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The treatment of radial club hand
The Treatment o/ Radial Club Hand--Douglas W. Lamb
THE TREATMENT
OF R A D I A L
CLUB HAND
Absent Radius, Aplasia of the Radius, Hypoplasia of the Radius, Radial Paraxial Hemimelia DOUGLAS W. LAMB, Edinburgh In recent years, following the great increase in congenital deformities of the upper limb attributed to Thalidomide (D,istaval) the incidence of radial club hand deformity due to complete or partial absence of the radius has been by no means unusual. Whereas in the past it was rare for any one surgeon to have seen and dealt with more than one or two such cases, this has now become a very important condition, and the writer has had over forty cases under his personal supervision. The radius may be completely absent (aDlasia) or may be present in part (hypo01asia). In those cases where the radius is deficient it is usually the upper end which is present and the bone shows little tendency to grow at the normal rate, The child who is born with aplasia or hypoplasia of the radius will usually have displacement of the wrist and hand to the radial side of the ulna with a characteristic deformity (Fig. 1). In most cases this deformity is correctable and the hand can be straightened on the end of the ulna by manipulation. Occasionally, however, even at birth, the soft tissues are very tight and contracted on the radial side of the forearm and wrist. A strong fibrous band representing the absent radius has been described which leads to this contracture and prevents correction. As growth takes place there is a tendency for increasing deformity to occur and the carpus is displaced to the radial side of the ulna and also in a volar direction so that the ulnar head becomes more prominent. If this deformity is allowed to persist as growth progresses and even if the deformity was readily correctable at birth, it will gradually become fixed in the deformed position with progressive tightening of all soft tissues on the radial side. Splinting is an essential part of early treatment, to prevent this deformity becoming fixed. ANATOMY OF THE CONDITION
It is necessary to understand the anatomical changes which are present and the natural development of the condition as growth continues. The radius itself is either absent or hypoplastic and the development of the radial or pre-axial border of the forearm and hand is usually also affected. The muscles which arise from the radius are either completely absent or atrophic and fibrous. The radial nerve is commonly absent and its area of skin sensation taken over by the median nerve, and the radial artery is often absent. It is very unusual for the radial side of the hand to be normal but this does occasionally happen. In most cases, however, the thumb is either absent or is very deficient in its structure and the scaphoid and trapezium bones are nearly always absent. If the thumb is present it is usually very small and hypoplastic and attached by a thin skin pedicle more proximal than is usual. The bony structure is deficient and there is seldom any useful muscle control. The term "floating thumb" (pouce flottant) has been applied to this type of thumb (Fig. 2) and it is seldom of any functional value and rarely grows. The structure and function of the digits has to be considered very carefully in all cases of absent radius. The ulnar two digits are near!y always normal in appearance, structure and function. In the author's experlence there is some impairment of joint structure and function in the radial two digits in all cases. The degree of involvement varies widely from almost complete stiffness and uselessness of these digits to relatively normal function. 22
Vol. 4
No. 1
1972
The Treatment of Radial! Club H a n d - - D o u g l a s W. Lamb
Fig. 1. Typical radial club hand deformity. Note flexion contracture deformity of radial two digits.
Fig. 2, Bilateral absence of radius. Note typical "pouce flottant', The metacarpo-phalangeal joints usually tie in extension and have a very restricted range of flexion. The proximal interphalangeal joint commonly has a flexion contracture with limitation of extension by twenty to thirty degrees (see Fig. 1) and seldom with more than a few degrees of active and passive movement. Radiographs of these joints commonly look normal but dissections have shown that the joint space is more apparent than real with the capsule adherent. Sometimes there are abnormal insertions of the superficial flexor tendon. This impairment in function of the radial two digits is of very great importance. We have found that many children prefer to use the ulnar two digits f o r attempts at prehension (Fig. 3). This may be because the wrist is lying in an abnormal position but we have found that even when the wrist is splinted straight that the same two fingers are often used in preference to the radial two digits. In VoI. 4
No. 1
1972
23
The Treatment of Radial Club Hand--Douglas W. Lamb
the same way, if the question of pollicisation of an index finger should arise, this impaired function in the index finger may prevent it having any useful function in the transposed position. A striking feature of absent radius in a large number of patients has been the stiffness of the elbow. Many children during the first year or two of life have had elbows lying in the extended position (see Fig. 3) with virtually no active or passive elbow movement. Fortunately, in many cases as the child gets older the elbow has begun to loosen and about ninety degrees of active and passive flexion has been obtained. The cause of this abnormality is unknown and radiographs in most cases show apparently normal development of the elbow. In those cases where electromyographic studies have been carried out the activity of the elbow flexors and extensors appears adequate. Despite this the development of full elbow movement is most unusual and in many cases the degree of flexion is less than ninety degrees. This is of obvious importance in deciding on operative correction of the radial club hand deformity, for a child with stiff elbows is only able to get the hand to the face for feeding and toilet if the wrist is deviated.
