CONGENITAL

BY FRANK SHALEEN Fellow RALPH K. in BABB, Orthopedic

COXA
M.D., Surgery, M.D.,

VARA
MINNESOTA

ROCHESTER, Mayo ROCHESTER, Foundation

GHORMLEY, on Orthopedic

MINNESOTA Clinic MINNESOTA St. Paul

Section AND CARL Chief C.

Surgery, M.D., State 5T.

Mayo PAUL,

CHATTERTON, of Staff, Gillette

Hospital,

Any feired
variety

deciease
as
of

in the
varatm4.

angle
Acquired

formed
coxa

by
vara

the

femoral

neck infrequent

with and

time fenmolal
and may

shaft
be dime

is reto a

to

coxa

is a common

deformity

causes. sufficiently

Congenital often,
The authors

coxa however,

vara to from

is relatively make
therefore,

of unknown clinical
current

etiology. entity
of concepts

It occurs
most

its

recognition
to summarize

as a distinct
the

important.

propose,

this fifteen ing

deformity, cases. Some titles

to

differentiate

it

multiple congenital described:

congenital coxa vara, so-called coxa

a the other form neck types

deformities, as evidenced congenital

.

and

to

repoit

confusion has under which

arisen regarding it has been and by

by the followcoxa vara 8,44 authors

occurring which
.

infantile referring
dren
ciently

coxa vara 1.2, 7,32,44 to a lesion best defined

associated characteristic with to distinguish

developmental Fairbank as
changes in the all

vara
of

These
vara femur deformity which

are
are paper

all

of coxa the of this

H,

in chilsuffiis

and

radiographic

it from

It is this

lesion, concerned.

characterized

by

a vertical

fissure

in the

femoral

neck

with

this

REVIEW

OF

THE

LITERATURE

In
non-tuberculous,

time year Fiorani,

1881, and in

disabilities congenital that had year, been of the

of the dislocation found made, femoral

hip fifteen and neck.

were was

still one cases

being of the that in which

considei-ed more this popular previously rare form w-as attracted of coxa separation
vara vara
41,42#{149}

as tuberculous non-tuberculous a diagnosis of limping by his article, vara 12; however, 42#{149} Hofmeister,
Finally, in 1896,

or

diagnoses. congenital in effect due

of
was

dislocation to a bending

concluded Little

attention

but MUller, in 1888, gave the first full anatomical description the condition he described was apparently an epiphyseal 1894, is generally given credit for coining the term coxa
Kredel gave the first detailed description of congenital coxa

in

3,26,44#{149}Zadek

imas given of eoxa

published

a careful vara his coxa lesion

two

review

of the

early

articles
As though

describing
in answer

this plea

condition. for a moie

paper,

In 1899, Whitman made on time basis of etiology. now famous monograph

an impassioned in which report he of

careful
Hoffa,

classification
in 1905,

to this

reported the

two microscopic

cases,

undoubtedly pathological of isolated

a detailed

congenital findings case reports

14

vara, and included the first in the femoral neck. The period from 1905 to 1913
outstanding papers. Delitala

of the and
of the states

is characterized
reported one case

by a series
and included

account

microscopic that mend

8

pathological in reporting coxa vara as

findings. on two infantile has

submitted
of

This gross coxa been

by Dr.

was specimens, vara universally

the

second was the Although a(lopted.

of the degree

such first this

report. term has

Fairbank much to

Elmslie,

to recommend

designating recom-

congenital

it, it unfoi-tunately
Abridgmimenmt of timesis

not
the

Babh

to time faculty for the

Graduate of Master

School of

of time University Science in Orthopedic

of

Minnesota,

in partial

fulfillment

requirements

Surgery.
VOL. 31-A NO. 1. JANUARY 1949

115

116 From reviewed vascular In gave fusion term

aplasia

F.

S.

BABB,

R.

K.

6HORMLEY,

AND

C.

C.

