Document Sample
					VOL.       124,      No.      3

                       STERNAL                                       ANOMALIES                                            AND                         CONGENITAL
                                                                        HEART                                         DISEASE*
                     By      R.      F. LEES,                 MB.,          B.S.,        and        ‘N.      J. H. CALI)ICOTT,                              M.B.,      B.S.,       M.R..A.C.R.
                                                                             NORTH           ADELAIDE,                SOUTH          AUSTRALIA

T           HE         association   ofcongenital                                      heart   dis-
            ease        and premature         fusion                                  of sternal
segments                    is well            recognized.                   However,                      the
incidence      of sternal      fusion    anomalies     in the
general     population,         and the significance         of
a chance     finding      ofsuch      an anomaly     in rela-
tion     to congenital          heart    disease   are not
known                and          were         the      subject              of the            present
    Of 1,915         children      who had consecutive
chest     roentgenograms            at the Adelaide    Chil-
dren’s      Hospital       for various    reasons,  135 had
anomalies                    of      sternal             fusion,             and         i     in             of
these         had            proved              congenital                  heart     disease.
A       by-product                       of    the study                   was     the finding
that    double      manubrial    ossification       centers,
known       to occur      in mongols,        is a common
condition,       but only a few of those          found      by
chance      proved      to be mongols.


    The sternum            develops         in cartilage        from
mesoderm;          by the ninth           intra-uterine        week
the cartilage          model     has assumed             its defini-
tive shape        and is continuous              with the costal
cartilages      lateralh’,      but lacks segmentation.2
The      manubrium            ossifies       from       i or   occa-
sionallv      2 centers         during         the fifth      intra-
uterine     month.         \Vhen       there      are 2 centers,
they          are          seen          to    lie     one        above            the       other             in
the       lateral             roentgenogram                            (Fig.     i).

        The                  segments       for                     the      body                of         the
sternum                    develop              separately;                     each     may                  be
represented                      by           either        I         or        2 ossification
centers,    lying                        side by side (Fig.   2; and                                        3).
The uppermost                               body center  commences                                           to
ossify             during            the       fifth         or sixth            intra-uterine
month                and the others                               ossify    in sequence
from               above      downwards                               with    the  fourth                                                          --   -   e manubrial            ossification     center.
center              appearing      during                          the first year after
birth.             The xiphisternum                                does not ossify      be-
    *    Presented         at the    a4th      Annual        Scientific     General       Meeting,           Royal        Australasian      College     of Radiologists,         Sydney,   Australia,   September
30-October            6, 5973.
   From           the Department              of Radiology,          Adelaide       Children’s            Hospital,         Inc.,   North   Adelaide,       South   Australia.

424                                                                       R.         F. Lees               and           W.     J. H. Caldicott                                                                        JULY,       5975

                           CEI’TRES          01 OSSWICkflO4                                                                   known             congenital                 heart              disease             was            ran-
                                           OF STEHUM
                                                                                                                              domlv           selected             from        the        files      of the            Cardiac
                                                                                                                              Clinic  of the Adelaide        Children’s        Hospital,
                                                                                                                              and their     roentgenograms            were     examined
                                                                                                                              for premature       fusion   of sternal      segments.
                                                                         MS jomt fuses in a
                                                                         minority of adults                                                                           RESULTS
 cenrres of ossification
 may be single or double                                                                                                             Three         patterns               of       premature                      fusion                of
 (side by side)
                                                                                                                              sternal          segments               were           identified:
                                                                                                                                     i.    The        sternal                     segments                      were            com-
                                                                                                                                           pletelv   fused                at       the        time          of examina-
  centre appears                                                         fuse.sin early             clutdhood                              tion (Fig. 4)
  first year of life
                                                                    SlerTlo         ziplosterrial                                    2.    Premature                 fusion              of       segments                 in     the
  centre a.ppears afrer              -                              fuses in adulthood                                                     usual           order           (from                  below            upward)
  3 years
  (may never appear)                                                                                                                       (Fig.       )
                                                                                                                                     3.    Premature                 fusion              in     an       abnormal                  or-
           FIG.      2. Centers                 of   ossification                   of sternum.

