male:Fem

ale

(N)

Mean

age(Y

)Mean

follo

w-up

(Y.M

)

Fam

ilyhistory(N

)Su

rgical

indication

sMasad

asubtyp

e

Ip:200

3[30]

66

5:1

7.6

2.5

5Daily

lifeactivities

&cosm

etic

I(6)

Song

:201

3[6]

1013

6:4

9.6

4.8

NR

Fun

ctiona

llim

itationof

motion,

pain

&definite

radiog

raph

iccriteria

I(12),IIB

(1)

Akita:200

7[8]

23<

3117

:6*

1112

.818

Osteochon

drom

aexcision

:pa

infulROM

orcosm

etic.

Lengthening

/osteotomy:

basedon

definite

radiog

raph

iccriteria

I(21),IIA

(2),IIB

(3),

III(5)

Matsuba

ra:200

6[24]

77

3:4

10.8

7.1

NR

Rad

ialhead

dislocation,

daily

lifeactivities,pa

inful

forearm

rotation

,deform

ity&

cosm

etic.

I(6),IIA

(1)

Ishika

wa:20

07[9]

1314

6:7

7.9

4.5

NR

NR

I(14)

Cheng

:199

1[25]

44

2:2

121.5

NR

Ulnar

shortening

≥2cm

,radial

head

instab

ility

&function

allim

itationof

ROM

NR

Rod

gers:199

3[33]

22

2:0

13.5

8.5

1Painful

radial

head

dislocation+/�

severe

elbo

w&

forearm

deform

ity

IIA

(1),IIB

(1)

Iran

i:199

3[43]

1012

4:6

10.8

6.5

NR

Pain&

cosm

etic,prog

ressivedeform

ity(≥

1cm

ulna

rshortening

).I(4),IIA

(4),III(4)

Fog

el:198

4[12]

1721

12:5

94.5

NR

Pain&

cosm

etic,prog

ressiveradiolog

icdeform

ity(≥

1.5cm

ulna

rshortening

,radial

articularan

gle≥

30°&

carpal

slip

≥30°),rotation

restriction&

symptom

atic

radial

head

sublux

ation.

NR

Arm

s:19

97[29]

∞22

NR

NR

Ran

ge7–

77(m

eanNR)

>2

NR

Symptom

atic

osteocho

ndromaprom

inent,pa

inful,

aesthetically

unacceptab

le.

NR

Nnu

mber,NR

notrepo

rted.

*gend

erdistribu

tion

&otherdemog

raph

icswereprov

ided

either

fortheov

eralln

umberof

child

rendiag

nosedwithHMO

andno

tforthoseop

erated,o

rforov

eralln

umberof

differentdiag

nostic

grou

psan

dno

tforHMO,o

rforbo

thskeletally

mature&

immature;

#pa

tientcharacteristics,follo

w-up,

metho

dology

,outcomean

dcomplications

(exceptradial

head

status)wereprov

ided

for10

patients

withva

riou

sdiag

nosticgrou

psan

dthe

details

ofHMO

patients

wereno

tprov

ided

sepa

rately;

†asepa

rate

meanwas

givenforeach

ofhe

3surgical

procedures

cond

ucted;

<4/

23pa

tients

wereskeletally

mature;

∞thestud

ycomprised

acoho

rtof

patients

treatedconserva

tively

andsurgically

37of

which

weresubjectedto

ateleph

onequ

estion

nairebu

tpa

tientdemog

raph

ics,

disease

characteristicsan

dintervention

swereno

tprov

ided

sepa

rately

forop

erated

grou

p.


Tab

le2.

Intervention

san

dou

tcom

es.

Autho

r:Year[R

eference]O

perative

procedure

(forearm

s)Preop

erativeradiog

raph

yPostoperative

radiog

raph

ymPreop

erativeclinical

tools

Postoperative

clinical

outcom

emCom

plications

*

Cho

:201

4[19]

Osteochon

drom

aexcision

(ulna),grad

ualmid-

diap

hyseal

ulna

rleng

thening+

5mm

overleng

thening,

multiplan

arIlizarov

external

fixa

tor(3).

Rad

ialarticularan

gle,

radial

leng

th,radial

bowing,

percentage

ofradial

bowing,

carpal

slip,

ulna

rleng

th,ulna

rshortening

,percentage

ofulna

rshortening

&radial

head

stab

ility

Statistically

non-

sign

ificant

improv

ementof

all

indices

Patient-reported

function

alassessmentscale,

&ROM

Allpa

tients

were

satisfied,statistically

non-sign

ificant

improv

ementof

ROM

Callusfracture,&

asym

ptom

atic

resublux

ationof

radial

head

(1each).

D’A

mbrosi:2

016[15]

Osteochon

drom

aexcision

(ulnas),grad

ual

middiap

hyseal

ulna

rleng

thening,

uniplana

rexternal

fixa

tor(15),+/−

distal

radiou

lnar

syno

stosis

sepa

ration

.

Rad

ialarticularan

gle,

carpal

slip,ulna

rshortening

,radial

head

dislocation,

&relative

ulna

rshortening

Statistically

sign

ificant

improv

ementof

all

indices.

Carpa

lslip

remainedin

3/7

cases.

Patient-reported

function

alassessmentscale,

ROM,MAYO

Elbow

Score,

VAS,

SF-12

Allpa

tient-repo

rted

andph

ysician-repo

rted

parametersshow

eda

statistically

sign

ificant

improv

ement.

Non

-union

successfully

treatedby

bone

grafting

&compression

plate

fixa

tion

(1).

Dem

ir:201

1[39]

Osteochon

drom

aexcision

(2ulna

s,1radius),

grad

ual

mid-diaph

yseal/prox

imal

ulna

rleng

thening,

uniplana

rexternal

fixa

tor,

+/−

radial

osteotom

y(2).