CHATTERTON

1913 the

to 1924,

very and

little reported

was

written

on this he was

subject. the coxa They vara types first vara. paper

Then, to suggest on coxa at

in 1924, an vara to solve birth of operating

Nilsonne embryonic in general, the under additional table, that

but roentintact

literature

five

cases;

disorder as the etiological 1926, Noble and Hauser, excellent on the description subject
femur

factor in congenital in a very comprehensive coxa varieties them

or absence

an

of congenital including then

the

vara. of coxa four

of othei

attempted present on die

contime

by and
and

all

congenital,
of the

dividing
presence

into to have

time basis
deformities.

congenital

Fairbank, of a massive
whereas the

in 1927, pulmonary
femoral neck

had
was article

the
not

misfortune
visible on infantile on

a patient examination

on the of the
it was,

thrombosis. following of the 1927 year,

Subsequent
the coxa

hip

(Iisclosed Jones

roentgenogram,

in fact,

cartilaginous.
Volume

In

the

Fairbank

contributed
vara,

to time Robert
including a discussion

Birthday

a comprehensive

of the

genographic In the coxa latter cellent to the

recognition years from appeared mentioned

deformity. 1946, approximately seventy references to congenital

Among the

through in the Barrs article

vara have might be and Royal

literature. paper in by Zadek, in 1938;

Twenty of these are in English. 1929, in which he reported five in a paper 1935; by coxa out Ollerenshaws Duncan, vara that in 1938, presidential

cases;

an address

cx-

comprehensive Society

of Medicine,

in which

he differcases; of microcharacterTavernier later, during in gento

entiated between congenital and developmental and Goldings paper, in 1939, in which he pointed scopic pathological findings in the istic about this lesion. Of the foreign
and under Pouzet, the in

and reported thirty-one the nine descriptions nothing and will

literature literature,
and treatment

up

to that date revealed Camitz in 1934, Pouzet reference

.

1934,

have

contributed case been during reports

substantially;
1,IO,1319,27,28,41,43

to them other papers

1939,

be made

discussions

of etiology

Among

this eral
the

period situation

were that

interesting little has occurred

the and
since

ai-ticles w-ritt-en on coxa vara which

War

36,38#{149}Very

written and

on

this

subject-

is analogous

immediately
ETIOLOGY

after

World

I.

Fimere
aimd 1)uncan

is still expressed
held that

no

universal a belief
the condition

agreement that
is the

as to the
result of

cause

of congenital
mi-as

coxa
error.

vai-a.
Pouzet Such

Kredel
and theories

Hoffa

intra-uterine

pressure

responsible.

a developmental

can neither be proved nor criticized 29 J suffices to say that no hereditary factor has been demonstrated. Then there are theories that have not withstood investigation. Rickets, which has never been shown to be coexistent with congenital coxa vara, can imaidly be responsible.
Trauma can be excluded, according to Hoffa, because of the unusual number of cases of

bilateral examination of man.

lesions. showed
in

B#{246}hmsuggested that
a careful

an angle

atavistic

theory,

but

Herz

denied
much

this
less

because
than

an
that the

the

of inclination
of the

of simians
uppei

mi-as not

Walmsley,

investigation

femoral

epiphysis,

proposed

theory
would

of a separately account for the

ossified diaphyseal spur. characteristic triangular

There is no doubt but that fragment, to be described in whicim

the most

such later;

a theory but how com-

this theory would explain the more severe cases, pletely cartilagiimous, is hard to understand. Nilsonne, iii 1924, presented what is perhaps
that of an

time fenmoral
attractive

neck

is almost

etiological

theoi-y,-----

sections,

embryoimic concluded

vascular that it

disturbance. was impossible

Camitz, in 1934, after exanuning histologically to (listingwsh congeimital coxa in the postulation Since
THE

inicioscopic between osteotheory explanacompletely

JOINT SURGERY

chondritis juvenilis (coxa and Camitz observations tion of the phenomena

plana) would observed

and so-called seem to coincide

ill

vaia. Nilsonnes of a plausible neck

OF BONE

this

lesion.

time fenmoral
JOURNAL

is not
AND

(ONGENITAL

COXA

\AlIA

I IT
a
postnatal vascular

ossified mvould

until explain

time cimild the

is at least

four findings.

yeai-s

of age

l2

even
in 1939,

distuibance
hypothesis,

subsequent timis

Piergrossi,

in

support

of this

demonstrated I)uncan
01

Bertolottis
#{176} Supporte(l

met-aphysitis
timeory, and of congenital to explain

as an
many

evolutionary of those
coxa

stage advocated
to be as an

in congenital the
in favor aseptic

coxa infantile
of

vara.