fore the third           year   and may          remain       carti-
laginous         throughout        life.    Fusion       between
the      body ossification           centers      occurs     in the
opposite          sequence       to their          appearance,
namely         from     below     upward,         beginning          in
early    childhood,          and is completed            by early
adult     life. The xiphisternal              and manubrio-
sternal            joints                fuse         in        only            a       minority                    of

                           MATERIAL                    AND           METHOD

      The         films        of        1,991            different                  children                who
had     consecutive                          chest              roentgenograms                                     for
various       reasons                      at the               Adelaide       Children’s
Hospital              during               the         first         3    months                    of     1972,

were         re-examined                             to      detect                   anomalies                     of
sternal              and            manubrial    ossification.                                               The
children              were           of all ages up to                                         14         years.
Seventy-six                    examinations                                   had            to          be re-
jected            either        because                     they              did        not         include
a lateral       film of the sternum,                 or the defini-
tion     of sternal           anatomy          was inadequate.
This      resulted          in 1,915        films      which         were
examined           for both        anomalies         of sternal          fu-
sion     and        multiple         manubrial           ossification
centers.       The       case records           of each        child       in
which        one       of the        above        anomalies           was
found       were       searched        for reference            to con-
genital      heart       disease,      in the case of sternal
anomalies,           and any other            congenital          anom-
alv in the case of multiple                      manubrial           ossi-                                                                                                                      ,
fication            centers.
                                                                                                                              FIG.        3. A normal           sternum            showing               both     double           and
      A separate                     group                 of        88         children                   with                                        single      ossification               centers.
VOL.         524,      No.     3                     Sternal            Anomalies             and        Congenital                Heart      Disease                                               425

               der       (upper               or     middle             segments          fusing
               before              lower           ones)        (Fig.       6)

                     PREMATURE                       STERNAL               FUSION

       One           hundred             and          thirty-five                of the 1,915
(7      per           cent)          children              studied               had prema-
turely               fused          sternal           segments               of one       of the
types   mentioned                              above.           Review    of the case
records    of these                             patients           showed     that  24
now            have           proved               congenital                heart       disease
and           that           2 others,                who        had         not       been        ex-

                                                                                                            FIG.        5.    Premature      fusion     of sternal             segments           occur-
                                                                                                                ring from           below   in an infant.        The second               and third
                                                                                                                ossification           centers do not         normally            fuse    until      late
                                                                                                                childhood            or puberty.

                                                                                                            tensively       investigated,           have       cardiac       mur-
                                                                                                            murs,      and       i   of these        had     electrocardio-
                                                                                                            graphic        and        radiographic            abnormalities
                                                                                                            which      also suggest           heart    disease.       The inci-
                                                                                                            dence     of association           of congenital         heart     dis-
                                                                                                            ease did not vary               among       the 3 patterns            of
                                                                                                            abnormal         sternal       fusion.    Ventricular          septal
                                                                                                             defect            (V.S.D.)         was     the      commonest                        asso-
FIG.     4.         A completely                 fused        sternum.           The    xiphister-           ciated            cardiac      lesion (Table                i).
                            num               remains          unfused.                                            In        the    group     of 88 children                     with      known
426                                                  R.    F. Lees           and      W7.    J.      H.     Caldicott                                                                        JULY,         1975

                                                                                            ent.      These               patients               had           a wide              spectrum                     of
                                                                                            other          anomalies                      but       only                        were       mongols
                                                                                            (Table            iii).