Fig. 3. Bilateral radial club hand. Note stiff extended elbow position. There is no active or passive elbow movement. Prehension is between ulnar two digits. The natural development of the forearm is of obvious importance. We are indebted to Heikel (1959) for his careful and painstaking study of forty-seven cases. He found in those cases which he followed to maturity and which had not been treated that the ulna was invariably shorter than normal. The lower ulnar epiphysis normally develops at the eighth year and closes at nineteen to twenty-one years and he has found that the epiphysis develops later and closes earlier than normal. This is of very great importance as any operations which are being contemplated m a y cause premature epiphyseal closure and if it is known that this is going to happen anyway and the ulna is going to be shorter than normal then the danger of producing this complication becomes less important. Quite apart from the diminished growth in length of the ulna there is a tendency for it to develop a curvature concave to the radius. In most descriptions of the established radial club hand deformity this bowing of the ulna is accepted as part of the deformity. The writer believes that ir~ most cases this curvature is a secondary one which develops during gr0wth ~/nd is due to the soft tissue tightness on the radial side. He has found in the cases under his supervision that if the soft tissue contracture is prevented by regular splinting then in most cases the ulna will grow straight. In two cases under supervision, however, the ulna has 24
Vol. 4
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The Treatment of Radial Club Hand--Douglas W. Lamb
been bowed at birth and in these cases it is usually found that there is a tight fibrous band replacing the radius and it is thought that this is responsible for the deformity. If this tight hand is stretched by positive splinting or released by operation then the ulna can be coaxed to grow straight. A S S O C I A T E D DEFECTS
Aplasia or hypoplasia of the radius may be unilateral or bilateral and is often apparently an isolated congenital deficiency. No known cause for its development other than that attributed to Thalidomide bas been discovered, but it is assumed that there is interference with the normal development of the upper limb bud towards the end of its differentiation, i.e. about the fifth or sixth week of embryonic development. On certain occasions, however, other congenital abnormalities have been described in association, e.g. cardiac anomalies, Fanconi syndrome and Thrombocytopenia, while the most common associated skeletal defect has been a dorsal scoliosis. FUNCTION A study of function in this condition is most interesting and important. In the unilateral case the function of the normal arm is such that the deformed and shortened limb is simply used as an aid. In these circumstances the child is usuatl~ perfectly capable of carrying out full self-care activities. In the case of the child with bilateral deformity, however, the function is often much less efficient than would be expected and we have found that many of these children are unable to perform the daily toilet and dressing functions which are necessary for an independent existence. The children tend to use the ulnar two fingers for gripping (Fig. 3) but obviously lack the strength of grip which is provided by normal prehension with a normal thumb. Where the function of the radial two digits is good enough these may also be used for gripping and it is striking how by the age of three or four years there may be an attempt by the index finger to swing round into a position of opposition. This is never, however, sufficient really to get proper opposition effect. The function of the digits does not seem to be affected by the abnormal club hand position. We have tested the function of a group of children both with the deformity uncorrected and then with the hand splinted straight and there is no apparent difference in the actual function of the digits themselves. It must be appreciated that the child with an absent radius has no pronation and supination of the forearm and this function is compensated to a remarkable degree by the radial deviation movement. Any operations which will stiffen the wrist in the straight position will immediately deprive the child of this movement. The importance of retaining this wrist movement when the elbow is stiff has already been stressed. TREATMENT
Treatment of this condition is extremely difficult. Allowance must be made for the difference between the unilateral and bilateral case. When there is one normal upper limb the function of the affected side becomes less important and operations which may improve the appearance, but not necessarily the function, are permissible. Similarly, in a bilateral case, the side which has less function can often be considered suitable for operation as long as it is realised that the appearance may be better but the function not necessarily so. Vol. 4
No. 1
1972
