VOL.
No.
124,
3
STERNAL
By
R.
ANOMALIES HEART
F. LEES,
MB.,
B.S.,
and
NORTH
T
HE
association ofcongenital and premature fusion
ease segments
is well
recognized.
AND DISEASE*
CONGENITAL
J. H. CALI)ICOTT,
‘N.
ADELAIDE,
SOUTH
M.B.,
B.S.,
M.R..A.C.R.
AUSTRALIA
heart disof sternal
However,
the
incidence of sternal fusion anomalies in the general population, and the significance of a chance finding ofsuch an anomaly in relation to congenital heart disease are not
American Journal of Roentgenology 1975.124:423-427.
known
and
were
the
subject
of the
present
investigation. Of 1,915 children who had consecutive chest roentgenograms at the Adelaide Children’s Hospital for various reasons, 135 had anomalies
of
these
proved
had by-product
A
sternal of
fusion,
and
congenital the study
i
in
of
heart disease. 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. ANATOMY
The sternum develops in cartilage from mesoderm; by the ninth intra-uterine week the cartilage model has assumed its definitive shape and is continuous with the costal cartilages lateralh’, but lacks segmentation.2 The manubrium ossifies from i or occasionallv 2 centers during the fifth intrauterine month. \Vhen there are 2 centers, they
the
are
seen
lateral The
sternum
to
lie
one
develop by
represented centers, lying The uppermost
separately; either I
at the
of
the
3). to
and the others above downwards appearing during The xiphisternum
6, 5973. the Department
in
side by side (Fig. 2; and body center commences
during
Presented
other
be
month from center birth. *
the
(Fig. i). the body
each may 2 ossification
ossify
30-October From
above
roentgenogram segments for
the
a4th
fifth
Annual
or
or sixth
intra-uterine
ossify in sequence with the fourth the first year after does not ossify beScientific
of Radiology,
General
Adelaide
Meeting,
Children’s
--
Royal Hospital,
423
Australasian Inc.,
North
College Adelaide,
-
e manubrial
of Radiologists,
South
Australia.
ossification
Sydney,
center.
Australia,
September
R.
424
CEI’TRES
F. Lees
and
W.
01 OSSWICkflO4 OF STEHUM
J. H. Caldicott known
congenital
domlv
5975
JULY,
heart
selected
from
disease
the
files
was
of the
ran-
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 (side by side)
Three
sternal i.
centre appears first year of life
fuse.sin early
2.
ziplosterrial
SlerTlo
centre a.ppears afrer 3 years (may never appear)
clutdhood
fuses in adulthood
-
American Journal of Roentgenology 1975.124:423-427.
2. Centers
of
ossification
of
were
The sternal pletelv fused tion (Fig. 4)
premature
fusion
segments
at
order
of
identified: the
fusion
Premature
usual (Fig. 3.
FIG.
patterns
segments
were
time of
segments
(from
com-
of examina-
below
in
the
upward)
)
Premature
fusion
in
an
abnormal
or-
of sternum.
fore the third year and may remain cartilaginous 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 manubriosternal
joints
fuse
in
only
a
minority
of
adults. MATERIAL
The
films
of
AND
different
1,991
had consecutive various reasons
chest at the
Hospital
the
were
during re-examined
sternal children
and were
Seventy-six jected
METHOD
first to
3
months
detect
because
who
of
had they
for
1972,
anomalies
manubrial ossification. of all ages up to examinations
either
children
roentgenograms Adelaide Children’s
did
of
The 14
to not
years. be reinclude
a lateral film of the sternum, or the definition of sternal anatomy was inadequate. This resulted in 1,915 films which were examined for both anomalies of sternal fusion 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 congenital heart disease, in the case of sternal anomalies, and any other congenital anomalv in the case of multiple manubrial ossification
centers.
A separate
, FIG.
group
of
88
children
with
3. A normal single
sternum ossification
showing centers.
both
double
and
VOL.
No.
524,
der
Sternal
3
(upper
before
or
lower
middle
One
hundred
per
turely
cent) fused
sternal
mentioned records of these types
have
and
that
proved 2 others,
segments
Disease
425
fusing
6)
of the 1,915 had premaof one
of the
Review of the case showed that 24
congenital
who
Heart
FUSION
thirty-five studied
above. patients
Congenital
heart
had
not
disease
been
ex-
American Journal of Roentgenology 1975.124:423-427.
now
(Fig.
