The congenital deformities of the hands and feet are very numerous. They include excessive growth, a variety of other irregularities and deficiencies of nutrition, numerical increase of parts and abnormal union of digits. These defects are often hereditary, they may co-exist in various combinations, and may be associated with faults of evolution in other parts of the body. In most cases the rôle of the surgeon is limited to the removal of useless or unsightly parts, but occasionally a higher function is open to him.

The most interesting of the congenital defects of the digits is the curious and often complex condition known as makrodactyly.

MAKRODACTYLY.

The earliest examples of “congenital hypertrophy” recorded in this country were those of Dr. Reid, published in the London and Edinburgh Monthly Journal of Medical Science for March 1843, but before this date a characteristic case of makrodactyly had been carefully described by von Klein,[15] and other instances had been brought forward by Beck[16] and Wagner.[17] Reid’s paper was followed by a valuable article by Curling,[18] and contributions by Ideler,[19] Adams,[20] Devouges,[21] Chassaignac,[22] Annandale,[23] and others, but the first attempt to classify the cases was that of Busch,[24] in 1866. Since this time many additions have been made to the literature of the subject, the most important of which are those of Trelat and Monod,[25] Vogt,[26] von Fischer,[27] Wittelshöfer,[28] and Humphry.[29]

The condition may be defined as a gigantic growth, congenital in origin, of various segments of the body exclusive of the viscera. It is generally unilateral, and limited to one extremity or portion of an extremity; it tends to implicate especially the bones and joints, and the adipose and vascular elements of the subcutaneous tissue. It does not, as a rule, impair to any important extent the functional capacity of the part.

The name “congenital hypertrophy” is open to criticism. There is little doubt that the abnormality always has its origin in fœtal life, but it has seldom been noticed immediately after the delivery of a child; and, on the other hand, in one case recorded by Lannelongue,[30] a “macrodactylie elephantiasique” in a child is said to have followed an operation for the separation of two united fingers. It is, at any rate, certain that if the enlargement be always present at birth, it is seldom so disproportionate as to attract attention and never so great as to interfere with parturition. Hence the colossal proportions subsequently observed are the result of excessive growth in early infancy and childhood, and occasionally in adult life.

The hyperplasia, moreover, is rarely hypertrophic in the higher sense of the term, for, although in a few cases the increased growth has been associated with apparent increase of functional utility, in the great majority of instances it is relegated to a lower status by its unequal distribution amongst the different tissues of the parts involved, its frequent association with other congenital deformities, and especially by the defective ratio of functional capacity to the amount of constructive material. In other words, the process is wasteful and unserviceable.

The origin of the affection is very obscure. It is not hereditary, although inheritance plays a notable part in many other congenital deformities, and it cannot be attributed to any known influences arising during intra-uterine life, but, as might be expected, the mother is often able to conjure up some reminiscence of the period of gestation which to her mind affords a perfectly satisfactory clue to the mystery. Von Klein relates that in his case of makrodactyly the mother while pregnant was bitten in the hand by a goose. A history of a fright was given in Adams’s case, and in an instance reported by the author[31] a giant growth of the lower extremity was attributed to the mother having been trodden upon by a cow. Such mental impressions are probably mere coincidences, and are often greatly exaggerated in narration, but until the possibility of their connection with fœtal lesions is disproved it is advisable to place them on record.

Parts affected.—The abnormality is usually limited to one side of the body. Comparatively few bilateral cases have been observed, and in most of these the makroplasia was limited to the digits.[32]

The nævi with which the hypertrophy is often associated are nearly always confined to the affected side, but in two cases of Chassaignac and Friedberg they were found also upon the non-hypertrophied side, and in a second case of Chassaignac’s were present only upon the unenlarged parts of the body. The upper extremity is attacked nearly twice as often as the lower, and the right side much more frequently than the left. In bilateral cases the affection is seldom symmetrical; thus, on one of Friedberg’s cases the predominant enlargement of the right lower extremity was associated with a small degree of hypertrophy of the left upper limb.