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The Treatme'nt of Radial Club Hand--Douglas W. Lamb
It is the author's opinion that there is little to be gained by early attempts at operation, provided the deformity is not allowed to become fixed, Soft tissue contracture in the deformed position is prevented by careful splinting. This can be done initially by small plaster, Glassona or plastic splints. When the child is a little older a ratchet type splint which has a positive correcting force has proved of great value (Fig. 4). These splints are simply worn at night and if the hand is left free during the day full function can be encouraged. If contracture of the soft tissues resists stretching by splintage this should be corrected by open operation. Sometimes there is a fibrous band replacing the absent radius which requires excision. Usually division of the soft tissues over the radial side of the carpus will allow correction. Not infrequently the skin has, however, become very tightened and requires a plastic procedure, such as a Z-plastic before it can be corrected. Once the deformity is correctable then splinting is continued. In most cases where the ulna is straight at birth and regular splinting has been used there has been no tendency for curvature of the ulna to develop. Where this has already developed, however, corrective osteotomy should be carried out so that the forearm can be splinted straight and encouraged to grow straight.
Fig, 4. Radial club hand splint. There is a ratchet which allows positive correction of deformity (a) Drawing from photograph of deformity before correction. (b) Drawing from photograph of same child after correction of fixed deformity by gradual distraction. If the elbow is stiff, this is an absolute contra-indication to any surgical operation to straighten or stiffen the wrist. As the child grows and the ulna increases in length it becomes more difficult to prevent further displacement of the carpus in a radial and volar direction. The parents are naturally anxious that something should be done to correct the deformity. However, it must be explained to them that while it is possible to straighten the wrist this often implies reduction in the mobility of the wrist with loss of function. In addition, operations may interfere with any growth potential at the lower ulnar epiphysis. For these reasons it is wise to delay any operative procedure as long as the deformity can be controlled by splinting. Various operations designed to control the displacement of the carpus without limitation of function and without interfering with growth have been carried out in the past but none of these are completely reliable. Start (1945) described some cases where he had attempted to replace the absent radius by transposition of the upper end of a fibula into the forearm. This operation was developed by Riordan (1959) who advocated that it should be done at an early age before the child was a year old. He described six cases and the operation seemed promising. The fibula was placed against the ulna with the upper fibular epiphysis at the wrist. The theory was that the epiphysis might con26
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The Treatment of Radial Club H a n d - - D o u g l a s W. Lamb
tinue to grow and encourage growth of the carpus and hand in a normal position. Unfortunately these hopes have not been fulfilled and it is very seldom that any growth occurs in the transplanted epiphysis. An operation which holds out considerable promise in this condition, and seems to be the most successful procedure in a small child, is to centralise the carpus over the end of the ulna. If there is a soft tissue contracture the operation should be done through two separate incisions, one on the radial side to release the contracture and the other on the ulnar side to free the lower end of the ulna and facilitate the placing of the carpus over its end, Great care is necessary with the incision on the radial side as the median nerve is usually found lying closely subjacent to the skin and should always be looked for and retracted carefully aside before freeing the soft tissues. If no soft tissue contracture requires correction then the lower forearm and carpus can be readily exposed through an S-curved incision. It is usually found impossible to hold the carpus satisfactorily over the end of the ulna without resection of a portion of the centre of the carpus and this usually requires removal of the lunate and part or all of the capitate. The lower end of the ulna is then placed in the gap provided (Fig. 5) and care is taken not