                                                                                               In 1956,    Monet    et al.8 reported        a case    of
                                                                                            premature     fusion  ofthe    sternal     segments       in
                                                                                            a child   with congenital      heart     disease.      Two
                                                                                            years          later,           Currarino                    and              Silverman3                  pub-
                                                                                            lished          a series                 of     isolated                      examples               of        the
                                                                                            same            association.                        Since            then              further                iso-
                                                                                            lated          examples                   have             been               reported,”6                     and
                                                                                            recently                   reviews              of         sternal                   anomalies                      in
                                                                                            groups             of children      with  known     congenital
                                                                                            cardiac              disease     have    been    published.”9
                                                                                            Previous              papers   have given     no incidence                                                          of
                                                                                            the       occurrence                     of abnormalities                                of fusion                  of
                                                                                            sternal              segments                   in      the           general                 pediatric
                                                                                            population,                      or       expressed                           the      significance
                                                                                            of      such          a       chance             finding.                     Our          study              of         a
                                                                                            hospital                    of children
                                                                                                                      population          up to the
                                                                                            age of 14 years      has shown      a 7 per cent mci-
                                                                                            cidence      of sternal     fusion      abnormalities.
                                                                                            Furthermore,       just less than       i in      of these
                                                                                            was associated         with  congenital       heart     dis-
                                                                                            ease.          The            potential                ability                  to     predict                pre-
                                                                                            viously              unrecognized                            congenital       heart     dis-
                                                                                            ease    as a result     of an                                      isolated  finding       of
                                                                                            sternal    fusion   anomalies                                        and the relatively
                                                                                            high           incidence                  of         these           anomalies                       in            the
                                                                                            general                   pediatric                 population                         may               be          of
                                                                                            practical                  importance.
                                                                                                Recently,       White     et al.9    have     reviewed
                                                                                            119     children    with    known      congenital       heart
                                                                                            disease      and found     a 59 per cent incidence            of
                                                                                            associated       premature      fusion     of the sternal

FIG.       6. Premature          fusion     of sternal       segments          from
      above.     ‘l’he first      and     second     sternal        ossification                                                           TABLE           I
      centers     normally         fuse   in the late         teens      or early             TYPES         OF        CONGENITAL                 HEART           DISEASE                ASSOCIATED
      adult     life.                                                                                            WITH        STERNAL              FUSION                  ANOMALIES

congenital        heart    disease,    17 (19.3      per cent)                              Ventricular Septal Defects                                           13
                                                                                            Patent     I)uctus     Arteriosus
had     prematurely           fused    segments.        Again,
                                                                                            Conduction         Defects                                                2
V.S.D.       was   the most frequent         cardiac     lesion                             Corrected     Transposition                                               I
noted      in these     17 cases    (Table    ii).                                          .Atrial Septal Defect                                                     i
       Multiple         manu        bri al     ossification               centers           Complex     Anomalies                                                     2

were          found     in ii8       of the      1,915         children         (6.
                                                                                                                                                                 24             out of 135 sternal
per cent).      All            patients   had              2    osSification
centers    except              for I, in which                 3 were       pres-
VOL.      524,      No.     3                   Sternal             Anomalies            and   Congenital                  Heart                 Disease                                                                        427

                                              TABLE       II                                                                                           TABLE           III
  MULTIPLICITY                    OF OSSIFICATION                   IN VARIOUS      TYPES                 MULTIPLE                MANUBRIAL                        OSSIFICATION                          CENTERS
                     OF CONGENITAL                    HEART          DISEASE
                                                                                                   Congenital              Anomalies                                         59             out          of     ii8             with
Ventricular               Septal Defects                                                                                                                                                          multiple ossifi-
Pulmonary                 Stenosis                              3                                                                                                                                 cation centers
Patent           Ductus          Arteriosus                     2                                  Mongols                                                                      4
Tetralogy            of Fallot                                  2                                  Spina        Bifida                                                          4
Aortic       Stenosis                                           2                                  Congenital              Heart               Disease
Atrial      Septal          Defect                              I                                  Celiac Disease                                                               2

Cushion            Defect                                       I                                  Tracheo-Esophageal                                   Fistula
Dextroversion                                                   i                                  Pierre Robin               and Cleft                    Palate                 I
                                                                                                   Mucoviscidosis                                                                 I
                                                               17     out      of 88 known         Thalassemia                                                                    i
                                                                            congenital             Muscular               Dystrophy                                               I
                                                                            heart disease


                                                                                                     I.     ANDRsN,               L., and                HALL,            P. Diminished                              segmen-
body      segments.      Our population                                    of patients                          tation        or        premature                    ossification                    of       sternum                   in
with    congenital      heart   disease                                was also small                           congenital                    heart         disease.              Brit.           Heart             7.,         1961,
(88 patients),       but only showed                                     a 17 per cent                          23,      140-142.