Rad

ialarticularan

gle,

carpal

slip,ulna

rva

rian

ce,

radial

bowing.

MRI,CT

scan

ning

andbo

nescintigrap

hywerealso

used

Sign

ificant

improv

ementof

all

indicesbu

tstatistical

sign

ificancewas

not

used.

Patient-reported

assessmentscales

for

daily

lifeactivities,

pain,cosm

etic,

DASH

&ROM

Allpa

tient-repo

rted

parameters&

ROM

improv

edan

dall

patients

weresatisfied

withtheircosm

etic

outloo

k.Onlyon

epa

tientrepo

rted

mild

pain.

Callusfractures

successfully

treatedby

plating&

intram

edullary

fixa

tion

(2).

Vog

t:20

11[21]

Gradu

aldiap

hyseal

ulna

rleng

thening,

uniplana

rorthofi

xexternal

fixa

tor

(12),+/−

osteocho

ndroma

excision

(5ulna

s),+/−

prox

imal

radiou

lnar

syno

stosis

resection(1)

+/−

radial

osteotom

y(4).

Rad

ialarticularan

gle,

ulna

rshortening

,carpal

slip,radial

head

dislocation

Rad

ialarticular

angle&

ulna

rshortening

show

eda

statistically

sign

ificant

improv

ement.

Carpa

lslip

disapp

earedin

3/5casesan

dasym

ptom

atic

radial

head

dislocation

persistedin

all5

cases.

ROM

only

Forearm

:41

%im

prov

ed,25

%deteriorated,4%

unchan

ged.

Elbow

&wrist

flexion/

extension:

84%

unchan

ged,

1case

improv

edan

dan

other

deteriorated.W

rist

addu

ction/

abdu

ction:

59%

improv

ed&

41%

unchan

ged.

Prematurecallu

sCon

solid

ation

successfully

treated

withrepeat

corticotom

y(1).

Tan

g:20

13[41]

Osteochon

drom

aexcision

(ulna),grad

ualdistal

ulna

rosteotom

y&

leng

thening

+/−

5mm

overleng

thening,

uniplana

rexternal

fixa

tion

(14).

Rad

ialarticularan

gle,

radial

bowing,

percentage

ofulna

rshortening

,&

carpal

slip

† .

Rad

ialarticular

angle&

carpal

slip

improv

edin

allbu

t2

cases.

Ulnar

shortening

was

correctedin

all

cases.

Objective

&subjective

assessmentof

wrist

function

for:

percentage

ofgrip

power,ROM,pa

in&

activity

ofha

nd.

Allbu

t2casesha

dno

limitationof

daily

activities,4cases

repo

rted

mild

pain.All

ROM

parametersin

forearm

andbiplan

ewrist

motionim

prov

edexcept

for(1)case.

Overallscores:10

excellent

&4go

od.

Malun

ionwas

reop

erated

successfully

(1),refracture

after

fixa

torremov

alman

aged

conserva

tively

(1).


Tab

le2.

(con

tinu

ed).

Autho

r:Year[R

eference]O

perative

procedure

(forearm

s)Preop

erativeradiog

raph

yPostoperative

radiog

raph

ymPreop

erativeclinical

tools

Postoperative

clinical

outcom

emCom

plications

*

Masad

a:19

89[3]

Osteochon

drom

aexcision

(12ulna

s&

2radiuses),

grad

ualulna

rleng

thening

withexternal

fixa

tor(3)&

acutewithbo

negraft(10),

radial

osteotom

y(10),

distal

radius

hemiepiph

ysiodesis(2),

open

redu

ctionof

dislocated

radial

head

(2).

Rad

ialarticularan

gle,

carpal

slip,ulna

rshortening

.Relativeradial

shortening

was

measured

fortype

III

Ulnar

leng

thwas

restored

inallbu

ton

eforearm.Rad

ial

articularan

gle&

carpal

slip

improv

edin

allcases.

ROM

Forearm

rotation

improv

eddram

atically

inallcases.

Recurrenceof

ulna

rshortening

(2),prox

imal

radio-ulna

rsyno

stosis,

tran

sientradial

nerve

palsy(1

each).

Beutel:2

014[20]

Gradu

alulna

rleng

thening

withmultiplan

arexternal

fixa

tor(1).

Ulnar

shortening

,ulna

rbo

wing,

posterolateral

radial

head

near

dislocationwereseen.MRI

revealed

entrap

mentof

the

annu

larlig

amentwithin

theradiocap

itellarjoint,

osteocho

ndralim

paction

injuries

ofthean

terior

radial

head

,capitellu

m&

injuries

ofthean

terior

trochlea

andcorono

idprocess.

Ulnar

leng

th&

bow

wererestored

&radial

head

relocated.

MRIwas

notcond

ucted

postop

eratively.

ROM

&pa

inCom

pleterestorationof

elbo

wROM

&resolution

ofpa

in.

Forearm

was

unaffected

preoperatively.

Non

e

Hill:201

1[22]

#Osteochon

drom

aexcision

,grad

ualulna

rleng

thening

(proximal

diap

hyseal),

uniplana

r,multiplan

arIlizarov

orspatial/Ilizarov

hybrid

external

fixa

tor(5),

&op

enredu

ctionof

radial

head

withneck

osteotom

y(1).

Rad

ialarticularan

gle,

ulna

rshortening

,radial

head

dislocation

2dislocated

radial

head

sremainedso,1

dislocated

&1

sublux

edafter

initially

being

locatedan

d1

remainedlocated

before

&after

surgery.

Degreeof

deform

ity

was

recorded

pre&

postop

erativelybu

tNova

lues

orfina

lou

tcom

eswere

prov

ided

NR

Poo

rcallu

sregenerate

successfully

treated.