STERNAL
and children
and
segments
ones)
PREMATURE
(7
Anomalies
FIG.
5.
Premature
fusion
of sternal
ring from
below
in an infant.
ossification childhood
centers do not or puberty.
segments
The second normally
fuse
occur-
and third until
late
tensively investigated, have cardiac murmurs, and i of these had electrocardiographic and radiographic abnormalities which also suggest heart disease. The incidence of association of congenital heart disease did not vary among the 3 patterns of abnormal sternal fusion. Ventricular septal
FIG.
4.
A completely num
fused remains
sternum. unfused.
The
xiphister-
defect
(V.S.D.)
ciated
cardiac
In
the
group
was
the
commonest
(Table of 88 children
lesion
asso-
i).
with
known
R.
426
F. Lees
and
W7.
J.
H.
ent.
Caldicott
These
other (Table
JULY,
patients
had
anomalies
but
a wide
only
1975
spectrum
were
of
mongols
iii).
DISCUSSION
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
lished
a series
same
association.
lated
examples
recently
American Journal of Roentgenology 1975.124:423-427.
Silverman3
have
then
been
of
pub-
examples
of
the
isoand
further
reported,”6
sternal
anomalies
in
of children with known congenital disease have been published.”9 papers have given no incidence
occurrence
sternal
of abnormalities
segments
population, of
and isolated Since
reviews
groups cardiac Previous the
of
or
such
a
hospital
in
the
general
expressed
chance
of
of fusion
finding.
of
pediatric
the
significance
Our
study
of
a
of children up to the age of 14 years has shown a 7 per cent mcicidence of sternal fusion abnormalities. Furthermore, just less than i in of these was associated with congenital heart disease.
population
The
viously
potential
ability
unrecognized
incidence
general
of
pediatric
practical
predict
pre-
heart disisolated finding of and the relatively
ease as a result of an sternal fusion anomalies high
to
congenital
these
anomalies
population
in may
be
the of
importance.
Recently, White et al.9 have reviewed children with known congenital heart disease and found a 59 per cent incidence of associated premature fusion of the sternal 119
6. Premature above. ‘l’he first centers normally
fusion and fuse
FIG.
adult
of sternal segments from second sternal ossification in the late teens or early
life.
were
found
TYPES
OF
CONGENITAL WITH
congenital heart disease, 17 (19.3 per cent) had prematurely fused segments. Again, V.S.D. was the most frequent cardiac lesion noted in these 17 cases (Table ii). Multiple
TABLE
bri al
manu
in ii8
per cent). All centers except
of the
ossification
children
1,915
patients had for I, in which
centers
2
STERNAL
I
HEART
DISEASE
FUSION
Ventricular Septal Defects Patent I)uctus Arteriosus Conduction Defects
13
2
Corrected Transposition .Atrial Septal Defect Complex Anomalies
I i 2
(6.
osSification 3 were pres-
ASSOCIATED
ANOMALIES
24
out of 135 sternal abnormalities
VOL.
524,
No.
Sternal
3
Congenital
Heart
IN VARIOUS
HEART
Patent
MULTIPLE
TYPES
MANUBRIAL
Tetralogy Aortic
Stenosis
Atrial
Septal
Cushion
3 2
Mongols
2
Spina
2
Congenital
I
Celiac Disease Tracheo-Esophageal
Defect
I
Defect
17
out
59
of
ii8
with
4
Bifida
4
Heart
Pierre Robin Mucoviscidosis
i
Dextroversion
CENTERS
multiple ossification centers
Arteriosus
of Fallot
OSSIFICATION
Anomalies
Septal Defects Stenosis Ductus
III
DISEASE
Congenital Ventricular Pulmonary
427
Disease TABLE
OF OSSIFICATION
OF CONGENITAL
and
II
TABLE MULTIPLICITY
Anomalies
out
of 88 known congenital
Disease 2
Fistula
and Cleft
Palate
I I
Thalassemia
i
Muscular
I
Dystrophy
heart disease REFERENCES
American Journal of Roentgenology 1975.124:423-427.
I.
body with
segments. Our population congenital heart disease (88 patients), but only showed incidence of sternal anomalies.
of patients was also small a 17 per cent
L., and
ANDRsN, tation
or
congenital
heart
segmen-
of
Brit.
disease.
G. T. Relationship
ASHLEY,
ossification
and
sternum
CONCLUSION
ossification
sternum
in
7.,
Heart
1961,
140-142.
