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CONGENITAL DISLOCATION OF THE IDENTICAL TWINS

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					          CONGENITAL                              DISLOCATION                                     OF        THE           HIP             IN           IDENTICAL                            TWINS

                                         M.      GEISER,                BERNE            AND        P.      BURl,           BIEL,           SWITZERLAND


                                                       From           the Orthopaedic              Deparuneizt,           Inseispital             Berne,
                                          and the            Medical          Department,            District        Hospital.           Biel,        Switzerland


          As early             as 1678          Pare          stated          that       lame      people           beget         lame           children,            and         in 1882              KrUnlein
 compiled              the      family         trees         of many              generations               of children                  with          congenital                dislocation                of the
 hip.        In     series           of patients               with          congenital             hip     dislocation                  a hereditary                      factor           was         noted        in
 202     per cent by Isigkeit                            (1928),   in 227                       per cent by Scaglietti          (1932) and in 6l per                                                     cent        by
 Francillon      (1937).   Mercer                          (1950)    stressed                     the importance        of the hereditary         influence.
         A different     emphasis                           is given      by                   those    who     believe    that     the essential        cause                                              of      the
 dislocation              is intra-uterine                    pressure     from                 oligohydramnios                 with unusual     transverse       lie, breech
 presentation               and prolonged                        abnormality                     of position              of the femoral     head     in relation       to the
 pelvis        during          intra-uterine                  life.         The      particular           relation          of this              to anteversion                     of the          upper         end
 ofthe         femur          was      discussed              by Krida               (1928),       Le Damany                     (1908),              Badgley         (1943)              and       Somerville
 (l953b).              Lorenz   and              Reiner  (1905) ascribed   an important                                                   role        in the         etiology               to hereditary
 influences,             but at the             same time tried to develop    a mechanical                                                        theory.
          It was          expected             that      a study             of twin           children          might           yield       a decisive              contribution,                       and      this
 was in           fact      possible             in the               remarkable                series    reported        by                 Idelberger                (1951),                  who        himself
 examined                138 living            identical               twins with              congenital       dislocation                      of the             hip.          Before              discussing
 his conclusions  we report    an example                                                   of congenital                  dislocation                    of the            hip          in Italian              twin
 sisters who came north    to Switzerland                                                 with the influx                 of italian                   workers.


                                                                                          CASE           REPORT

            Maria         and        Giacomina                 B. were            born         in 1932          in the      neighbourhood                           of Brescia                  in Northern
 Italy.           They   were identical twins   as shown    by the identical                                                                       phaenotypus,       the genotypical
 identity          of the ABO and Rhesus      systems,   and the Kell, NN                                                                        and Duffy      blood    factors  which
 were        present           or     absent           in like          quantities.               The       twins        had         found             difficulty           in learning                  to walk
 but were first seen by a doctor                                        at the age of three years.                                They limped                       but had               no pain.   Even
 now at the age of twenty-five                                          years  Maria  has no pain                                 and cleverly                      conceals               her limp;   but
 Giocomina                   developed      pain in the dislocated      hip after                                        an appendix                       operation                and         has      recently
 been        treated           by subtrochanteric      abduction     osteotomy.
            Figures           1 to 4 show the clinical      and radiographic                                            appearances.                        These          are          mirrored           almost
 exactly  in the two girls.                              Giacomina                    has congenital     dislocation of the right   hip                                                         with a false
joint above the acetabulum,                                 a stress              fracture   of the right pubic bone and a left-sided                                                            convergent
 strabismus.                 Maria        has         a congenital                dislocation               of the       left hip            with         a false          joint,          stress        fracture
 of the        right         pubic       bone          and       right-sided                strabismus              concomitans                        convergens.


                                                                                            DISCUSSION

          These           mirrored               defects               in     twins         are      well        recognised                      as     favouring                   a     vitium          primae
formationis,             as demonstrated                               for example       in Speemann’s         string    experiments      on salamander
eggs,        in     which   the resultant                             identical    twins    showed     bilateral      symmetry       in the 50 per cent
occurrence        of situs   inversus.     Ophthalmologists      report  that when strabismus         occurs in identical
twins     it affects     opposite      eyes in 70 per cent.        We cannot    explain       the fact that the fatigue
fractures     occurred       in the right pubic         bone in each child.   Their     occurrence     on the same      side

314                                                                                                                            THE       JOURNAL             OF     BONE          AND       JOINT        SURGERY
                                      CONGENITAL                      DISLOCATION                  OF     THE          HIP      IN      IDENTICAL              TWINS                                       315