who
vara

term
necrosis

developmental This mo(lern

instead

1,2,39,44 appeared

it..
iS

teimdeimcy

congenital

makes

it all

time more An
cause

essential

that

the

condition be made
be congenital

be to

separated demonstrate

from
is not

otimer
time

obvious that
as this

congenital the coxa

infantile

deformities. vara occurring

lesion,

attempt will also as one of multiple

of which may even

pathologically
same

deformities postnatal.
CHANGES

the

1ATHOLOGICAL

Noble that, at of time femur

and birtim,

Hauser the upper

state end of carti-

is a mass

lage.
verse,

Timere
ascending

is a single,
edge

transof ossifi-

cation fourth 1)older

being the

mvimicim, during year, reaches the of time neck. The

completely ossified
sepaiates

time upper imeck,

at

foiiitim

yeam,

the at
for timat

capital
twelve time greater

epiphysis
months trochantei,

appearing
from

which
only the
in

begins fourth
of

to year.
ossification

ossify Any
of must

in

the neck,
time
FIG.

disturbance

therefore,

occur of life.

first
.

four

years
.

1-A
.

I he earliest
genital coxa vara

stages
are

of coimseldonm

Illustrative cludinmg (OXR

case

of

multiple

vara.

Coronmal

conmgemmital section througim

(lefornmlt-les hip joint.

of

i)ommes,

in-

seen. Ollenenshaw was simown by Herzog roentgeimogm-ams ilhistiating snmall areas which appareimt-ly coalesced to pm-odiice time typical
yeans (low-n, cartilage, cally. This descemmt of timat
jim a

of

iarefact-ion

in imas it from

time

femoral

neck

fissuie,
femoral separates

Sel(loITl
head

evi(lent
definitely time

before
remainder

two
slipped of

of and

age. the What and

By

time appears

fifth to

oi- sixth latemal be time

year to Imead

of is in

life,
reality

the

vertical a triangular

fissui-e

time epiphysis

time neck.

the
will be

anatomical
demonstrated

head,

the

epipimyseal

fm-agment

of time neck,

as

i-oentgenograpimi-

of time Imead, change, except occurs

theim,
for at the

due
the upper

to a defect
more than end underlying Such and

in time femoral
occasional appears disturbance as coexistence to he

imeck,

comistitutes
of a short

the
femur. of an as dis-

gross
aplasia (usse(1 epiphyseal in gross

patimological is the
preceding

Shortening

time femur result

a manifestation in ossification, a vertically

of some
part of timis

common
paper.

clmaiacterist-ics

line pathological

and

anteversion specimens.

of the

neck

are

roentgenograpimic appearance Nilsonne, microscopic

features,

not

(lisplaced so eVi(lPflt

neck
Duncan

1)escriptions have been

Timere

of the microscopic given by Hoffa,

is nothing

pathological Elmslie, 1)elitala,

in

of time defect in the femoral Camitz, Barr, Zadek, and appearance of tissue reof embryonic has been cart-ireported

characteristic

the

move(1 fmonm the femoral neck lage in normal bone, and the
VOL. 31-A, NO. 1, JANUARY 1949

Barr occurrence
#{176}8, 11

reported finding of non-calcified

the inclusion osteoid tissue

118

F.

5.

BABB,

R.

K.

GHORMLEY,

AND

C.

C.

CHATTERTON

FIG.

1-B epiphysis (X 35).

FIG.

1-C
region (X
6).

Section by
Noble and

through

Section
can apparently

through be

mid-cervical said

Hauser.

Otimervise,

little

about patimological

time microscopic criterion

upper end

pathology. congenital the femur and

Therefore, coxa witim

in an

attempt

to arrive
we can

at a fundamental
say that it

for
of

vara as herein an incompletely ; and

defined, ossified

is a lesion

of the

segment

in the

neck

; it is composed femoral and coxa

of cartilage diaphysis. vara con-

osteoid tissue The coexistence

it sometimes includes of multiple congenital

gross aplasia of time upper deformities of other bones

stitutes in this
was

the differential problem. relationship satisfies the

In an attempt to determine whether or not coxa vara pathological criterion laid (10mm previously, such a case

invest-igate(I.
A white girl, aged (-ongemmital
COX1t

ciglmt Spina to

s-ears, bifida have included

msa.s

first

seen was to This fat

at also severe

time

Mayo flexion

Clinic During

in

Septenmber of botim and (lied

1928. knees under shortly

Examination and 1)0th anaesthesia afterward. and arid the

rehips, mm At head,

veaie(l .Jamiuary
necropsy,

flmUltil)le 1929, (heath and vara upper appeared was blocks the a nornmal before

deformimities,

including occuita

club-feet, present.