23, 2.
P. Diminished
HALL,
premature
in
between
definitive
7. Anat.,
man.
pattern
shape
of
o
1956,
of meso-
(Part
I),
87-105.
Many types of congenital heart disease were associated with sternal fusion anomalies but ventricular septal defect was the most common (Table i). This is in agreement with the findings of Fischer et al.#{176} in their group of acyanotic children and adolescents. Multiple manubrial ossification centers were less common (6. per cent) than suggested by Horns and O’Loughlin7 (i per cent), and Currarino and Swanson4 (20 per cent), but these latter reports related to groups of children under the age of 5 years, whereas ours ranged up to 14 years of age. Our patients with manubrial segmentation had a wide spectrum of other anomalies, but only 4 were mongols (Table III). Although duplication of the manubrial center occurs frequently in mongolism, the mcidence of mongolism among children found by chance to have multiple manubrial ossification centers is low.
3. CURRARINO,
R. F.
9.
Lees,
M.B.,
B.S.
Department
of
Adelaide
Children’s
King William North Adelaide South Australia
72
Radiology
Hospital,
Road oo6
Inc.
ture
G., and
obliteration
breast
F. N. Prema-
SILVERMAN,
ofsternal
deformity.
sutures
and
pigeon-
Radiology,
5958,
SWANSON,
G. E. Develop-
70,
532-
540. 4.
G.,
CURRARINO,
mental
and
variant
of
ossification
of
manubrium
Radiology, 5964, 82, 916. K. C., WHITE, R. I., JORDEN, C. E., J. P., and NIELL, C. A. Association of abnormalities in patients with con-
sterni in mongolism. 5. FISCHER, DORST, sternal genital RAD.
T. 0.,
GABRIELSEN, closure
of
disease.
&
sternal
AM.
J.
NUCLEAR
and
1973,
119,
MED.,
P.,
G. H. Early
congenital RAD.
89,
1963,
B. J. Multiple
centers
in
RAD.
vasculaire formation
&
5965, 93, 395-398.
GRAVIER,
retour
mongolism. THERAPY
J.,
veineux thoracique
anomal par
J.,
GAUTHIER,
R. Association d’une partielle (dextroposition
VERNEY,
heart THERAPY
975-983.
O’LOUGHLIN,
ROENTGENOL.,
NUCLEAR
avec
LADYMAN,
ossification
J.
MONNET,
and sutures
MED.,
manubrial AM.
ROENTGENOL.,
MED.,
ROENTGENOL.,
J. W., and
7. HORNS,
8.
disease.
8.
530-53
6.
J.
Ase. & NUCLEAR
heart THERAPY
and
transposition
aortique) et
ossification
d’une
malpr#{233}ma-
tur#{233}e du sternum. Pediatric, 1956, II, 95-98. R. I., JR., JORDAN, C. E., FISCHER, K. C., LAMPTON, L., NEIL, C. A., and DORST, J. P. Skeletal changes associated with adolescent congenital heart disease. AM. J. ROENTGENOL., RAD. THERAPY & NUCLEAR MED.,
WHITE,
1972,
116,
53
1-538.
No.
124,
3
STERNAL
By
R.
ANOMALIES HEART
F. LEES,
MB.,
B.S.,
and
NORTH
T
HE
association ofcongenital and premature fusion
ease segments
is well
recognized.
AND DISEASE*
CONGENITAL
J. H. CALI)ICOTT,
‘N.
ADELAIDE,
SOUTH
M.B.,
B.S.,
M.R..A.C.R.
AUSTRALIA
heart disof sternal
However,
the
incidence of sternal fusion anomalies in the general population, and the significance of a chance finding ofsuch an anomaly in relation to congenital heart disease are not
American Journal of Roentgenology 1975.124:423-427.