The extent of the parts involved ranges from a single digit to an entire half of the body. When a whole limb is implicated the growth is always proportionately greater at the distal extremity, but colossal development of the hand or foot seldom includes the whole of the digits. The localisation of the affection does not appear to be related to any special vascular or nervous territories; thus, in makrodactyly, the regions of distribution of the digital branches of both median and ulnar nerves may be encroached upon, while parts supplied by either or both of these may escape intact.

Commonly, however, both in the hand and foot, the hypertrophy selects two or three neighbouring digits, for example, the second and third; and co-existent lipomata are almost invariably limited to that portion of the palm or sole which corresponds to the enlarged fingers or toes. In the hand the digit most commonly affected is the third, the fifth is that most exempt.

Its course is always in a certain sense progressive. Occasionally it appears to keep pace throughout with the general growth of the body, but nearly always the abnormal growth strides in advance, sooner or later, so that the disproportion of the affected part to the rest of the body becomes more and more pronounced. Its progress may be uniform and continuous, or terms of slow increase or apparent arrest may alternate with a new and rapid development.

In some instances the process does not extend beyond the primary limits, while in others it may spread centripetally, and often at a very rapid rate, as in v. Fischer’s case (l.c.), in which amputation of an hypertrophied finger was followed within a few months by hypertrophy of the whole limb. In two cases reported by Mr. F. S. Eve[33] a growth became disproportionally active at the ages of twenty-three and thirty-seven respectively. Similar instances have been published by Wittelshöfer, and the author (l.c.).

The addition of new tissue goes on, as a rule, without pain, inflammation, or interference with function; but in a case of von Fischer’s, burning pain was associated with trophic ulceration; and Friedberg’s patient,[34] who appeared to be the subject of an associated elephantiasis Arabum, had symptoms that somewhat resembled the inflammation crises of elephantiasis.

In the majority of cases the functional power of the enlarged part is not augmented, but true unilateral hypertrophy, i.e., increased tissue hypertrophy with proportionally increased functional activity, is sometimes found. The cases described long since in general terms by Geoffroy St. Hilaire,[35] as marked by an unequal development of the two sides of the body in one or more regions, were probably of this nature, and those of Ollier, Finlayson, and Langlet, may also claim a place in the same class. In Ollier’s case[36] the face, thorax, abdomen, and both extremities of one side, were uniformly larger than the corresponding parts of the opposite half of the body, the vascularity of the hypertrophied side was increased, the temperature elevated, and the limbs were more powerful. There were no nævoid growths, and it is noteworthy that in association with an absence of evident abnormality of thoracic, pelvic, and abdominal viscera, the mammary glands preserved their symmetry. The cases of Devouges, Adams, and Finlayson, also presented certain points of resemblance to true hypertrophy. In the first the whole of one side was hypertrophied, except perhaps the abdominal wall, where nothing unusual was noticed. The details are very imperfect, but according to the patient’s statement, the strength of the arm on the hypertrophied side was greatly in excess of that of its fellow. In Adams’s case the giant growth of the right lower extremity was apparently uniform, and the femoral and all other accessible arteries were considerably enlarged, but nothing is said as to muscular power. In this as in the last example nævi were present. In an unpublished case of the author’s the hypertrophied hand was much stronger than its fellow; but in the example[37] already quoted there was no evidence of increased development of muscle. In two instances of unilateral hypertrophy of the face described by Friedrich[38] and Passauer,[39] the increased growth involved the whole of the parts of the affected side, even to the teeth and tongue, and in Friedrich’s patient, and in a case brought by Dr. Heumann before a Medical Congress in Darmstadt, the hair was more developed, and the secretion of saliva was excessive on the affected side, but the muscular power and arterial supply were not specially referred to. In a case of Eve’s the facial hypertrophy extended to the brain.