(leformities distress

ami(I I)ilibteritl

vara. ciiiid found s)ec-inmens l)art

time m-a.s

a nmanipulation

tue

suddenly

experiermced
been one shaft. due hip

respiratory embolisnm. consisting had

The resected
neck,
l)laneS through

joint,

of part i)een

of time inmnormminate sectioned in the

hone

of time femoral

specimen Accurate angle 1-A).

coronal

horizontal

coxa lar

middle of time head anmd neck. to be present, with a coxofenioral in the cut
,

reconstruction was of about 1 10 degrees.

inmipossible, i)ut moderate No gross (lefect or triangu-

fragment Two

noted were

neck

of the

femur

(Fig.

omme to include

epipimyseal

cartilage

amid time other

an(l bone eosirm,

fronmi time mmmid-cervical

neck. mounted. region Both (Figs. blocks 1-B and and Examination

region,
were re1-C).

imicludinig (lecalcified Veale(i

inferior they

cortex
were with

and extending
sectioned, stained

almost
with and

across
cancellous

time width

of time available in the cervical

imenimatoxylin

epipimysis

nornmmal

compact

It logical herein available,

must

be

concluded, out-lined Sucim an help

therefore, previously,
observation,

that

since

the

condition

(lid

not

satisfy

the

pathovara as

criterion defined. may

the case was not one of congenital coxa if made on otimer sucim specimens whenever are two types of
THE

they

are

to prove

that

there

congenital
JOURNAL OF

coxa
BONE

vara
AND

in inf ants,SURGERY

JOINT

CONGENITAL first, a true congenital in ossification lesion accompanied confined to

COXA

VARA

119 other of one deformities, or both femora. and, second,

by the upper

multiple portion

a disturbance

CLINICAL

FINDINGS

formity, at the
not with

Congenital coxa vara is usually discovered such as a short leg, had been previously appearance of a painless limp. Occasionally
until fatigue. adolescence. By this time the undue

when the child starts to walk If no denoted, the parents then become alarmed the limp is ignored and medical aid is
.

requested

presenting

complaint

is a painful

limp

42,

Physical (lemonstrated
hip.

examination when in cases identical

associated

typically child those hip

shortness

reveals and congenital of congenital measured

of the femur.

an coxa

otherwise that vara, shortening,

There

normal the waddling which

is no spasm

child. gait

The and

limp

is easily of the

the with of the

walks,

resembles dislocation.

of congenital

dislocation

In

fact, are
vith

of bilateral

time marked
in those

lordosis

Examination
patients

reveals and and

is considerable
or tenderness,

although

al)duction, inmterimal rotation, greater trocimanter is elevated

the involved surprised to

sometimes prominent physician, telescoping of the

other congenital

extension and the

are mechanically Trendelenburg

test

limited 42#{149} Time is positive on is timen head is

case.

side find

42

The

that

examining there is no the pulsations

no

suspecting a congenital of the femur and that femoral artery.

deformities
EXAMINATION

dislocation, the femoral

in a typical

still

palpable
As mentioned

beneath

previously,

are

present

ROENTGENOGRAPHIC

Roentgenographic timis deformity. Unless

examination one is familiar

offers with

the the

most condition,

readily the

available correct

means diagnosis

of

studying

is usually

not

made.

The

femoral part than

that

neck

is at once

observed
it

to be bent,
26#{149} The

so that epiphyseal branched

superior

the

head

is depressed head V inferior medial is

42#{149}

and the distal more vertical

Brailsford states

of the limb is thereby it is normally, and

this abnormal

adducted appears
runs

to
from

be
the

line for the like an inverted

medial to the

branch

lateral quadrant. segment process

osteochondritis

extremity sometimes
resulted
6

of the

neck,

and

isolates

a triangular of the fissure,

that seen

fragment line,
aseptic

in the but
necrosis

inferior

It- is not,
,

however, referred
in a defect-

a branching to as the
not

epiphyseal in which
in

a disorganised ossification
18

vertical
unlike

an abnormal

has

or

localized The of the ini)ut

The femoral acetabulum. feriorly

At

greater head ; shadows

first glance,

trochanter is comparatively acetabuhim resembling

such

is elevated large,
may

and somewhat

may

have

a peculiar and and have

a fracture

beaked lies shallow been

of

appearance. bottom (lefective

42#{149}

translucent, in outline,
suggests

in the and
femoral

The

be (leformed seen are

those fractures
formed

in osteochondritis extremely
neck,

not-ed
the

a roentgenogram

neck,

Zadek
discloses

has
a

stated
short,

that

such

rare
a
25,43,

in children.
of rarefaction

When

closer
which

scrutiny
contains

imperfectly

with

zone

osseous nuclei and teria for a correct

apparently to an

cuts off a triangular diagnosis. When,

extensive failure

fragment a-s occasionally

at

one should have the necessary happens, the femur is short,

upper end, the diagnosis

enowing needs a

of ossification

the

is obvious.

