468
METACARPAL
SIGN
7. Kidder, L. A. : Congenital glycogenic tumors of
heart. Arch. Path.,
49:55,
1950.8. Nadas, A. S., and Ellison, R. C. : Cardiac tu-mors in infancy. Amer. J. Cardiol., 21:363, 1968.
9. Simopoulous, A. P., and Breslow, A. : Tuberous sclerosis in the newborn. Amer. J. Dis.
Child., 111:313, 1966.
10. Rowe, R. D., and Mehrizi, A. : The Neonate with Congenital Heart Disease, Vol. 5. Prob-lems in Clinical Pediatrics. Philadelphia:
W. B. Saunders, 1968.
11. Ehlers, K. H., Hagstrom, J. W. C., Lukas, D. S.,
Redo, S. F., and Engle, M. A. : Glycogen storage disease of the myocardium with
ob-struction to left ventricular outflow.
Circula-tion, 25:96, 1962.
12. McCue, C. M., Henningar, C. R., Davis, E., and Ray, J.: Congenital subaortic stenosis
caused by fibroma of left ventricle.
PEDIAT-BICS, 16:372, 1955.
13. Wegman, M. E., and Egbert, D. S. : Congenital rhabdomyoma of the heart associated with
arrhythmia. J. Pediat., 6:818, 1935.
14. Harvey, W. P.: Clinical aspects of cardiac
hi-mors. Amer. J. Cardiol., 21 :328, 1968. 15. Taber, R. E., and Lam, C. R. : Diagnosis and
surgical treatment of intracardiac myxoma and rhabdomyoma. J. Thorac. Cardiov. Surg., 40:337, 1960.
16. Golding, R., and Reed, C. : Rhabdomyoma of
the heart: Two unusual clinical presenta-lions. New Eng. J. Med., 276:957, 1967.
17. Steinberg, I., Miscall, L., Redo, S. F., and
Goldberg, H. P. : Angiocardiography in the diagnosis of cardiac tumors. Amer. J. Roent-gen., 91:364, 1964.
18. Ziegler, R. F.: The electrocardiogram in left
ventricular rhabdomyoma. Presented at the
Thirty-seventh Annual Meeting of the
Ameri-can Academy of Pediatrics. Chicago, Illinois, October 19, 1968.
An Evaluation
of the Metacarpal
Sign
(Short
Fourth
Metacarpal)
Albnight, et a!. described patients who had
clinical and biochemical evidences of
hypo-parathyroidism but who did not respond to
administered parathormone. These patients
were
also
of short
stature
with
a rounded
face
and
short
metacarpal
and
metatarsal
bones.
This symptom complex is known as
pseudo-hypoparathyroidism and more recently has
been characterized
by
a failure
of adenyl
cy-clase
in bone
and
kidney to respond topara-thormone.2 Short metacarpals have also been
described in patients with Turner’s syndrome3
and pseudopseudohypoparathyroidism which differs from pseudohypoparathyroidism, of
which it may be an incomplete form, by the
absence of the biochemical changes of the
latter.’
The reported normal relation of the distal
ends of the third, fourth, and fifth metacarpal
bones is such that a straight edge which is
held tangential to the distal ends of the fourth
and fifth metacarpals should pass distal to the
distal end of the third metacarpal (Fig. 1);
this relationship is hereinafter referred to as a
negative metacarpal sign (-
)
.If
the straight edge is tangential to the distal end of the thirdmetacarpal as well as the other two, the
meta-carpal sign is considered borderline
(
±).
If
the straight edge while held in the
aforemen-tioned
relation
to the
fourth
and
fifth
meta-carpal bones intersects the third metacarpal
proximal to its distal end, the metacarpal sign
is considered positive
(
+)
. Stated anotherway, a positive metacarpal sign exists
when
a
straight edge which is held tangential to the
distal ends of the third and fifth metacarpal
bones
passes
distal
to the
distal
end
of
the
fourth metacarpal bone (Fig. 2) . Since I had
previously noted the presence of a positive
sign in persons without the aforementioned
diseases, this study was undertaken to
ascer-tam the clinical prevalence of short
metacar-pals (specifically, the fourth) in a sample of
the general population, and to compare the
clinical and x-ray evaluation of short
metacar-pals.