incidence       of sternal    anomalies.                                                             2.     ASHLEY,            G. T. Relationship                                      between                 pattern                   of
                                                                                                                ossification                     and            definitive                   shape              of          meso-

                                                                                                                sternum                 in     man.             7. Anat.,               1956,             o         (Part           I),
    Many          types       of congenital                 heart       disease                      3. CURRARINO,                           G., and               SILVERMAN,                        F. N. Prema-
were      associated            with       sternal          fusion         anom-                                ture       obliteration                     ofsternal                 sutures                 and         pigeon-
                                                                                                                breast            deformity.                       Radiology,                     5958,             70,          532-
alies but ventricular                     septal        defect         was the
most       common            (Table          i).     This      is in agree-                          4.     CURRARINO,                       G.,        and        SWANSON,                      G. E. Develop-
ment       with       the findings               of Fischer           et al.#{176} in                           mental             variant                 of      ossification                    of         manubrium
their       group          of acyanotic                    children             and                             sterni in mongolism.              Radiology,     5964, 82, 916.
adolescents.                                                                                         5. FISCHER,                   K. C., WHITE,      R. I., JORDEN,       C. E.,
    Multiple            manubrial               ossification            centers                                 DORST,             J. P., and NIELL,     C. A. Association      of
                                                                                                                sternal            abnormalities     in patients      with con-
were less common                     (6.       per cent)           than        sug-                             genital            heart                disease. Ase.                       J.     ROENTGENOL.,
gested         by Horns            and O’Loughlin7                     (i        per                            RAD.         THERAPY                      & NUCLEAR                         MED.,               1973,             119,
cent),      and Currarino                 and Swanson4                  (20      per                            530-53            8.
cent),        but      these      latter          reports         related          to                6.     GABRIELSEN,                        T. 0.,            and      LADYMAN,                        G. H. Early
                                                                                                                closure           of         sternal            sutures           and            congenital                     heart
groups        of children           under          the age of 5 years,
                                                                                                                disease.               AM.         J.     ROENTGENOL.,                             RAD.             THERAPY
whereas          ours ranged             up to 14 years                   of age.                               &      NUCLEAR                     MED.,            1963,             89,        975-983.
Our patients              with manubrial                    segmentation                             7. HORNS,               J. W., and                         O’LOUGHLIN,                         B. J. Multiple
had      a wide          spectrum             of other           anomalies,                                     manubrial                      ossification                  centers                in        mongolism.
but      only       4 were        mongols              (Table         III).      Al-                            AM.          J.          ROENTGENOL.,                                 RAD.               THERAPY                         &
                                                                                                                NUCLEAR                      MED.,           5965, 93, 395-398.
 though        duplication            of the manubrial                      center
                                                                                                     8.     MONNET,                    P.,      GRAVIER,                  J.,          GAUTHIER,                          J.,      and
occurs         frequently           in mongolism,                    the mci-                                   VERNEY,                      R. Association         d’une                                transposition
dence       of mongolism                 among           children            found                              vasculaire                    partielle     (dextroposition                                         aortique)
 by chance           to have        multiple            manubrial              ossi-                            avec         retour                 veineux               anomal                   et         d’une              mal-

fication        centers       is low.                                                                           formation                     thoracique                  par          ossification                       pr#{233}ma-
                                                                                                                tur#{233}e sternum.      Pediatric,       1956, II, 95-98.
 R. F.     Lees,          M.B.,       B.S.                                                           9.     WHITE,       R. I., JR., JORDAN,            C. E., FISCHER,
 Department                 of    Radiology                                                                     K. C., LAMPTON,        L., NEIL,      C. A., and DORST,
 Adelaide           Children’s            Hospital,            Inc.                                             J. P. Skeletal    changes      associated      with adoles-
 72  King William                    Road                                                                       cent congenital      heart disease.        AM. J. ROENT-
 North Adelaide                                                                                                 GENOL.,    RAD.     THERAPY         & NUCLEAR        MED.,
 South Australia                    oo6                                                                          1972,       116,             53    1-538.

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