known
and
were
the
subject
of the
present
investigation. Of 1,915 children who had consecutive chest roentgenograms at the Adelaide Children’s Hospital for various reasons, 135 had anomalies
of
these
proved
had by-product
A
sternal of
fusion,
and
congenital the study
i
in
of
heart disease. 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. ANATOMY
The sternum develops in cartilage from mesoderm; by the ninth intra-uterine week the cartilage model has assumed its definitive shape and is continuous with the costal cartilages lateralh’, but lacks segmentation.2 The manubrium ossifies from i or occasionallv 2 centers during the fifth intrauterine month. \Vhen there are 2 centers, they
the
are
seen
lateral The
sternum
to
lie
one
develop by
represented centers, lying The uppermost
separately; either I
at the
of
the
3). to
and the others above downwards appearing during The xiphisternum
6, 5973. the Department
in
side by side (Fig. 2; and body center commences
during
Presented
other
be
month from center birth. *
the
(Fig. i). the body
each may 2 ossification
ossify
30-October From
above
roentgenogram segments for
the
a4th
fifth
Annual
or
or sixth
intra-uterine
ossify in sequence with the fourth the first year after does not ossify beScientific
of Radiology,
General
Adelaide
Meeting,
Children’s
--
Royal Hospital,
423
Australasian Inc.,
North
College Adelaide,
-
e manubrial
of Radiologists,
South
Australia.
ossification
Sydney,
center.
Australia,
September
R.
424
CEI’TRES
F. Lees
and
W.
01 OSSWICkflO4 OF STEHUM
J. H. Caldicott known
congenital
domlv
5975
JULY,
heart
selected
from
disease
the
files
was
of the
ran-
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 (side by side)
Three
sternal i.
centre appears first year of life
fuse.sin early
2.
ziplosterrial
SlerTlo
centre a.ppears afrer 3 years (may never appear)
clutdhood
fuses in adulthood
-
American Journal of Roentgenology 1975.124:423-427.
2. Centers
of
ossification
of
were
The sternal pletelv fused tion (Fig. 4)
premature
fusion
segments
at
order
of
identified: the
fusion
Premature
usual (Fig. 3.
FIG.
patterns
segments
were
time of
segments
(from
com-
of examina-
below
in
the
upward)
)
Premature
fusion
in
an
abnormal
or-
of sternum.
fore the third year and may remain cartilaginous 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 manubriosternal
joints
fuse
in
only
a
minority
of
adults. MATERIAL
The
films
of
AND
different
1,991
had consecutive various reasons
chest at the
Hospital
the
were
during re-examined
sternal children
and were
Seventy-six jected
METHOD
first to
3
months
detect
because
who
of
had they
for
1972,
anomalies
manubrial ossification. of all ages up to examinations
either
children
roentgenograms Adelaide Children’s
did
of
The 14
to not
years. be reinclude
a lateral film of the sternum, or the definition of sternal anatomy was inadequate. This resulted in 1,915 films which were examined for both anomalies of sternal fusion 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 congenital heart disease, in the case of sternal anomalies, and any other congenital anomalv in the case of multiple manubrial ossification
centers.
A separate
, FIG.
group
of
88
children
with
3. A normal single
sternum ossification
showing centers.
both
double
and
VOL.
No.
524,
der
Sternal
3
(upper
before
or
lower
middle
One
hundred
per
turely
cent) fused
sternal
mentioned records of these types
have
and
that
proved 2 others,
segments
Disease
425
fusing
6)
of the 1,915 had premaof one
of the
Review of the case showed that 24
congenital
who
Heart
FUSION
thirty-five studied
above. patients
Congenital
heart
had
not
disease
been
ex-
American Journal of Roentgenology 1975.124:423-427.
now
(Fig.
STERNAL
and children
and
segments
ones)
PREMATURE
(7
Anomalies
FIG.
5.
Premature
fusion
of sternal
ring from
below
in an infant.
ossification childhood
centers do not or puberty.
segments
The second normally
fuse
occur-
and third until
late
tensively investigated, have cardiac murmurs, and i of these had electrocardiographic and radiographic abnormalities which also suggest heart disease. The incidence of association of congenital heart disease did not vary among the 3 patterns of abnormal sternal fusion. Ventricular septal
FIG.
4.
A completely num
fused remains
sternum. unfused.
The
xiphister-
defect
(V.S.D.)
ciated
cardiac
In
the
group
was
the
commonest
(Table of 88 children
lesion
asso-
i).
with
known
R.
426
F. Lees
and
W7.
J.
H.
ent.
Caldicott
These
other (Table
JULY,
patients
had
anomalies
but
a wide
only
1975
spectrum
were
of
mongols
iii).
DISCUSSION
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
lished
a series
same
association.
lated
examples
recently
American Journal of Roentgenology 1975.124:423-427.
