Paper - A rare malformation of the hand (1938)

From Embryology

Stacey RS. Cohen H. and Davies S. A rare malformation of the hand. (1938) J Anat. 72: 456-457. PMID 17104712

A Rare Malformation of the Hand

By R. S. Stacey

From the Royal College of Medicine, Baghdad


Concentra abnormalities of the hands are common and have been classified into a number of well-established groups. The case reported here is described because it has features in common with one of these groups but differs from it in certain important respects.

The patient is a somewhat undersized male Arab from Mosul, who was admitted to the Royal Hospital, Baghdad, with lobar pneumonia. His right hand is very much smaller than his left and this difference is largely, though not entirely, due to the extreme shortness of the second phalanx of each of the fingers of that hand with the exception of that of the thumb. The fingers are partly webbed, the skin extending to within half an inch of the proximal interphalangeal joint. These changes can be seen in the accompanying photographs. There is no fusion of the phalanges but flexion at the interphalangeal joints of the four fingers is limited. The patient uses his right hand for writing but for purposes where more force is required he uses his left, which is normal.


The right arm is, throughout, slightly smaller than the left, both in the lengths of the bones and in the size of the muscles. Measurements are given below. All muscular power in this limb is diminished, but there is no evidence of any lesion of the nervous system. The tendon reflexes are normal. The smallness of the arm may be due to similar factors as those responsible for the changes in the hand but are of such a degree as might result from disuse due to the relative uselessness of the hand for manual work.

The legs are equal and symmetrical and there is no malformation of the toes. The Wasserman reaction is negative and there are none of the stigmata of congenital syphilis.

The right hand is stated always to have been as it now is, and it is not possible to ascertain when the deformity was first noticed. The patient’s parents are dead but their hands are said to have been normal. He has six children, all of whom have been examined; none show any anatomical abnormality, and questioning, both of the patient and of his family, has failed to bring to light any evidence of any similar condition in any of his relatives.

Measurements Height: 155 cm. Sitting height: 86 cm. Arm (acromion to lower end of radius): right, 52 cm.; left, 54 em. Upper arm circumference: right, 18 cm.; left, 19 cm. Forearm circumference: right, 18 cm.; left, 19-5 em. Journal of Anatomy, Vol. LXXII, Part 3 Plate I


Comment. The deformity is of the type described by Drinkwater (1912) as ‘“‘minor-brachydactyly” in that the main abnormality is the extreme shortness of the middle phalanges. The shortness of stature also conforms with the usual findings in this condition. It differs from it, however, in two important respects:

(1) In this case the changes are confined to one hand, while in the other cases which have been described, so far as I am able to ascertain without the facilities of an extensive reference library, both hands and both feet have been affected.

(2) This is a sporadic case without evidence ,of any inheritance, while brachydactyly behaves as a mendelian dominant. Since the parents have not personally been examined and the progeny are limited to one generation, this point is open to question. Hasselwander, cited by Lewis (1909), has described a case in which a brachydactylous individual arose from normal stock, but this mutation was transmitted in later generations.

The interest in this case lies in the appearance of a malformation, usually hereditary, being not hereditary in an individual in whom, moreover, it is unilateral instead of bilateral.


My thanks are due to Dr A. C. Norman for the X-ray of this case.

References

Drinkwater, H. (1912). J. Genet. vol. u, p. 21.

Lewis, T. (1909). Treasures of Human Inheritance, pt. 1, pp. 14-17.


Explanation of Plate I

X-ray pictures of the hands. Notice the shortness of the second phalanx in digits ii, iii, iv and v in the X-ray on the left. The photographs illustrate the webbing.


Cite this page: Hill, M.A. (2020, July 3) Embryology Paper - A rare malformation of the hand (1938). Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Paper_-_A_rare_malformation_of_the_hand_(1938)

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