#

Litzelm

ann:20

12[7]

Inmild

deform

ity:

isolated

osteocho

ndromaexcision

(3radiuses).Isolated

radial

osteotom

ies(2).

Inmod

eratedeform

ity

typically

>11

yold:

corrective

distal

1/3radial

osteotom

ywithacute

ulna

rleng

thening+

bone

grafting

(3).In

severe

deform

ity:

grad

ualulna

rleng

theningov

er

Rad

ialarticularan

gle,

carpal

slip,radial

epiphy

seal

angle,

ulna

rva

rian

ce,radial

bowing&

radial

head

dislocation

assessed

byStoren

line.

Non

-significant

improv

ementof

all

radiolog

icpa

rameter

was

noted.

Twoof

the5dislocated

radial

head

spreoperatively

remainedso

postop

eratively.

ROM,pa

in,Patient-

repo

rted

function

alassessment:

QuickDASH

.

ROM

didno

tshow

statistically

sign

ificant

improv

ement.

One

out

ofthe3pa

tients

with

radial

head

instab

ility

&pa

inpreoperatively

remainedso

postop

erativelyan

drequ

ired

asuccessful

radial

head

resectionat

age17

years.

Revisionsurgeriesat

age17

yfordeform

ity

recurrence

(2)(1

radial

head

resection&

1radial

osteotom

y),

fracture

callu

sat

2yearspo

stop

erative(1).


Tab

le2.

(con

tinu

ed).

Autho

r:Year[R

eference]O

perative

procedure

(forearm

s)Preop

erativeradiog

raph

yPostoperative

radiog

raph

ymPreop

erativeclinical

tools

Postoperative

clinical

outcom

emCom

plications

*

Intram

edullary

pinat

diap

hyso-m

etap

hyseal

junction

,un

iplana

rexternal

fixa

tor(7)(4

with

radial

osteotom

y&

3with

osteocho

ndromaexcision

).

QuickDASH

.Sh

owed

asign

ificant

improv

ement.

Jiya

:199

7[11]

Isolated

osteocho

ndroma

excision

(4),

acuteulna

rleng

theningwithscrew

fixa

tion

,osteocho

ndroma

excision

(8),

+/−

radial

osteotom

y.

Linearax

is,radial

articular

angle,

carpal

slip,ulna

rshortening

Rad

ialarticular

angle,

carpal

slip

improv

edin

most

forearmsor

remained

unchan

ged.

Ulnar

shortening

was

frequent.

NR

(onlychief

complaintswere

recorded)

NR

Recurrenceof

ulna

rshortening

(5),

reop

erationfor

recurrentexostosis(1),

reop

erationfor

fracture/no

n-un

ionof

callu

sto

solid

union

(2).

Shin:200

6[10]

Isolated

osteocho

ndroma

excision

(11)

(6ulna

s&

5radiuses).Osteochon

drom

aexcision

&ulna

rleng

thening(4)(2

grad

ual

withun

iplana

rfixa

tor&

2acute),osteocho

ndroma

excision

withSa

uvé-

Kap

andjiprocedure(7).

Linearax

is,radial

articular

angle,

carpal

slip,ulna

rshortening

Isolated

osteocho

ndroma

excision

&ulna

rleng

thening:

statistically

non-

sign

ificant

improv

ement.

Sauv

é-Kap

andji:

Statistically

sign

ificant

improv

ement

ROM

inforearm

&elbo

wOsteochon

drom

aexcision

&ulna

rleng

thening:

statistically

non-

sign

ificant

improv

ement.

Sauv

é-Kap

andji:Statistically

sign

ificant

improv

ement

36.4

%recurrence

after

simpleosteocho

ndroma

excision

(allrequ

ired

reop

erations).Open

redu

ctionfor

persistently

symptom

atic

radial

head

dislocationafter

ulna

rleng

thening(2).

Reoperation

sfor

recurrent

osteocho

ndromaafter

Sauv

é-Kap

andji(2).

Rasoo

l:200

8[31]

One-bon

eforearm

(rad

ioulna

rfusion

)(2).

Rad

ialarticularan

gle,

carpal

slip

&ulna

rshortening

Residua

lulna

rshortening

inon

eforearm

ROM,grip

streng

thBothpa

tients

sign

ificantly

improv

edin

elbo

w,forearm

rotation

&grip

streng

th

Non

e

Pritchett:198

6[26]

Gradu

almid-diaph

yseal

ulna

rleng

thening,

uniplana

rexternal

fixa

tor

(6).Acute

ulna

rleng

thening(4)includ

ing

(2withiliac

crestgraft

andplatefixa

tion

&2ov

erRushrods).+/−

radial

osteotom

y(5).

Rad

ialarticularan

gle,

carpal

slip,relative

ulna

rshortening

,

Ofthe6sublux

ed/

dislocated

radial

head

spreoperative,5

becamestab

lepo

stop

erative.

ROM

ROM

improv

edin

most

forearms

Recurrenceof

ulna

rshortening

(6),

especially

child

ren&

youn

gad

olescents,

asym

ptom

atic

ulna

rno

n-un

ion&

deep

infection(1

each).

Massobrio:201

5[27]

Simultaneou

sgrad

ual

prox

imal

ulna

anddistal

radius

leng

theningwith

uniplana

rexternal

fixa

tor

(2).

Rad

ialarticularan

gle,

carpal

slip,ulna

rshortening

,relative

ulna

rshortening

&radial

leng

th

Sign

ificant

improv

ementin

all

measurements

Fun

ction,

ROM

&cosm

etic

Sign

ificant

improv

ementin

ROM

&function

Non

e


Tab

le2.

(con

tinu

ed).

Autho

r:Year[R

eference]O

perative

procedure

(forearm

s)Preop

erativeradiog

raph

yPostoperative

radiog

raph

ymPreop

erativeclinical

tools

Postoperative

clinical

outcom

emCom

plications

*

Kelly:201

6[28]

Distalradius

hemiepiph

ysiodesis(15).