It is felt 14 that any such more specific designation. The of the

was

lesion that

w-ith is seen

roentgenographic during adolescence this

features or later

so characteristic is much the state dissolution neck be present.

does this

untreated

lesion ununited a femoral may and

more

deserving

diagnosis, originally trochanter

strangely

fracture. defect has much true telescoping

In effect, now become higher

present

is usually a complete the may

Not

of affairs: What of continuity. may

late

The
head,
VOL.

be riding

than of the
and

at first, femur
viable.

deficient now
only

have The
lesion

been
re-

completely

absorbed,
enough,
1, JANUARY

femoral

is usually
1949

31-A,

NO.

12()

1.

S.

BABEl,

II.

K.

GHOILMLEY, 3 i.
.::

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TI1I

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OF

BONE

AND

JOINT

SURGERY

CONGENITAL

COXA

VARA

121

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Vol.. 31-A,
NO. 1. JANUARY 1949

;;

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2-

122 semble an ununited as such.

F.

S.

BABB,

R.

K.

GHORMLEY,

AND

C.

C.

CHATTEHTON

fracture,

but

also,

as will

be shown

later,

it should

probably

1)e treated

DIAGNOSIS

1mma cimild,
l)aiIiless

therefore,

a diagnosis

of congenital

coxa

vara

should

be considered

for

any

palpable
considered

limp resembling a congenital beneath the femoral vessels suggest

before diagnosis

dislocation of the hip, if the femoral head and telescoping of the femur is not present. of the
a rare

is still If the be is in
is done

roent-genograms if the
fact

a fracture
of such

femoral
condition

neck, for an

the ununited

criteria
No

outlined
harm

should that

is made.

particular

lesion
what

in an
is present,

adolescent
even

person though the

is mistaken etiological
TREATMENT

fracture,

since

agent

is non-traumatic.

Congeimital O55it1CLt-iOfl

rarely
sequelae

coxa vara occurs,

resultant

was

and

originally considered the condition proceeds

a disruption of the

hopeless. to an
hip joint,

If it is untreated, established non-union

including arthritis,

nonmal with
pain,

the and

usual

upon

increasing disability. A conservative attitude

has

been

recommended limb in wide of reconstruction more

by

by

Barr

and

by Nilsonne.

They

have

advocated Barr has agreed

The
ossification

non-weight-bearing with the suggested that a Brackett type that

aim
of

abduction and under traction, and be done after puberty. Nilsonne deformities.
n, is twofold : first, to promote

osteotomy
of treatment,

is necessary
as

for the
recommended

marked
Pouzet

the

cartilaginous intervention neck of the

neck,

and,

second,

to correct

the

deformity.

It is generally aims. Surgical In the

agreed
procedures

timat

on

surgical the

is necessary to accomplish femur have proved universally

both of these unsuccessful

child,
27.29,31,33,

(lefect-,

subtrochantotic osteotomy has by far the greatest number of supporters II, 14, 17,23, 11,43,44 In correcting the deformity, this procedure converts what was a vertical across wimich played a sheering force, into a horizontal defect with a compression This has almost
to

force.
Zadek,

universally
the osteotomy,

proved
drills

successful
the neck,
.

in promoting
such a procedure was The objection

ossification.
does raised not by

Althougim
appear Noble to and

in addition

i)e necessary. Hauser what

limb

Neither

is a bone

graft

required

that Duncan
at the

subtroehanteric had in mind

time of osteotomy.

osteotomy when he

offers only urged extreme Peabody 31, in fact, that portion

temporary abduction suggested

benefit. This seems to be of the distal part of the placing the limb in adduc-

tion ment
by

before the be brought

is to with
to Blount

osteotomy into line be maintained.

the

is done. He claimed with the proximal

With

only in this way could the distal segof the neck, an essential if maximal fixation, such as that secured
may be as subtroehanterie osteotomy

correction

the

advent
procedures

of internal

blade-plate, i)ett-er

such results.