METHOD
The hands of 1,510 consecutive patients
over
6 years
of
age
were
examined
and
a
judgment made of the relationship of the distal
ends of the third, fourth, and fifth metacarpal
bones. Consecutive subjects were selected from
a prepaid family internal medical practice as
detailed
in
a previous
study.4 Any personsreferred for an endocrine evaluation were
ex-cluded from the study. Where possible,
pos-tero-anterior x-rays of the hands were taken
when
the
clinical
findings
seemed
“abnormal.”
Some of the patients had had x-rays of the
hands taken at other times for other purposes
and these
were
studied
where
available.
The
same criteria have been applied to the clinical
appraisal of the hands and
the
x-rays
of the
i.
TABLE I
EXPERIENCE AND REASON-BRIEFLY RECORDED 469
metacarpal sign was positive, to estimate the
perpendicular distance in millimeters between
the distal end of the fourth metacarpal and the line joining the other two bones. This
distance has been used as an indication of
the degree of shortness of the fourth meta-carpal. Several members of a family may have
been examined by chance, but no systematic attempt was made to examine as many mem-bers of a family as possible, where one
mem-ben had a positive sign. The physical develop-ment of the subjects was noted as was the menstrual and fertility history where appro-priate.
RESULTS
The clinical evaluation of the metacarpal sign is listed in Table I.
A positive sign was present proportionately
at least as frequently in the parous as the
nulliparous women. Where the laterality was
recorded in those with a unilateral positive sign, it was on the right in 15 and the left in 26. The maximum degree of shortness as esti-mated clinically was 3 mm. Four subjects, in-eluding a mother and daughter, who had ab-normally short fifth metacarpal bones have
been excluded from the study. Of 61 subjects
in whom a positive metacarpal sign was
pres-ent clinically at least unilaterally, the rela-tionship on x-ray was considered normal or
borderline in only 6 subjects. Of 11 subjects
with clinically borderline signs, only one had
short metacarpals on x-rays. X-rays of the hands
were
available in only four of the pa-tients with a clinically negative metacarpal sign, and in each patient the sign was nega-tive on x-ray also. The maximum degree ofshortness of the fourth metacarpal noted on
the x-rays was 2.5 mm.
____
I/’FIG. 1. A negative metacarpal sign is illustrated
by the line, tangential to the distal ends of the
fourth and fifth metacarpal bones, passing distal
to the distal end of the third metacarpal bone.
None of the patients presented the clinical picture of gonodal agenesis,
pseudohypopara-thyroidism, or
pseudopseudohypoparathyroid-ism. One male with brachymetacarpalism
was known to be infertile; most of the married
males were fathers. Amenorrhea was not
pres-ent in any of the female subjects who had attained or passed the age of puberty. Many of the nulliparous women were unmarried. At least two females who married subsequent to
the termination of the study have become
mothers. The somatic and sexual maturation
of the adolescents were normal. Serum
cal-CLINICAL EVALUATION OF THE METACARPAL SIGN
Number of Patients
Male Female
Metacarpal Sign Exainine4
Age
(<13) Male Female
1,510 789 71
Negative 75.6% 78.1% 7.8%
Borderline 14.8% 13.6% 16.1%
Positive 9.6% 8.3% 11.1%
less than 1 mm 4.0% 4.0% 4.%
between 1 and mm 5.1% 3.7% 6.6%
more than mm 0 .5% 0 .6% 0.8%
18 104
76.7% 78.9% 74.0%
16.0% 15.6% 16.3% 7.3% 5.5% 9.7%
1.7% 1.6% .0%
5.6% 3.9% 7.7%
1
Fic. 2. The failure of the line, tangential to the
distal ends of the third and fifth metacarpal bones,
to intercept the fourth metacarpal indicates the
presence of a positive metacarpal sign.
ciun.1 determinations were available in 15 sub-jects who had a positive sign clinically. In
this group, short metacarpals were
corrobor-ated by x-ray in eight subjects, with a border-line sign being present in a ninth. In each of the 15, the serum calcium was normal. This included one mother who had the greatest shortening noted on any of the x-rays, and
another with a 2 mm shortening on x-rays.