Rad

ialarticularan

gle,

carpal

slip,ulna

rtilt,

luna

tesubsidence,

metap

hyseal

epiphy

seal

angle

Statistically

sign

ificant

improv

ementin

all5

measurements

except

luna

tesubsidence

NR

NR

Non

e

Ton

ogai:201

5[18]

Osteochon

drom

aexcision

&interosseous

mem

bran

edissection

,grad

ualulna

(2)

orradial

(1)leng

thening,

multiplan

arIlizarov

orun

iplana

rfixa

tor.

Rad

ialarticularan

gle,

ulna

rshortening

Improv

edROM

Improv

edNon

e

Refslan

d:20

16[14]

Gradu

almid-diaph

yseal

Ulnar

leng

theningwith

uniplana

rexternal

fixa

tor

(17),+/−

osteocho

ndroma

excision

(14),+/−

radial

osteotom

y(5).

Rad

ialarticularan

gle,

carpal

slip,radius

ofcurvature,

ulna

rva

rian

ce,

angleof

theradial

and

ulna

rph

yses,elbo

wcarrying

angle,

amou

ntof

radial

head

coverage

Statistically

sign

ificant

improv

ementin

radius

ofcurvature,

ulna

rva

rian

ce,

carrying

angle,

radial

head

coverage

ROM

&pa

inStatistically

non-

sign

ificant

improv

ement

inROM

&5pa

tients

who

hadpa

inpreoperative

hadno

pain

postop

erative.

Osteotomyfor

deform

ityrecurrence

(1),external

fixa

tor

failu

rerequ

iring

exchan

ge(2),

prem

atureconsolidation

(1).

Bau

er:201

7[17]

Gradu

alulna

leng

thening

(1).Com

binedulna

&radius

osteotom

ies(2)

Ang

ular

&rotation

aldeform

ityof

radius

&ulna

Statistically

sign

ificant

improv

ement

ROM

inrotation

Statistically

sign

ificant

improv

ement

Extensorpo

llicis

Lon

gusweakn

ess(1).

Yan

g:20

13[34]

Recon

structionof

the

distal

ulna

repiphy

sisby

vascularized

prox

imal

fibu

lawithepiphy

sis(2).

Rad

ialarticularan

gle,

carpal

slip

&relative

ulna

rshortening

Sign

ificant

improv

ement

(persistentbu

tasym

ptom

atic

radial

head

dislocation)

Fun

ction,

pain

ROM

&cosm

etic

Sign

ificant

improv

ement

Non

e

Bilen:20

09[23]

Osteochon

drom

aexcision

,grad

ualulna

rleng

thening

multiplan

ar(2)or

uniplana

r(6)external

fixa

tor+/−

radial

osteotom

y.Lengthening

was

Rushrods

guided

in(2)cases.

Rad

ialarticularan

gle,

carpal

slip

&ulna

rshortening

Sign

ificant

improv

ement&

all

radial

head

sthat

weredislocated

preoperative

were

redu

ced.

NR

Sign

ificant

improv

ementbu

tno

goniom

etric

measurements

cond

ucted

Non

e

Mad

er:200

3[40]

Osteochon

drom

aexcision

,grad

ualulna

rleng

thening,

uniplana

rfixa

tor(4).

Rad

ialarticularan

gle,

carpal

slip

&ulna

rshortening

Sign

ificant

improv

ement

ROM,function

.Sign

ificant

improv

ement

Non

e

Peterson:20

08[32]

One-bon

eforearm

(rad

ioulna

rfusion

)(1).

Previou

sresectionof

distal

ulna

&radial

head

dislocation

Restoration

offorearm

leng

thdiscrepa

ncy.

ROM

&stab

ility

inelbo

wSign

ificant

improv

ement

Non

e

Eralp:201

6[16]

Osteochon

drom

aexcision

,grad

ualmid-diaph

yseal

ulna

rleng

thening,

uniplana

rexternal

fixa

tor

Ulnar

shortening

,radial

bow

Restoration

ofulna

rshortening

&radial

bow.

Daily

lifeactivities

Sign

ificant

improv

ement

Recurrent

radial

deform

ity(1).


Tab

le2.

(con

tinu

ed).

Autho

r:Year[R

eference]O

perative

procedure

(forearm

s)Preop

erativeradiog

raph

yPostoperative

radiog

raph

ymPreop

erativeclinical

tools

Postoperative

clinical

outcom

emCom

plications

*

(4)(overSteinm

anin

2cases)

andcombined

radius

osteotom

y.W

aters:19

97[13]

Acute

ulna

rleng

thening

withplatefixa

tion

(17),

osteocho

ndromaexcision

(12)

&radial

osteotom

y(11).

ulna

rshortening

,ulna

rva

rian

ce,radial

inclination,

radial

articularan

gle,

carpal

slip

(AP

and

lateral),forearm-third

metacarpa

lan

gle,

status

ofossification

ofradial

&ulna

rph

yses,radial-head

sublux

ation&

cong

ruence

ofthedistal

radiou

lnar

joint.

Sign

ificant

improv

ement

ROM

Mostpa

tients

improv

edReoperation

swithbo

negraftforno

n/delayed

union&

brok

enplate

(3),prog

ressiveradial

head

sublux

ation(1),

annu

larlig

ament

reconstruction

(2),

chronicelbo

wpa

in(2),

creation

ofon

e-bo

neforearm

(1),repeat

leng

thening(2).

Ip:200

3[30]

Osteochon

drom

aexcision

,grad

ualulna

rleng

thening,

multiplan

aror

uniplana

rexternal

fixa

toror

acutely

withplating(6),

&radial

osteotom

y(5)ba

sedon

definite

radiog

raph

iccriteria.