(Xpecte(l

In
ilig
it

yiel(l even time adolescent

fracture of found type
heads

or a(hllt

group

of patients,
neck. in Should this Of favor type

time ploblem
the reconst-I-uction of repair because of

is essentially
operations high incidence impossible, prove the

that

of treatavailable, of viable selection

aim tiimtiiiited Brackett

of time femoral is particularly

operation. at

repair

femoral of time

alternative

is obviously

governed

by

the

conditions

encountered

by

the

operating

surgeon.
PROGNOSIS

If the shaft correctly,

ILh)dtlctiOfl l)mLti(11ts

congenital

coxa

vat-a

remains

untreated,

the

head

slips

farther

and

farther

down

of the the
is gait

femur greater

until

it comes
owing to iLIl(I

to lie on the atop the atRi

impingement l)aitm

floor
of

of the shaft,
trochanter to

acet-abulum. goes higher

agaitmst

To state and
time

it more until
The

trochanter,

femoral
the add fatigue

higher
ilium.

impossii)le, i)((01fl(s

\VO1S(,

thu (usability.
O1
hONE ANI) JOINr I4IJRGERY

viii;

J()(RNAL

CONGENITAL

COXA

IARA

123

FIG. Case 15.

2-A of hip.

Roentgenogram

FIG.

2-B
(

Fn;.
)f lnnimonal of fenmonal

2-C
(

5(ctionIs I niset shovs

VOL.
31-A.
NO. 1,

fnonnm (listal (-(1gm gross aI)l)eananire

JANUARY 1919

hi(a( I . No h(-a( I.

(-nhl)1-yonlal

(ant i lag(-

on

)st-noi(

I I ISSU(

is

visit

,l-.

I 24 It has years, result

Such

P.

5.

BABE,

II.

K.

GHORMLEY,

ANt)

C.

C.

CHATTERION

been

said

that

with and
constitute

efficient

immobilization,

Imealmg

occur-s
tried to were

vithuim

about

t-vo

just with
treatment

as in Legg-Perthes complete rest

does not

disease. Early invest-igatoi-s the linmb in abductioim and

adequate immobilization

who
ti-action

accomplish (lisappointed.4 eit-Imer

conTecte(1

timis in coa
into a

of time defect-,

vara or in fracture horizontal defect

chanteric osteotomy

of time femoral neck. Until the vertical (lefect imas been witim a compression force a-cross it, healing will not take
accomplisimes this effect, and the out obviously six been to years of entimusiasnm the claims not age. so for ma(le good Some of this

Place. form for as it. in time the

Subt-noof treat-

ment,
treated

p1115 the
The by
prognosis

results
in

in this
adolescents

series
osteotomy

and
and

otimers,
adult-s at about have

bears
is

child
can
opela-

subtrochanteric

latter
iiomvever,

even
irreversible lions

expect
on adult

a imip functionally
traumatic arthritis

as good
may

as on the
a hip

normal

side.
established.

In

older

l)eisons,

already to restore

Hecoimstiuction

Imips

are with

not the

likely

imormal. and

Therefore,

tue

ptogimosis

itt

these

hips

mi-ill vary

condition,
CASES iN

time patient,
TI-lB PRESENT

time sut-geon.
STUDY

THE

Of time fifteen 1927,

Cases referred ten 11 to since to 15, are

cases
1937. inclusive,

reported
Cases were in 1 to Tainle

here,
10, I. tteated

all of the
itmclusive, at the

patieimts
were treated Clinic

imave
at

beeim

seetm or treated
State

sinmce

Gillette

Hospital;
numi)ers

Mayo females

(Table

I).
was

The

case

timose

given

This
hipalone

series
was

included
involved

seveim
in nine

males
cases,

and

eight

; onie in two live

gii-l
cases, was

a imegro. an(l botim vere

Time hips in

rigimt time

time left

imip alone infancy.

other four cases. Time aenage age at majority of patient-s had ima(l symptoms

time time
ft-urn

of a(lmissiolm

8.8

years,

although

the

of time l)mmtielmts

1)011m wit-h

JIG.

3-A of right trip,

-ilii;

(use

11)1(itl

((mmIg(-niital

C(IXIL

vara

at tlnn-e yettis of age.

JOURNAl. ()I 1l(NI-.

AND

.O)1NT

SI

CONGENITAL

COXA

VARA

1 2?
time shot-tening was In

the
to or

involved hypoplasia wad(lling the limp

exttemity
of time upper

obviously

slmott,

and

in each

case

end
onset

of the
shortly

femur.
after

Seven
they

patients
had begun

complained
to walk.

(Inc primarily of a painless limp

time other three

gait,

witim

cases y(ans,

(hi(l not
The

(levelop
last two

until
patients

the

iPSj)PCti\el.

ages of five years, twelve years, and thirteen Imad only minor (iegnees of this (leformity. lime

Fn;.
Al)1)CLrLm1CC

3-B
years. Inimnl((hiately

FIG.