Fig. 5. Tracings from radiographs of a child with radial aplasia. (a) Before operation. (b) After resection of central carpus and insertion of lower ulna into notch created. Position maintained by intra-medullary Kirschner wire. to interfere with the lower ulnar epiphysis and to free its soft tissue attachments as little as possible. The position m a y be held by a Kirschner wire passed across •die carpus and along the third or fourth metacarpal shaft. It is then passed out through the head of the metacarpal and re-introduced in a retrograde manner through the centre of the lower ulnar epiphysis and up the ulnar medullary cavity (Fig. 5). This can be a difficult procedure and should be done under X-ray control. This operation has b e e n very successful in controlling the wrist deformity in fourteen cases and if the Kirschner wire is passed across the centre of the epiphysis there seems little danger of interfering with its growth so that the ulna can develop satisfactorily. The Kirschner wire should be left in as long as possible and cases have been described where it has been left in for several years (Swanson, 1965) but there is a tendency for it to back out within a few months. Following removal of the wire the wrist remains stable with some slight movement, particularly of flexion and extension. Because of the technical difficulty in getting the Kirschner wire in satisfactory position and also because of its tendency to slip out after a few weeks or months, an attempt has been made to stabilise the hand :'vet the ulna without the necessity of pin fixation. In the last four cases the soft tissue over the carpus and wrist has been carefully dissected off bone and retained as a strong flap which could be repaired over the lower end of the ulna and carpus after insertion of the ulna into the notch cut in the carpus. This has proved very satisfactory and has remained as stable as those cases where a wire has been used. Vol. 4
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The Treatment of Radial Club Hand--Douglas W. Lamb
Provided the soft tissues on the radial side have not been allowed to contract the hand can usually be placed over the ulna without difficulty following resection of an adequate portion of carpus. In some cases, however, the fingers have flexed strongly following this and this has been found to be due to superficial flexor tightness. While this tightness can be overcome sufficiently in some cases by flexion of the elbow to a right angle in others the tightness remains and it is necessary at operation to lengthen or to resect a portion of the superficial tendon. Care must be taken to avoid excessive stretching of the median nerve. Flexion of the elbow usually relieves any undue tightness. The abnormal situation of the median nerve has already been mentioned and in two cases in this series the median nerve divided into two large equal divisions about two inches above the wrist. One division ran through the carpal tunnel, the other deviated radially to the situation normally occupied by the radial nerve. Post operative swelling has been invariable and severe in this series. While it may be limited in extent by careful preservation of dorsal veins and by careful haemostasis after release of the tourniquet the lack of good active mobility of the hand due to the structure of the finger joints and
Fig. 6. Bilateral radial club hand. (a) Before operation. (b) Two years after centralisation of carpus on right side.
Fig. 7. A typical case of radial club hand after operative correction by centralisation of carpus on ulna. (a) Showing digital extension and flexion from volar aspect. (b) Showing digital extension and flexion from the ulnar aspect. 28
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T h e Treatment o/ Radial Club H a n d - - D o u g l a s W. L a m b
poor flexor muscles makes swelling inevitable. For this reason no post operative plaster is applied. A firm crepe compression bandage is applied and the hand elevated. The swelling is usually settled within a week of operation when a long plaster cast from metacarpal head to upper arm is applied with the elbow at ninety degrees. This plaster should be maintained for at least six weeks following which a plastic splint to hold the wrist in the corrected position is used for three months. The appearance of the hand in this corrected position is much improved (Fig. 6 and 7) and the loss of some of the wrist mobility has not in our cases resulted in any impairment of function. There is no doubt that function can be further improved in suitable cases by transfer of the index finger on a neurovascular bundle as described by Littler and using the techniques of Riordan and Buck-Gramcko. This has been carried out on seventeen occasions in this series. Surgery has been undertaken in this group of children only after very considerable thought and discussion with other surgeons who have had experience in this field. It was thought that the cosmetic appearance would be definitely enhanced but there was considerable division of opinion regarding the effect on function. In the light of experience and following careful pre- and post-operative assessment of function by a skilled occupational therapist it became evident that function was not impaired. Once the wrist had been stabilised the advantages of providing a thumb for prehension became apparent and in most cases following pollicisation there has been a significant increase in function. It may take several months for the child to become orientated to using the radial side of the hand in preference to the ulnar digits which have predominated previously. In the light of this experience and in an attempt to provide an early pattern of radial prehension there would appear to be good indications for early operation (within the first year of life) to stabilise the carpus by centralisation and pollicisation. SUMMARY