Rad

ialarticularan

gle,

carpal

slip

&ulna

rshortening

,relative

ulna

shortening

Sign

ificant

improv

ement

ROM,subjective

simplequ

estion

naire

toassess

satisfaction

Sign

ificant

improv

ementin

ROM

&allpa

rentswere

satisfied

withfunction

&cosm

etic.

Rad

ialhead

dislocation

during

leng

thening(1)

was

successfully

redu

cedby

pin

stab

ilization

.

Song

:201

3[6]

Gradu

alulna

rleng

thening

withmultiplan

arexternal

fixa

tor&

mon

ofocal

ulna

rosteotom

y(13),+/−

radial

osteotom

y(5),

+/−

osteocho

ndroma

excision

(4).

Rad

ialarticularan

gle,

carpal

slip

&ulna

rshortening

,relative

ulna

shortening

Statistically

sign

ificant

improv

ements

&the

only

dislocated

radial

head

preoperative

was

redu

ced

postop

erative.

Subjective

qualityof

lifequ

estion

naire,

function

allim

itation

ofmotion&

pain

Mostpa

tients

were

satisfied

withforearm

appearan

ce,ha

dno

pain

onstrenu

ous

activities

&could

man

ageactivities

daily

lifeactivities

easily.

Recurrenceof

osteocho

ndroma&

deform

ity(2),delayed

union/

nonu

nion

(2).

Akita:200

7[8]

Isolated

osteocho

ndroma

excision

(13).

Osteochon

drom

aexcision

&ulna

rleng

thening(18)

(8grad

ualwithun

iplana

rexternal

fixa

tor&

10acute

withbo

negraft),+/−

grad

ualradial

leng

thening

(4),+/−

ulna

osteotom

y(2),+/−

radial

osteotom

y(14),+/−

open

redu

ction

radial

head

(2).

Rad

ialarticularan

gle,

carpal

slip

&ulna

varian

ceStatistically

non-

sign

ificant

improv

ementof

all

measurements

Subjective

qualityof

lifequ

estion

naire,

pain,ROM

&grip

streng

th

Statistically

non-

sign

ificant

improv

ement

inROM

&grip

streng

th.Mostpa

tients

werepa

infree

&ha

dno

restrictions

ofda

ilyactivities.

Unsatisfactorycosm

etic

appearan

ce(8).

Non

unionsuccessfully

treatedwithbo

negraft

andinternal

fixa

tion

(3),fracture

callu

s(2),

tempo

rary

radial

nerve

paresis(1)&

symptom

atic

radiocap

itellarjoint(2).


Tab

le2.

(con

tinu

ed).

Autho

r:Year[R

eference]O

perative

procedure

(forearm

s)Preop

erativeradiog

raph

yPostoperative

radiog

raph

ymPreop

erativeclinical

tools

Postoperative

clinical

outcom

emCom

plications

*

Matsuba

ra:200

6[24]

Osteochon

drom

aexcision

,grad

ualulna

rleng

thening,

uniplana

ror

multiplan

arexternal

fixa

tor(7),+/−

radial

osteotom

y(5),

+/−

grad

ualradius

leng

thening

(2).

Rad

ialarticularan

gle,

carpal

slip,ulna

varian

ce&

radial

bow.

Recurrenceof

ulna

rshortening

in(5)

cases.

The

other

measurements

improv

edmod

erately.

ROM

&pa

inSign

ificant

improv

ementin

pain

&forearm

rotation

except

onecase.

Recurrenceof

ulna

rshortening

(5)on

eof

which

was

releng

thened.

Rad

ialhead

sublux

ation

(1).

Ishika

wa:20

07[9]

Isolated

osteocho

ndroma

excision

(14)

(6from

distal

ulna

&8from

distal

ulna

+radius.

Rad

ialarticularan

gle,

carpal

slip,ulna

rshortening

,radial

leng

th,

radial

bowing.

Excisionfrom

distal

ulna

:statistically

sign

ificant

improv

ementin

ulna

rshortening

,radial

bow

only.

Excisionfrom

distal

ulna

+radius:no

n-sign

ificant

improv

ementan

d/or

deteriorationin

all

measurements.

NR

NR

Osteochon

drom

arecurrence

ofva

riou

sdegrees(7)(2

from

ulna

&5from

ulna

+radius).

Cheng

:199

1[25]

Gradu

alulna

rleng

thening

withun

iplana

rexternal

fixa

torwitho

utbo

negraft

(4).

Ulnar

leng

thon

lySa

tisfactory

corrected

ROM,cosm

etic

Non

-significant

improv

ementin

ROM,

butim

prov

edcosm

etic

&da

ilylifeactivities.

Non

e

Rod

gers:199

3[33]

One-bon

eforearm

(rad

ioulna

rfusion

)witha

pinor

plate(2),+/−

grad

ualleng

thening&

radial

osteotom

y(1).

Rad

ialarticularan

gle,

carpal

slip

&ulna

rshortening

Sign

ificant

improv

ementexcept

forresidu

alulna

rshortening

in(1)

case.

ROM,activities

ofda

ilylife

Satisfactory

inelbo

w&

wrist,returned

tofull-

timeman

ual

occupa

tion

/com

petitive

sports.

Non

e

Iran

i:199

3[43]

Isolated

osteocho

ndroma

excision

from

ulna

&radius

(8),

grad

ualulna

rleng

theningwithun

iplana

rfixa

tor(2)&

bone

graft

withplating+

radial

osteotom

y(1).