3-C after operationi.

at age of eight

FIG.

3-D after operation.

Three
VOL. 31-A, NO. 1, JANUARY 1949

years

I 26

F.

5.

BABB,

B.

K.

(HORMLI\,

ANI)

C.

C.

CHATTI-IITON

be

be

--a

C -C

a
C

I2

S
C

C
be

S S S S S
C S

be
C-

S S

c-i
S

TUE

JOURNAL

OF

BONE

AND

JOINT

SURGERY

CONGENITAL

COXA

VARA

I 27 birtim
patients

average

age

at

operation

was

1 1 .5 years.
except

The

family
that only

histories,
two

histories,
were smaller

and

pimysical
in stature

examinations

were

not

remarkable,

FIG.

4-C hip, at age of fourteeni years.

Inmmnimt-(liatelv

after

operationm

omm left

FIG.

4-D after last

operation.

Eighteen
VOL. 31-A. NO. 1, JANUARY 1949

months

I2
th1LI1

F.

S.

IIABI4,

11.

K.

CHORMLEY,

AND

C.

C.

CILATTERTON

imornmal were Surgical

(Cases noted

1 and
except

10).
in was

Apait Cases 8,

fiom
10,

shortening
14, and 15, 8, coxa to

of the
which and

femur-,
special

imo congenital
reference will

niebe

formities
made. No had

tteatment short tibia

carried
on the left,

out
and

in Cases
the

10,
vara

14.

In
right

Case
hip

8 the
was coxa

a congenitally

of the than

patient an mciand time

vara.

dental
flattening

finding.
of the

The
head

roentgenogram
mi-as more

in this
character-ist-ic

case

did
of coxa

not

reveal

a vertical
of congenital

fissure,

plana

FIG.

5-A

Case

1 1 . lvpical

congemutal

coxa

yam

mm a

young

a(lult

, at

age

of t-wemity-tvo

years.

FIG.

5-B after subtrochanteric osteotomy (Brackett

FIG.

5-C
was technically

Fig.

5-B:

Immne(Iiat,eiy

reconstruction

inmipossible).

Fig. 5-C: Three

months

after

operation.
THE JOURNAl. OF BONE
ANt) JOINT SURGERY

CONGENITAL

COXA

VARA

I 29 and a history
and Case follows. 14, perhaps

In

Case
in

10 there
The this series. from

were

multiple
in case intetest This

congenital
timis case is illustrative,

deformities
are not as typical, is also

of birth
time case

injury
does of congenital

and
not

rickets. belong hip Case

roentgenogtams

of time type

deformity

wimich

congeimital
and

coxa

aia,

as imeiein
report

defined,

5110111(1 i)e distinguished.

15 is of panticulat
CASE
15. A

a detailed
was extremity an higher head could presenmt the ilium shorter on first seen had otimerwise than the not in the (llaphysLs

wimite timat

i)oy,

aged

niinme years, right lower revealed centinmeters) and femoral head

at been the right

the

Mayo

Clinic sirmce

iii

March

1947. and whose

His family right confined

complaint histories lower to test ves.sels extime on and renot rami to visible; were

on admission was were not renmiarkable.

tremity femur. the vealed trochanter right the telescoping wa-s Time seven trochanter of the faint i)einig surgical

a simort Exanmination (17.8

wa-s

present left, than of the the

birtim.

Birth boy,

normal

nine-year-old shortening the felt was but The ossified with inferior head left; beneath on be hip

inches

all being
the the femoral neck and

prominent the present,

Trendelenburg Rocntgenograms was

for epiphysis

wa-s positive.
fermi ur
upper

Since was

definitely acetabulum, was 2-A).

congenital still on

dislocation

suspected. the femoral

time

outline

of a fennoral

gross aplasia ununited,

Upon of the performed. No

of the

portion
and riding

of the
high hip, (Fig. or

femoral

evident,

the greater

present

(Fig.

ischial wa-s found,

pubic stuck

and the upper

acetabulunm. fenmoral It neck, was

end of the right

of the removed

fibula
2-B, osteoid,

was absent.
incompletely and a modified time floor imip was the distal inset) was Colonna grossly. reconstruction A block of the cut from

exploration

a small,

cartilagitmous

demonstrated

was

edge of time head, decalcified, sectioned, cartilage or osteoid tissue, suggestive In genograms
case
explains
13

stained
of a remnant

with
of

hematoxylin the neck, that with

that

and eosin, arid mounted. could be found. of a congenital dislocation. neck,

had no neck,

No embryonal

this
Surgical

case suggested

the

lesion

resembled coxa
however,

clinically vara
revealed

Roenta fact which

a congenital observed

a cartilaginous
the femur

as in Fairbanks probably be classiaplasia of of course, patients

in Case

exploration,

the

telescoping

on clinical

examination. congenital right fibula.