In the ordinary forms of congenital hypertrophy the size of the part is augmented by an unequally distributed hyperplasia of the skeleton and soft parts. Almost all the cases narrated in detail are of this kind. In nearly all, the arterial supply is proportionate only to the normal size of the part, and the functional capacity (power, sensibility, &c.), although not seriously altered, is to some extent deteriorated. The cases may be for practical purposes divided into two groups:

1. Without deformity, the enlarged segment preserving the normal contour in other respects.

2. With deformity.

The condition of the different elements of the enlarged part is as follows:

The bones are always enlarged. Their general shape is commonly preserved, but occasionally their extremities are deformed by outgrowths at the line of junction with the articular cartilage. The hypertrophy reaches its greatest proportionate extent in the digits, while increase in size of the bones of the forearm, arm, leg, and thigh, is, as a rule, only demonstrable during life by admeasurement of length. No histological abnormality has been detected in the osseous tissue, but Eve (l.c.) found the cancellous tissue of the enlarged bone soft and the medulla fatty.

The articular surfaces may present no peculiar features, but in some cases the cartilages show irregularities of surface. The ligaments are usually greatly thickened. The synovial membrane commonly has the ordinary characters, varied only by an excessive development of the plicæ adiposæ.

The range of motion is sometimes normal; in other cases the voluntary movements are more or less diminished in extent, while a fair amount of passive mobility is preserved; and in others there is complete ankylosis.

Distortions, such as lateral deviation and hyper-extension, are frequent. The former appears to depend upon malformation of bone.

The tendons inserted into the enlarged bones have been found proportionately over-developed. The muscles, however, are seldom hypertrophied, and occasionally, as in Busch’s case, they may undergo an atrophy secondary to the impaired mobility of the joint. In a case of von Fischer’s, muscular hypertrophy was demonstrated, but no evidence of increased power was present; and, judging by the comparative disuse of the limb, it is probable that the strength was lessened. The muscular power is, as a rule, so far augmented in response to functional necessity that the enlarged part may be used without more sense of effort than that which accompanies the same actions on the normal side, as in Wagner’s case (l.c.), in which the hand weighed about twelve pounds and yet could be moved with perfect ease; there is seldom, however, any such absolute increase of strength as would indicate a genuine muscular hypertrophy.

The arteries in all the instances of false hypertrophy, with the exception of Reid’s[40] cases, were of ordinary size and apparently not enlarged in proportion to the gigantic development of the part to be nourished. This is a fact of considerable pathological significance.

The veins and capillaries, unlike the arteries, appear especially liable to change. Varicosities of the veins are very common, and in some cases great thickening of the walls may be superadded (Busch, l.c.). In Dr. Finlayson’s case the cutaneous vessels over the enlarged parts and in patches over the trunk became enlarged when the child cried, but subsided during quiescence.

In Wagner’s case the fingers of the hypertrophied hand were said to become as red as a “Blutschwamm” when the member hung down, the arteries being nevertheless of normal size.

Angiectases and angiomata are frequently seen. Capillary nævi of enormous extent sometimes co-exist (as in Adams’s case, in which the whole lower extremity and buttock were involved), and are usually, but not invariably, confined to the hypertrophied parts.

The lymphatics are liable to dilatation, and may form diffuse swellings or soft tumours of considerable extent. The glands, however, are never affected.

The nerves appear to be normal. The condition of the nerve centres has not yet been investigated except in a case of Eve’s, in which the lateral asymmetry of the head and face extended to the brain.

The integuments nearly always participate in the changes, and are necessarily augmented in extent where covering the enlarged bones. The derm does not appear to be much altered in structure, but Eve found in the skin over the hypertrophied sole a thinning of the rete with almost complete obliteration of papillæ and enormous thickening of the fibrous tissues and inter-fascicular lymph spaces; but these changes were probably due to the abnormal pressure and friction to which the part was exposed. The subcutaneous adipose layer commonly shows a diffuse thickening, and in addition may undergo a circumscribed hypertrophy in the form of large lipomatous pads, which are mostly found upon the flexor aspect of the member affected, and correspond in situation and extent to the bony enlargement (Fig. 18); but the papillary and epithelial layers do not undergo any sensible changes, nor, as a rule, are the hairs or sebaceous follicles more developed than on the opposite side. The nails of hypertrophied digits generally undergo proportionate development. The secretion of perspiration is seldom altered in amount or character, but in a case of Wittelshöfer the patient complained of profuse sweating from the affected limb. The cutaneous sensibility is variously described as unaffected or diminished, never increased. A reduction of the sensibility to heat and pain was found to accompany the tactile defect in von Fischer’s case, and it is probable, as he suggests, that careful examination would generally reveal an impairment of the faculties. Pain is rarely complained of. Burning pain was present in association with trophic ulceration in a case of von Fischer, and pains of a rheumatic character appeared in the author’s case, but this symptom probably formed no part of the original disease.