Ulnar

shortening

,&

radial

head

sublux

ation/

dislocation(3)

Relocated

&asym

ptom

atic

ROM

Noim

prov

ementin

forearm

rotation

Non

e

Fog

el:198

4[12]

Isolated

excision

ofthe

osteocho

ndromas

(ulnaor

radius)(12),acuteulna

rleng

thening&

excision

osteocho

ndroma(2),acute

ulna

rleng

thening,

excision

osteocho

ndroma,

&distal

radius

hemiepiph

ysiodesis

Rad

ialarticularan

gle,

carpal

slip

&ulna

rshortening

Isolated

excision

:no

improv

ementin

radial

articularan

gle

&carpal

slip.Ulnar

leng

thening&

excision

:no

improv

ement.

Ulna

leng

thening,

excision

&distal

radius

ROM,pa

in&

cosm

etic.

Isolated

excision

:no

improv

ementin

neither

rotation

norulna

rshortening

but

sign

ificant

improv

ement

inpa

in,no

osteocho

ndroma

recurrence.Ulnar

leng

thening&

excision

:

Non

e


Tab

le2.

(con

tinu

ed).

Autho

r:Year[R

eference]O

perative

procedure

(forearm

s)Preop

erativeradiog

raph

yPostoperative

radiog

raph

ymPreop

erativeclinical

tools

Postoperative

clinical

outcom

emCom

plications

*

(7).Fixations

werewith

plate/rush

rod,

+/−

bone

graft.

hemiepiph

ysiodesis:

sign

ificant

improv

ement.

noim

prov

ement.

Ulna

leng

thening,

excision

,&

distal

radius

hemiepiph

ysiodesis:

sign

ificant

improv

ement.

Arm

s:19

97[29]

∞Osteochon

drom

aexcision

s(36),radial-headexcision

s(6),distal

radius

hemiepiph

ysiodesis(5),

distal

radial

osteotom

ies

(2),an

dulna

rleng

thenings

withexternal

fixa

tors

(4).

Com

binedprocedures

performed

onasing

lepa

tientin

(11)

occasion

s.

Rad

ialarticularan

gle,

carpal

slip,relative

ulna

rshortening

,an

dforearm-

thirdmetacarpa

lan

gle.

Majorityof

patients

demon

strated

radiog

raph

icab

norm

alities

Telepho

nepa

tient-

repo

rted

question

naireof

qualityof

life

Majorityof

patients

werein

full-timejobs

withminim

alim

pact

onactivities

ofda

ilylife.

NR

Nnu

mber,NR

notrepo

rted,ROM

rang

eof

motionin

forearm

&elbo

w,+/−

wrist,VASvisual

analog

scale,

SF-12aqu

alityof

lifescalethat

measuresph

ysical

andmental

compo

nents,DASH

disabilitiesof

thearm,sho

ulderan

dha

ndscore.

mclinicorad

iologicresultsat

fina

lfollow-up;

*on

lysign

ificant

complications

weremention

ed;

†carpal

slip

couldno

tbe

measuredin

5casesbecausetheluna

tewas

poorly

ossified;

#meanag

ean

dfollo

w-up,

metho

dology

,outcomean

dcomplications

(exceptradial

head

status)wereprov

ided

for10

patients

withva

riou

sdiag

nosticgrou

psan

dthedetails

ofHMO

patients

wereno

tprov

ided

sepa

rately;

∞thestud

ycomprised

acoho

rtof

patients

treatedconserva

tively

andsurgically

37of

which

weresubjectedto

ateleph

onequ

estion

nairebu

tpa

tientdemog

raph

ics,

disease

characteristicsan

dintervention

swereno

tprov

ided

sepa

rately

forop

erated

grou

p.



surgical intervention expressed variability among studies.Restriction of daily activities and/or range of motion inforearm/elbow were the most common indications ofintervention. Most studies tended to favour objectivephysician-reported clinicoradiologic data as an outcomemeasure. Contrastingly, the majority of subjectivepatient-reported rating scales/questionnaires were non-validated and statistical significance was inadequatelyimplemented. One study [7] restricted the implementationof the patient-reported rating scale on the sub-category ofpatients subjected to gradual ulnar lengthening by anexternal fixator. This is seen as an effort to reduce theimpact of confounding variables. All studies employedplain radiography as a principal diagnostic tool. Twostudies [20,39] employed MRI as an adjuvant imagingmodality one of which [39] used CT scan and bonescintigraphy in addition. One study [27] employedultrasound to monitor callus progression. The vastmajority of studies assessed the radiographic outcome inaccordance with proposed measurements by Fogel andcolleagues which are a widely reported [12]. Five studies[6,9–11,41] used another referenced radiographic measure-ment by Burgess and Cates [42]. Among the variousradiographic measurements, radial articular angle, carpalslip, ulnar shortening, and radial head stability were themost commonly shared by included studies. Contrasting-ly, radial length was the least used measurement. Ulnarlengthening +/� an associated on demand procedure wasconducted on 210 forearms (66.7%) of the summednumber of forearms. Associated procedures included acombination of one or more of the following: angularcorrection, osteochondroma excision, corrective radialosteotomy distal radial hemiepiphysiodesis and openreduction of radial head. Of the 210 forearms (66.7%)subjected to ulnar lengthening, 145 forearms (69%) wereperformed gradually and 65 forearms (31%) were per-formed acutely mostly with bone grafting [3,7,8,10–13,26,30]. Of these later studies two used acute ulnarlengthening exclusively [12,13]. All “gradual” ulna length-enings were performed by a uniplanar fixator except in 17forearms (11.7%) a multiplanar fixator was used [6,19,20]and five studies with 22 forearms (15.2%) used bothuniplanar and multiplanar [18,22–24,30]. All ulnarlengthening osteotomies/cortectomies were performed ata mid or proximal diaphyseal level. One study employed asimultaneous radial and ulnar lengthening in one forearm[27] and three others (seven forearms) conducted isolatedradial lengthening as part of a series including ulnarlengthenings [8,18,24]. The vast majority of radialosteotomies were performed in association with ulnarlengthening procedures. Isolated corrective radial osteot-omies were done in two forearms (0.5%) only [7].