1. A series of forty-one children with radial club hand deformity is described. In twenty-two this was bilateral. 2. The importance of early splintage to prevent soft tissue contracture is stressed. A simple ratchet type of splint, which has proved effective, is described. Splintage is only required at night. 3. Operation by centralisation of the carpus over the ulna has proved satisfactory in correcting the deformity and producing wrist stability. A limited range of wrist movement is still possible and so far there does not appear to have been any detrimental effect on the growth of the lower ulnar epiphysis. A satisfactory correction can be maintained by a soft tissue periosteal flap. 4. While the expected improvement in appearance has been achieved the loss of function which had been anticipated has not materialised. 5. Surgical correction of the wrist deformity should not be contemplated unless there is active flexion of the elbow to about ninety degrees. In many cases where the elbow is stiff in extension at birth there is a tendency to gradual loosening of the elbow during the first year or two. Where this does not occur a posterior release of the elbow capsule (as carried out in arthrogryphosis) can be successful. Vol. 4
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The Treatment o / R a d i a l Club Hand--Douglas W. Lamb
. Pollicisation of the i n d e x finger should be seriously considered. The result will depend to a considerable extent on the structure a n d f u n c t i o n of the index finger. 7. I n order to get the best possible f u n c t i o n a l result early operation should be considered. ACKNOWLEDGEMENTS
I wish to thank all my colleagues who have referred patients. I am particularly indebted to the surgeons in many countries who have generously given advice from their experience of the management of this difficult condition. The help given by Miss Aline Macnaughtan, Head Occupational Therapist, with her careful assessment of the functional capabilities of these children has been invaluable. I am indebted to Miss Anne Sim for her willing secretarial assistance. Figures 4 and 5 are reproduced by kind permission of Butterworth & Co. (Publishers) Ltd., from Operative Surgery Second Edition, Vol. 11, The Hand.
REFERENCES
BARSKY, A. J. (1958) Congenital Anomalies of the Hand and their Surgical Treatment. Springfield, Illinois. Charles C. Thomas. BROWN, J. J. M. (1962) Surgery of Childhood. London. Edward Arnold. BUCK-GRAMCKO, D. Personal Communication. BUNNELL, S. (1964) Surgery of the Hand. Editor J. Boyes. 4th Edition, Philadelphia, J. B. Lippincott Company. HEIKEL, H. V. A. (1959) Aplasia and Hypoplasia of the Radius. Acta Orthopaedica Scandinavica, Supplement 39. JAMES, J. I. P. and LAMB, D. W. (1963) Congenital Abnormalities of the Limbs. The Practitioner, 191, 159. KATO, K. (1924) Congenital Absence of Radius. Journal of Bone and Joint Surgery, 6: 589. LAMB, D. W. (1970) Club Hand: Absent Radius. Operative Surgery, 2nd Edition. Editors C. Rob and R. Smith. London. Butterworths. ¥ol. 11, 12-16. LIDGE, Ralph. Quoted by A. B. Swanson. LITTLER, J. W. (1953) The Neurovascular Pedicle Method of Digital Transposition for Reconstruction of the Thumb. Plastic and Reconstructive Surgery. 12, 303. LLOYD-ROBERTS, G. C. (1963) Orthopaedic Abnormalities in "Congenital Abnormalities in Infancy". Edited by A. P. Norman, Oxford. Blackwell Scientific Publication. MERCER, W. and DUTHIE, R. B. (1964) Orthopaedic Surgery. 6th Edition. London. Edward Arnold. PULVERTAFT, R. G. and REID, D. A. C. (1963) Surgery of the Hand in Great Britain. British Journal of Surgery. 50: 673. RIORDAN, D. C. (1955) Congenital Absence of Radius. Journal of Bone and Joint Surgery. 37A, 1129. RIORDAN, D. C. (1966) Inter-clinic Information Bulletin. Vol. 4, No. 6. STARR, D. E. (1945)Congenital Absence of Radius. A Method of Surgical Correction. Journal of Bone and Joint Surgery. 27, 572. SWANSON, A. B. (1965) The Treatment of Congenital Limb Malformations. Kobe Journal Medical Sciences. Vol. 11. Supplement p. 41. 30
VoL 4
No. 1
1972
THE TREATMENT
OF R A D I A L
CLUB HAND