The temperature of the part is generally normal. By exception, a rise of 2°-2½° Fahr., of 2°-6° Fahr. (Reid), 2°-4° Fahr. (Finlayson), and of ½°-1° Cent. (Trelat and Monod) has been recorded.

Pathological complications are rarely met with. Trophic ulceration appeared in a case of von Fischer’s; and in Friedberg’s case an eruption resembling pemphigus was noted, but probably was not directly connected with the congenital hypertrophy. Inflammatory crises, corresponding to those of elephantiasis Arabum, have been seen in only one instance, and in this the condition appeared to be complicated by true elephantiasis.

Associated defects of development are frequent in connection with makrodactyly. They may consist of multiplication, dwarfing, absence, or fusion of parts. The most common defect is syndactyly. Secondary distortions of the spine are found, however, when the hypertrophy of a single limb is very great. The general health does not appear to be affected by the condition.

Pathology.—The older views as to the pathological origin of congenital hypertrophy leave us little advanced towards a solution. The condition has been attributed to a congenital lesion of the vaso-motor centres; to a primitive vice in the mesoblast; and to an inherent tendency of the affected tissues to appropriate an excess of nutriment; but these theories only lead to the same point—that for some reason which our pathology is still inadequate to explain, there is a weakening of the governing power that regulates the ratio between supply and demand in the tissues; or, to quote from Professor Humphry (l.c.), “The cases obviously consist in an excess, an abnormally excessive growth of a normal part of the body—an excess not depending upon any superabundance of nutritive supply, or any modification of nerve influence, but upon an excess, a want of due restraint, of that developmental force by which the several organs and structures acquire and maintain their proper dimensions and relations to one another, and by which their relative growth at different periods of life and under different circumstances (as of the genital organs at puberty). The nature and essence and habitat or source of the force is a mystery, perhaps past finding out.”

This sums up the whole question. It is probably the same kind of misgovernment of tissue that leads to certain other deformities occurring both before and after birth, and to the development of some forms of new growth. How it originates remains for the pathologists of the future to decide.

Treatment.—No means yet devised has any power of restraining the tendency for the excessive and irregular growth in this condition. The only resources of the surgeon are to correct associated deformities as far as possible, and to remove parts that are sufficiently inconvenient or disfiguring to warrant the use of the knife.

SUPERNUMERARY FINGERS AND TOES.

The occasional occurrence of supernumerary fingers and toes is well known. As a rule the extra digit is a mere pediculated appendage bearing a nail and a more or less perfect representative of the ungual phalanx; in other cases, however, it is complete and well formed, and furnished with a metacarpal or metatarsal bone of its own, or sharing the proximal bone with a neighbouring finger or toe. In rare instances the digits may undergo still further numeral increase even to a complete duplication of the normal complement.

The condition is commonly bilateral, and may affect all four extremities. It is liable to association with syndactyly and other congenital deformities, and it is occasionally handed down by inheritance as a family peculiarity. Sir William Lawrence refers to a condition of the kind which was traced through four generations, and other examples are on record.

SYNDACTYLY.

The absence of one or more of the normal clefts between the fingers or toes is a common congenital deformity, and, like most of the other inherited defects of the parts, is often transmitted by descent, and associated with other malformations.

Syndactyly presents all varieties in extent and degree. Most frequently two neighbouring digits are joined together by a web of integument involving the whole or a portion of their length, but in some instances the connecting material is much thicker, and in the most extreme cases even the bones and joints are fused, leaving nothing but a furrow to indicate the line of union. (Fig. 17, Nos. 16 to 19.)

Treatment is rarely necessary in the case of the foot, and in the hand is practicable only when the band of union is cutaneous or cellulo-cutaneous.