does       not      suggest           a direct            hereditary               influence           ; but      on the other                 hand       if the fractures               developed
from        simple           mechanical                   stress         it seems             likely      that         they       should          both        have       developed               on        the
side      of the normal                    hip with             its greater            weight          bearing,             or both        on the side             of the dislocated                       hip
with       its porotic              bones.
        Congenital                   dislocation        of the hip in twins has been                                              reported       by Hale (1926,           one case in
identical     twins),                Hooff       (1928,    one case in identical   twins),                                          Nitsche      and Armknecht             (1933, one
case in identical                       twins      and one in non-identical         twins)                                          and     Ihlenfeldt       (1948,      one case in
identical     twins).                   Idelberger       (1951)  reviewed    the reports                                          of 22,004       congenital        dislocations    of
the hip and found   among     them                                      236 pairs             of twin           children.      He personally       examined      138 living
identical twins with congenital                                        dislocation              of the          hip.      From  his investigations        he showed     that
there        was      no         increased                incidence             of     this      congenital                  disorder           in twin          children        ; in          fact        the
incidence            tended            to be            less     in twins             than       in the           average            population.                A similar              conclusion
was reached    by                   Hass (1951).     Clearly       such                           intra-uterine     constriction       or pressure       as may arise
in twin pregnancy                      has no decisive       influence                              in the occurrence         of congenital     dislocation    of the
hip       as has      been           asserted             by the         supporters               of mechanical                      theories          of etiology.             Furthermore,
there       was      no greater                  incidence              of breech              presentation                  or other           abnormal             position           at birth            in
Idelberger’s               twins          than         is the        average          with      twin      births.             The       frequency             of other         malformations
corresponded                  in these                 twins   to that recorded                           in single     babies.                    Of the identical                 twins     with
congenitally               dislocated                  hips 427 per cent were                           concordant-that                         is, both twins had                 a dislocated
hip;  whereas     the concordance                                     in binovular                twins is only 28 per cent.*
      The proof      that mechanical                                    influences               of the twin pregnancy     do not play a decisive                                                       part
in the occurrence        of dislocation                                 of the hip,              and the great concordance     in identical twins,                                                    show
that hereditary        factors    are of prime       importance       in congenital      dislocation.         The hereditary
process   seems,       according      to ldelberger,       to be dependent          on sex, and        to be an irregular
dominant       and of varying         penetration.       In idelberger’s      large   series     of identical       twins   there
were many        cases     in which     the correspondence          extended     even to anatomical             details   of the
dysplasia            and       even         to some                of the          secondary             osteoarthritic                  lesions.             However,           the      fact         that
congenital       dislocation                    of hip is determined                              by hereditary                   factors           does not            mean       that genetic
factors    alone      bring                 about   dislocation.     The                          part played                  by external             factors,         such      as extension
of the       hips      resulting             from            upright           posture          and      gait,         is quite         plausible.             It is known              that      many
hips       do not          dislocate              until        the     child         begins        to stand             up and           walk.          But     these       external            factors
can       cause      dislocation                  only          if dysplasia             of the          hip         is present.           The         dysplasia         of genetic               origin
seems   to be                the prerequisite                      for the             development                     of typical    congenital    dislocation       of hip.
This inherited                 dysplasia     can                 be present              in varying                  degree:   often the dysplasia       is so slight that
the external             causes      are                not      enough               to produce                dislocation,               yet        it is sufficiently               marked               to
predispose             to osteoarthritic                        changes              at a relatively               early age.
          The       idea      that         congenital                dislocation               of the          hip     develops            as an inherited                  dysplasia            of the
acetabulum                 and       upper             end       of the        femur,           whereby               the     upright           posture         cannot          be supported,
has an influence    on our                              choice  of treatment.      The inherited    dysplasia     prejudices                                                    every           form of
treatment,   as experience                               has proved.     Reduction    of the dislocation      alone suffices                                                     only          in a few
cases-namely                       those          in      which          the       dysplasia             has         reached            only      moderate              proportions                   or       is
discovered             very     early.     Reduction       alone   does not correct         the disturbance    in the mechanics          of
the joint           which       is associated        with the dysplasia.       The aim of treatment           therefore     consists     in
restoring            functional        conditions        as nearly    normal    as possible        in the dysplastic    joint     and in
allowing           function       of the joint as soon as possible.          Early use has a favourable          effect on dysplasia,
whereas            prolonged         immobility       has an unfavourable         influence.      Even with treatment        that fulfils
the requirement                      of restoring   early function                                (Somerville                   and Scott    1957)                 there remains                  a risk
that osteoarthritis                    will develop    years later                              in consequence                    of the original                  dysplasia.

*   The     concordance               of congenital                  dislocation         of the         hip      in identical           twins thus exceeds       that in the case of
congenital         club foot.                    found in 242 twin pairs with congenital
                                          Idelberger                                                                                     club feet a concordance      of 229 per cent
in identical         twins         and of 23 per cent in binovular twins.

VOL.      41 B,      NO.      2,    MAY          1959
316   M.    GEISER      AND       P. BURl




            FIG.     1-Giacomina




           FIG.    2-Giacomina.




                                        THE   JOURNAL   OF   BONE   AND   JOINT   SURGERY
                      CONGENITAL   DISLOCATION    OF THE     HIP   IN IDENTICAL   TWINS   317




                                             FIG. 4-Maria.



VOL   41 B, NO.   2, MAY   1959
318                                                                     M.        GEISER          AND       P.    BURl


                                                                                         SUMMARY
1    .   Congenital   dislocation       of the hip in identical   twins is reported.
2.       The heredity      of congenital      dislocation   of hip is discussed.                                                           Studies           in twins       show         that
congenital          dislocation                 of hip is probably      a hereditary  dysplasia                                          of the      acetabulum             and        upper
end of the          femur,      and             that external   factors   play a less important                                            role.

We wish to thank               Dr       A. Haessig,            of the     Central           Blood         Laboratory           of the Swiss           Red      Cross,     for    the    blood
examinations.


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