DIscussIoN
Hortling, et al. found no abnormalities in
x-ravs of the metacarpal bones of 158 school
children, and 606 other persons with no ap-parent endocrine abnormalities who attended an orthodontic department. Archibald, et al.6
noted an abnormal or borderline metaarpal
sign in 12.4% of 2,594 persons attending a child growth study and endocrine clinic. They
noted, as did Albright, et a!.,’ that a positive
sign could be detected clinically, but apparent-ly x-rays were used in evaluating the subjects
in their study. They conjectured that a posi-tive sign would be expected to be much less
frequent in the general population. They had the impressions that patients with a positive
sign tend to show a delay in skeletal
matur-ation; that when present in only one member
of a family, brachymetacarpalism is likely to
be associated with some gonadal aberration;
and that a positive sign occurs more frequently
on the left, unrelated to handedness.
The present study canfirms the clinical de-tectability of a positive metacarpal sign.
Corn-parison of the x-ray and clinical findings in
those considered to have a positive sign
clini-cally indicates 10% false positive. Even if the
clinically-arrived-at-prevalence of 9.6% persons
with a positive sign is decreased to 8.6%, it
would exceed the figure of 5.1% positive signs
noted by Archibald, et al.6 This would make
no
allowance for possible false negatives inthe clinical study. The magnitude of the latter is not known, but at least one persoii with a
clinically borderline sign had a positive sign
on x-ray. Positive signs were somewhat more
frequent in females as was found by
Arch-bald,
with the left side involved morefre-quently than the right. My limited data do
not indicate a familial tendency toward a positive sign. A comparison of the findings in
those subjects aged 7 through 12 years with
the entire group suggests that the positive
metacarpal sign is defined, and identifiable, sometime during the first decade of life.
The maximal shortness of a fourth
rneta-carpal noted clinically was 3 mm, and on x-ray,
2.5 mm. No markedly shortened fourth
meta-carpals as illustrated by Aibright, were en-countered. The only subjects with shortened
bones of a similar degree were the mother
and daughter, with markedly foreshortened fifth metacarpals. Baker, et al. noted that the
shortening of the metacarpal, when present
in Turner’s syndrome, may vary from the gross
abnormality such as demonstrated by
Al-bright,’ to a barely detectable finding. It is not clear from the literature whether the
meta-carpals in patients with pseudohypoparathv-roidism are either normal or markedly de-formed, or whether all gradations from normal
EXPERIENCE
AND
REASON-BRIEFLY
RECORDED
471
and patients with ovarian agenesis and
pseudo-hypoparathyroidism. The present data
mdi-cate that shortening in excess of 3 mm
cmi-cally or 2.5 mm on x-ray, may be abnormal.
Such shortening may be a clue to the existence of ovarian agenesis, pseudohypoparathyroidism,
or pseudopseudohypoparathyroidism. In the absence of associated clinical findings, lesser degrees of shortening need not be considered an indication for endocrine evaluation.
SUMMARY
Short fourth metacarpal bones have been detected clinically in 9.6% of an unselected
group of 1,510 patients over the age of 6
years. Ten percent false positives were noted,
based on x-ray examination of some of the
subjects. The maximum shortening noted was
3 mm clinically and 2.5 mm on x-ray. No
endocrine abnormalities were associated with
the shortening noted in this group. STANLEY SLATER,
M.D.
Bay Ridge Medical Building
6740 Third Avenue
Brooklyn, New York 11220
T.
Lattof and V. Baldwin, technicians in theMedical Building took the x-rays used in the
study and P. Gonzalez and M. Slater, secretar-ies, assisted in the preparation of the manu-script.
REFERENCES
1. Albright, F., Forbes, A., and Henneman, P.: Pseudopseudohypoparathyroidism. Trans. Ass.
Amer. Physicians, 65:337, 1952.
2. Aurbach, C. D., Potts, J. T., Chase, L. R., and
Melson, C. L. : Polypeptide hormones and cal-cium metabolism. Ann. Intern. Med., 70:
1243, 1969.
3. Baker, D., Berdon, W., Morishma, A., and Conte, F. : Turner’s syndrome and
pseudo-Turner’s syndrome. Amer. J. Roentgen., 100:
40, 1967.
4. Slater, S. : The occurrence of thyroid nodules in
the general population. Arch. Intern. Med.,
98:175, 1956.
5. Hortling, H., Puupponen, E., and Koski, K.:
Short metacarpal or metatarsal bones: Pseu-dohypoparathyroidism. J. Clin. Endocr., 20: 466, 1960.
6. Archibald, R., Finby, N., and DeVito, F.:
Endo-crine significance of short metacarpals. J.