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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 to

para-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 third

metacarpal 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 another

way, 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 persons

referred 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

(2)

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 of

shortness 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%

(3)

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 in

the 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 more

fre-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

(4)

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 the

Medical 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.

(5)

1970;46;468

Pediatrics

Stanley Slater

An Evaluation of the Metacarpal Sign (Short Fourth Metacarpal)

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(6)

1970;46;468

Pediatrics

Stanley Slater

An Evaluation of the Metacarpal Sign (Short Fourth Metacarpal)

http://pediatrics.aappublications.org/content/46/3/468

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