If the web is composed only of a thin double fold of integument it is sufficient to divide it, provided that a return of the condition is prevented from taking place through union of the raw surfaces at the root of the web. This was formerly effected in the slighter cases by a preliminary perforation of the base of the fold, and the insertion of a piece of gold wire until a cicatricial canal has been established—as in piercing the lobule for the suspension of an ear-ring—and the same end may be gained after the division by putting a small epidermic graft upon the angle. Zeller’s flap method is, however, the best. A small triangular flap is cut from the dorsum of the hand with its base opposite the heads of the metacarpal bones, its apex at the level of the first inter-phalangeal joint. This being dissected up and reversed, the whole length of the web is divided. The apex of the flap is then brought forwards between the separated digits into the proximal end of the cleft, and fixed to the cut edge of the palmar integument.

When the membrane is very thick, and the bones are drawn close together, a more complicated proceeding is required, and the ingenious method of Didot may be employed. A longitudinal incision is carried through the whole thickness of the integument along the middle of the dorsum of one of the two united fingers, a second along the middle of the palmar aspect of the other finger, and by dissection each digit is made to furnish a rectangular flap to cover in the raw surface of its neighbour. The division of the tissue left after raising the flaps must be made with great care, in order to avoid injury to the digital nerves.

ECTRODACTYLY.

Simple congenital ectrodactyly, like the loss of a larger segment of an extremity, may arise either by intra-uterine traumatism or defective development. Amputation by an amniotic band or a coil of umbilical cord probably explains the majority of the cases, as well as the congenital constrictions sometimes found in the limbs or digits of the newborn child, but the occasional appearance of fingers or toes at the end of a congenital stump can only be accounted for on the hypothesis of a temporary suspension of development in the proximal portion of the stump, and we may assume the possibility of a like origin for the absence of the most distal portions of a member. In some instances an ectrodactyly is complicated with other developmental errors, and may be transmitted through several generations. Attention has been especially drawn to cases of this class in the last few years. Two were recorded in detail in 1886 by Dr. Fotherby[41] and the author,[42] and a third was added five years ago by Messrs. Parker and Robinson.[43] In all of these the defect was traced through three or four generations, selecting in an apparently indiscriminate way a large portion of the members of the family, whilst leaving others exempt. In some individuals all four limbs were attacked, in others the upper or lower only, but usually with a more or less perfect bilateral symmetry. In most cases the ectrodactyly was associated with deformities of the remaining digits, such as syndactyly, hypertrophy, and joint distortions; and where, as frequently happened, the middle digits were imperfectly developed and the others hypertrophied and distorted, the member assumed the appearance of the pincers of a lobster. (Fig. 17, Nos. 11 to 14.)

In Dr. Fotherby’s and Messrs. Parker and Robinson’s cases the defects were traced through three generations, affecting sixteen out of thirty-seven descendants in the one and sixteen out of thirty-three in the other; in the author’s case the history extended to four generations, attacking twenty-four out of thirty-six children, and the divergence from the normal state tended to increase with the later scions.

As a rule, little or no treatment is called for in this condition, because habit has given the member a good deal of functional utility that surgical interference might injure or destroy; but sometimes, and more particularly in the feet, it is permissible to operate for the purpose of lessening the deformity, as in Messrs. Parker and Robinson’s case, where a plastic operation was performed with much benefit to the patient.

BRACHYDACTYLY.

Undue shortness of the fingers or toes may occur either as a congenital or as an acquired defect. The congenital form, due to imperfect formation of any or all of the bony elements of a digit (including the metacarpal bone), is insusceptible of treatment except by amputation, should that step be justified by the inconvenience or deformity attached to the defective member.

Acquired brachydactyly may arise in several ways. Occasionally it is to be traced to an arrest of development either due to an injury during childhood or adolescence, or without apparent explanation, but more frequently it is a result of disease. In younger subjects the most common cause is the destruction of bone in tuberculous disease, which may practically eliminate a phalanx, leaving the rest healthy. In older people it may be met with in cases of perforating ulcer, the proximal phalanx of the great toe and sometimes other bones undergoing a slow and painless disintegration, which is manifested chiefly by a progressive shortening of the digit with few or none of the more obvious signs of caries.

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