This

should

fled as a coxa vara time right innominate

is of more

associated with multiple bone, right femur, and

deformities, including Such a differentiation,

In the operated

than academic interest, eleven remaining cases upon; ten

w-as

as the treatment is obviously different. (exclusive of Cases 8, 10, 14, and 15) the had osteotomy.
vai-iety

were and
7, no

of the
of the

patients
subtrochanteric

In six cases
and, except

(Cases
for

5, 6, 7, 9, 11,
a wire

12) osteotomy internal fixation

rection of the

was
deformity

required.
and the

Uniformly
promotion

good years

results of age

were
being

obtained
secured

in these
in every

cases,
instance.

cor-

of ossification

It is felt and that

that wide

in the abduction

child

of six of the

to eight limb after

this

procedure
.

is the

one

of choice, higher age

osteotomy

is essential

In time next

group, wide abduction is more difficult to obtain. 13) , whose average age was thirteen years, iequired of the fragments mi-as used to advantage.

Four such patients high osteotomy,

(Cases 1, 2, 4, an(1 and internal fixation

One patient (Case 3) underwent a reconstruction operation. This patient was sixteen yeais of age at the time of operation and imad what amounted to non-union of the femoral neck, with a viable and movable head. A Brackett type of reconstruction, followed by transplantation of time greater trochanter, proved successful. The 5-C, ioentgenogtaphic appearance in Cases 1, 2, and 11 is shown in Figures
3-A
to

inclusive.
SUMMARY
AND CONCLUSIONS

Congenital

coxa

vara

is an infrequent

deformity

of the

femoral

neck

in children,

rec-

ognized since 1896 and characterized by a defect which pathologically and graphically is not unlike that seen in aseptic necrosis. Except for its frequent with a short femur, congenital coxa vara seems to occur entirely apart from genital deformities and to possess recognition of it as a separate entity. The
VOL. 31-A,

roentgenoassociation other conto short warrant leg or

sufficiently cimild

distinguishing w-ho is born

with

characteristics an obviously

lesion
NO. 1.

is to be suspected
JANUARY 1949

in any

130
who
a

F.

S.

BABB,

It.

K.

(tHORMLEY,

AND

C.

C.

CHAI1ERTON

limps

wheim

walkirmg

is first
of time

attempted. Imip, with

The shortening,

findings high

on

physical

examination of time trochanter,

suggest and

congenital

dislocatioim

position

a positive Treimdelenburg test. However, still palpable beneath the femoral vessels the diagnosis if the lesion is not mistaken
a rare condition

there is no telescoping and the femoral at the groin. The roentgenogram will for an ununited fracture of the femoral ossification in the and
axis

head is confirm head, to corof strain

complete

The
rect any

aim part

in children. of treatment

is to promote present. will correct the

force

neck

of the with
neck

femur wide

and abduction

deformity

already of the
into

Subtrochanteric coxa
along

osteotomy vara,
the

the
across

distal
the

limb

conversion
of the

of the

sheering

defect

a compression

promotes

ossification Adolescent

in a high and

proportion adult neck. patients

of cases. with untreated lesions exhibit what amounts to nonBrackett found at inavailable for high percentage is fairly 8, 10, such lesions, the of viable heads and 15), from is not femur vara
which

union of the femoral operation is particularly operation.

The

Of the reconstructions suitable because of the cases reported coxa vara the specific congenital association factor, osteotomy of the limb, possibly
is not the

series four

of fifteen

here

representative 14, and

purposely which

eludes seem

examples

of unusual

(Cases

it would con-

advisable to differentiate It) can be said, then, that deformity. to a common

multiple

lesion defined in this paper. coxa vara as herein defined of this

same

a proved

geimital due
witim

The
congenital

frequent
deformities

lesion
lesion

with necrosis.
as

a short Coxa
with that

is probably associated
this paper

etiological

avascular about treatment six to

is conceined. abduction

Subtrochanteric of the distal part

at is the
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VOL.

31-A,

NO.

1. JANUARY

1949