Isolated excision of osteochondroma or at least withoutbone lengtheningwas conducted on 65 forearms (20.6%) ofthe summed number of forearms [7–12]. Temporaryhemiepiphysiodesis of distal radial physis was performedon 29 forearms (9.2%) [3,12,28,29] one of which [28] 15forearms (4.8%) was exclusively devoted to hemiepiphy-siodesis. Sauvé-Kapandji procedure +/� osteochondroma

excision was performed on seven forearms (2.2%) [10].Creation one-bone forearm through radioulnar fusion waspracticed on five forearms (1.6%) [31–33] and reconstruc-tion of the distal ulnar epiphysis by vascularized proximalfibula including epiphysis was practiced on two forearms(0.5%) [34]. An open reduction of radial head +/� neckosteotomy was successfully performed on four forearms(1.3%) [3,10] and unsuccessfully on three forearms (1%)[8,22]. Resection of distal or proximal radioulnar synosto-sis was practiced on demand in two studies [15,21]. Radialhead excision was not practiced in pediatric patients atleast as a primary procedure. Proximal radio-ulnar fusionwas practiced on one forearm to manage symptomaticradial head subluxation [12]. The comparative prevalenceof the main interventions and techniques used in thisreview is demonstrated (Figures 2A, B, C). The overallcomplication rate was tolerable and showed no specificpredilection for any of the main interventions employed inthis review.

Missing data

The included studies had missing data related to thefollowing items: gender distribution (five studies)[10,11,16,28,29], number of forearms (one study) [29]and Masada subtype (seven studies) [12,17,22,25,26,28,29]. The overall skeletal burden of HMO wasreported in only four studies [11,16,33,43] and detailedin one [27]. Hand dominance was reported in only fourstudies [19,31,33,39] and 11 studies used pain as anoutcomemeasure. Previous surgeries were reported in fourstudies [13,16,27,32] and age of initial presentation in four[20,27,32,33]. Results of histopathologic examination werereported in six studies [16,20,24,27,34,41] and familyhistory in six [3,8,20,30,33,34]. No study reported genetictesting and physiotherapy protocols were either poorly ornot reported at all except for one study [22]. One study didnot report the follow-up period as it was primarilydesigned to assess the utility of a computer simulationmodel [17]. One study reported patient ethnicity [28]. Thestudy that practiced reconstruction of the distal ulnarepiphysis by vascularized proximal fibula did not reportdonor site morbidity [34]. One study reported conclusionsthat were discordant with the results [11]. The authorsunderscored the importance of ulnar lengthening inpreventing progressive deformity and minimising func-tional disability despite reporting frequent deformityrecurrence rates [11]. Grippingly, the authors employedneither objective nor subjective clinical outcomemeasures[11]. Generally speaking, we suggest that some of themissing data may have a potential impact on the validityof results and conclusions. For example failure to reportthe overall skeletal burden of HMO in terms of number andlocation and pain can influence the subjective patient-reported quality of life assessment [44–47]. Pain that wasgrossly underreported by the primary studies of thisreview has been found to be a major drive for surgery andnegatively influenced by surgery [47]. The incidence of apositive family history in patients with HMO has been

(A) (B) (C)

Figure 2. Comparative prevalence of the main interventions and techniques used in this review as percentages of forearms; (A) themain intervention groups used. Associated procedures include; osteochondroma excision, corrective radial osteotomy, distal radialhemiepiphysiodesis and open reduction of radial head. “Others” refers to isolated procedures as one-bone forearm, reconstruction of thedistal ulnar epiphysis by vascularized proximal fibula epiphysis, radial osteotomy and lengthening; (B) gradual versus acute ulnarlengthening; (C) fixator choice of included studies.


estimated to range from 62–96% [1]. Genotype-phenotypecorrelation studies demonstrated that certain types ofgene mutations and the overall skeletal burden of HMOare associated with a worse clinical presentation particu-larly with respect to deformity and function [1,2,44,45,48–50]. This may actually have a predetermined impact ontreatment outcomes.

DiscussionSummary of evidence

This systematic review included many chief surgicalinterventions each of which was multifaceted. For clarityand consensus, we will discuss each chief interventionseparately. We believe that this practical approach willassist in answering our first research question. The mostprevalent combination of surgical procedures encounteredin this review was ulnar lengthening, +/� an associatedprocedure. Generally speaking, this surgical technique wasadequately described and fairly constant across studieswith a tolerable complication rate on the short-term.Nevertheless, there is poor evidence to demonstrate thatthe seemingly satisfactory results of many short-termstudies are maintained on the intermediate-term [10] andmore precisely on the long-term [7,8]. These long-termrelatively sizable well-designed studies have questionedthe value of surgical intervention even in association withdeformity and radiographic abnormalities [7,8]. Thesestudies correlated the clinicoradiologic outcomes withpatients’ self- reported functional outcome measures inchildren operated for HMO of forearm and argued for thechoice of conservative treatment [7,8]. They foundinsufficient functional gains to justify surgery. In otherwords, preoperatively patients reported minimal func-

tional impairment despite major clinical and radiologicabnormalities [7,8]. The only exception was symptomaticradial head dislocation. This discrepancy between thefunctional capacity and amount of forearm deformity wascorroborated by three relatively sizable studies [22,24,29].These findings have been confirmed by a natural history ina large cohort of untreated adult subjects with HMO [35].