Silverman3
have
then
been
of
pub-
examples
of
the
isoand
further
reported,”6
sternal
anomalies
in
of children with known congenital disease have been published.”9 papers have given no incidence
occurrence
sternal
of abnormalities
segments
population, of
and isolated Since
reviews
groups cardiac Previous the
of
or
such
a
hospital
in
the
general
expressed
chance
of
of fusion
finding.
of
pediatric
the
significance
Our
study
of
a
of children up to the age of 14 years has shown a 7 per cent mcicidence of sternal fusion abnormalities. Furthermore, just less than i in of these was associated with congenital heart disease.
population
The
viously
potential
ability
unrecognized
incidence
general
of
pediatric
practical
predict
pre-
heart disisolated finding of and the relatively
ease as a result of an sternal fusion anomalies high
to
congenital
these
anomalies
population
in may
be
the of
importance.
Recently, White et al.9 have reviewed children with known congenital heart disease and found a 59 per cent incidence of associated premature fusion of the sternal 119
6. Premature above. ‘l’he first centers normally
fusion and fuse
FIG.
adult
of sternal segments from second sternal ossification in the late teens or early
life.
were
found
TYPES
OF
CONGENITAL WITH
congenital heart disease, 17 (19.3 per cent) had prematurely fused segments. Again, V.S.D. was the most frequent cardiac lesion noted in these 17 cases (Table ii). Multiple
TABLE
bri al
manu
in ii8
per cent). All centers except
of the
ossification
children
1,915
patients had for I, in which
centers
2
STERNAL
I
HEART
DISEASE
FUSION
Ventricular Septal Defects Patent I)uctus Arteriosus Conduction Defects
13
2
Corrected Transposition .Atrial Septal Defect Complex Anomalies
I i 2
(6.
osSification 3 were pres-
ASSOCIATED
ANOMALIES
24
out of 135 sternal abnormalities
VOL.
524,
No.
Sternal
3
Congenital
Heart
IN VARIOUS
HEART
Patent
MULTIPLE
TYPES
MANUBRIAL
Tetralogy Aortic
Stenosis
Atrial
Septal
Cushion
3 2
Mongols
2
Spina
2
Congenital
I
Celiac Disease Tracheo-Esophageal
Defect
I
Defect
17
out
59
of
ii8
with
4
Bifida
4
Heart
Pierre Robin Mucoviscidosis
i
Dextroversion
CENTERS
multiple ossification centers
Arteriosus
of Fallot
OSSIFICATION
Anomalies
Septal Defects Stenosis Ductus
III
DISEASE
Congenital Ventricular Pulmonary
427
Disease TABLE
OF OSSIFICATION
OF CONGENITAL
and
II
TABLE MULTIPLICITY
Anomalies
out
of 88 known congenital
Disease 2
Fistula
and Cleft
Palate
I I
Thalassemia
i
Muscular
I
Dystrophy
heart disease REFERENCES
American Journal of Roentgenology 1975.124:423-427.
I.
body with
segments. Our population congenital heart disease (88 patients), but only showed incidence of sternal anomalies.
of patients was also small a 17 per cent
L., and
ANDRsN, tation
or
congenital
heart
segmen-
of
Brit.
disease.
G. T. Relationship
ASHLEY,
ossification
and
sternum
CONCLUSION
ossification
sternum
in
7.,
Heart
1961,
140-142.
23, 2.
P. Diminished
HALL,
premature
in
between
definitive
7. Anat.,
man.
pattern
shape
of
o
1956,
of meso-
(Part
I),
87-105.
Many types of congenital heart disease were associated with sternal fusion anomalies but ventricular septal defect was the most common (Table i). This is in agreement with the findings of Fischer et al.#{176} in their group of acyanotic children and adolescents. Multiple manubrial ossification centers were less common (6. per cent) than suggested by Horns and O’Loughlin7 (i per cent), and Currarino and Swanson4 (20 per cent), but these latter reports related to groups of children under the age of 5 years, whereas ours ranged up to 14 years of age. Our patients with manubrial segmentation had a wide spectrum of other anomalies, but only 4 were mongols (Table III). Although duplication of the manubrial center occurs frequently in mongolism, the mcidence of mongolism among children found by chance to have multiple manubrial ossification centers is low.
3. CURRARINO,
R. F.
9.
Lees,
M.B.,
B.S.
Department
of
Adelaide
Children’s
King William North Adelaide South Australia
72
Radiology
Hospital,
Road oo6
Inc.
ture
G., and
obliteration
breast
F. N. Prema-
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