Generally speaking, most studies tended to focus on theradiographic outcome measures at the expense of objectiveclinical parameters. Likewise, the objective physician-reported clinical outcome measures were implemented atthe expense of the subjective patient-reported measureswhich were mostly non-validated and lacked in depth.Notwithstanding, these studies reported recognized cosmet-ic satisfaction of patients. Interestingly, in some studiespatients were selected for surgery exclusively based uponradiographic criteria while clinical (objective or subjective)outcome measures were neither reported before nor aftersurgery [9,11,22,28]. It is noteworthy that complicationssuch as recurrence of osteochondromas, and/or forearmdeformityneed longerdurations to resurface especially in theskeletally immature population [9,24]. This undoubtedlyoveremphasizes the significance of conducting long-termfollow-up studies and greatly undermines the quality ofevidence extracted from such short-term studies. Addition-ally, this critically calls attention to thevalueof thevalidatedsubjective patient-reported overall quality of life scales.Using these scales has shown that HMO patients had lowerscores compared to the general population [46,48]. Weunderstand that a comprehensive assessment of patients’outcomes entails both subjective patient-reported andobjective physician-reported instruments. In the light ofsuch observations, it is important to rethink the cost/benefitprofile of surgical intervention in paediatric HMO. In thatregard, retrospective natural history studies may refine the


wide and crude indications of surgery currently used inchildren withHMO.Two natural history studies are praisedfor aiming to identify radiographic predictors of radial headdislocation [4,51]. Likewise, well-designed randomizedcontrol studies where one group receives a definite surgicaltreatment and the other receives no treatment should berethought. To satisfy the ethical demands of such studies,strict inclusion/exclusion criteria will have to be imple-mented before enrolment and randomization. Besides,adequate patient orientation in regard to risks and benefitsof each treatment group and nature of the study will have toprecede enrolment and randomization. Such study designscan generally yield valid results and generalizable conclu-sions. Nevertheless, we acknowledge the logistic andpractical difficulties associated with such study designs.We believe that insufficient consensus about the indicationsof surgery and outcome scores in paediatric HMO is a majorlimitation of this systematic review. These discordantindications of surgery have also been noticed withinindividual study participants, further complicating evidenceextraction. Likewise, the heterogeneity of the surgicalprocedures used, diversity in patient characteristics andrelatively small study populations complicates evidenceextraction. These above-mentioned features are inherent toretrospective studies that constituted the majority ofincluded studies. Nonetheless, certain studies were remark-able for their methodologic quality. They implementedstudy designs that allow for control of selection andperformance bias [6,8,10]. They neatly and separatelyreported the results of HMO patients in accordance witheach distinct surgical procedure implemented. Additionally,some used assessor blinding methods and standardisedsurgeon related factors [6,8,10]. Isolated excision of osteo-chondroma can relief pain, satisfy cosmetic concerns andoccasionally improve range of motion. Nevertheless, there isinsufficient evidence for its use to initiate spontaneousdeformity correction or improve overall limb function. Inthis review the cases of radial hemiepiphysiodesis, one-boneforearm, vascularized fibular graft, isolated radial osteoto-mies or lengthenings practiced as “stand alone” procedureswere insufficiently prevalent to allow for meaningfulconclusions. These procedures may be better suited tospecificpatientprofiles andbasedupon surgeon’s preference.

Limitations and strengths

We acknowledge limitations of this review. Themajority of the included studies were of low methodologicquality. Studies with low methodologic quality mayimpact negatively on outcome validity and conclusions.Narrative/traditional reviews are usually biased andsubjective in contrast to systematic reviews which areoften unbiased and objective [52]. The reliability andaccuracy of systematic review recommendations shouldnot be determined by methodologic quality of the primarystudies included, but rather by the degree of methodologi-cal integrity implemented by researchers [52]. In thissystematic review we formulated focused research ques-tions that require specific answers. Additionally, we

implemented a comprehensive review methodology thatallowed for a reasonable control of bias. Therefore, weestimate that our comprehensive systematic reviewstrategy can counterbalance the shortcomings of includingstudies with low methodologic quality. Of the 18 studiesexcluded on basis of language 11were provisionally eligiblefor inclusion in this review. These 11 studies comprised 54forearms. It is noteworthy that in three of these 11 studiesthe size of study population could not be accounted for dueto missing or deficient abstracts. In general, the excludedstudies were comparable to the included studies in terms ofmethodology and individual sample size. Hence, theselanguage exclusions seem inconsequential.

Conclusions

Ulnar lengthening +/� associated procedures canrestore radiologic anatomy, improve appearance and to alesser extent objective clinical parameters on the short/intermediate term. There is Poor evidence to demonstratethatthesegainsaremaintainedonthe long-term.The impactof surgery on quality of life and function has not beenadequately investigated. Considerable evidence suggeststhat surgery minimally impacts preoperative function.Predictors of surgical success in regard to patient anddiseasecharacteristics remain elusive. The complex interplaybetween the arrays of confoundingvariables hasunderminedthe capability of most studies to provide well-groundedevidence to support and generalize their conclusions.

Recommendations

–
Comprehensive reporting of all actually and potentiallyrelevant patient and disease characteristics that providescope for determination of predictors of surgical successis prompted.
–
Multicenter studies that allow for greater patientpopulations are encouraged because of disease rarity.
–
Well-designed ethically tolerable prospective random-ized “control” trials in carefully selected patient popula-tion groups should be considered.
–
Validated quality of life assessment scales should beincorporated into patients’ outcome measures.
Conflict of interest

The authors Tamer A. EL-Sobky, Shady Samir,Ahmed Naeem Atiyya, Shady Mahmoud, Ahmad S. Alyand Ramy Soliman declare that they have no conflict ofinterest in connection with this article.

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Cite this article as: EL-Sobky TA, Samir S, Atiyya AN, Mahmoud S, Aly AS, Soliman R (2018) Current paediatric orthopaedicpractice in hereditary multiple osteochondromas of the forearm: a systematic review. SICOT-J, 4, 10.

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Current paediatric orthopaedic practice in hereditary