Absent Radius, Aplasia of the Radius, Hypoplasia of the Radius, Radial Paraxial Hemimelia DOUGLAS W. LAMB, Edinburgh In recent years, following the great increase in congenital deformities of the upper limb attributed to Thalidomide (D,istaval) the incidence of radial club hand deformity due to complete or partial absence of the radius has been by no means unusual. Whereas in the past it was rare for any one surgeon to have seen and dealt with more than one or two such cases, this has now become a very important condition, and the writer has had over forty cases under his personal supervision. The radius may be completely absent (aDlasia) or may be present in part (hypo01asia). In those cases where the radius is deficient it is usually the upper end which is present and the bone shows little tendency to grow at the normal rate, The child who is born with aplasia or hypoplasia of the radius will usually have displacement of the wrist and hand to the radial side of the ulna with a characteristic deformity (Fig. 1). In most cases this deformity is correctable and the hand can be straightened on the end of the ulna by manipulation. Occasionally, however, even at birth, the soft tissues are very tight and contracted on the radial side of the forearm and wrist. A strong fibrous band representing the absent radius has been described which leads to this contracture and prevents correction. As growth takes place there is a tendency for increasing deformity to occur and the carpus is displaced to the radial side of the ulna and also in a volar direction so that the ulnar head becomes more prominent. If this deformity is allowed to persist as growth progresses and even if the deformity was readily correctable at birth, it will gradually become fixed in the deformed position with progressive tightening of all soft tissues on the radial side. Splinting is an essential part of early treatment, to prevent this deformity becoming fixed. ANATOMY OF THE CONDITION
It is necessary to understand the anatomical changes which are present and the natural development of the condition as growth continues. The radius itself is either absent or hypoplastic and the development of the radial or pre-axial border of the forearm and hand is usually also affected. The muscles which arise from the radius are either completely absent or atrophic and fibrous. The radial nerve is commonly absent and its area of skin sensation taken over by the median nerve, and the radial artery is often absent. It is very unusual for the radial side of the hand to be normal but this does occasionally happen. In most cases, however, the thumb is either absent or is very deficient in its structure and the scaphoid and trapezium bones are nearly always absent. If the thumb is present it is usually very small and hypoplastic and attached by a thin skin pedicle more proximal than is usual. The bony structure is deficient and there is seldom any useful muscle control. The term "floating thumb" (pouce flottant) has been applied to this type of thumb (Fig. 2) and it is seldom of any functional value and rarely grows. The structure and function of the digits has to be considered very carefully in all cases of absent radius. The ulnar two digits are near!y always normal in appearance, structure and function. In the author's experlence there is some impairment of joint structure and function in the radial two digits in all cases. The degree of involvement varies widely from almost complete stiffness and uselessness of these digits to relatively normal function. 22
Vol. 4
No. 1
1972
The Treatment of Radial! Club H a n d - - D o u g l a s W. Lamb
Fig. 1. Typical radial club hand deformity. Note flexion contracture deformity of radial two digits.
Fig. 2, Bilateral absence of radius. Note typical "pouce flottant', The metacarpo-phalangeal joints usually tie in extension and have a very restricted range of flexion. The proximal interphalangeal joint commonly has a flexion contracture with limitation of extension by twenty to thirty degrees (see Fig. 1) and seldom with more than a few degrees of active and passive movement. Radiographs of these joints commonly look normal but dissections have shown that the joint space is more apparent than real with the capsule adherent. Sometimes there are abnormal insertions of the superficial flexor tendon. This impairment in function of the radial two digits is of very great importance. We have found that many children prefer to use the ulnar two digits f o r attempts at prehension (Fig. 3). This may be because the wrist is lying in an abnormal position but we have found that even when the wrist is splinted straight that the same two fingers are often used in preference to the radial two digits. In VoI. 4
No. 1
1972
23
The Treatment of Radial Club Hand--Douglas W. Lamb
the same way, if the question of pollicisation of an index finger should arise, this impaired function in the index finger may prevent it having any useful function in the transposed position. A striking feature of absent radius in a large number of patients has been the stiffness of the elbow. Many children during the first year or two of life have had elbows lying in the extended position (see Fig. 3) with virtually no active or passive elbow movement. Fortunately, in many cases as the child gets older the elbow has begun to loosen and about ninety degrees of active and passive flexion has been obtained. The cause of this abnormality is unknown and radiographs in most cases show apparently normal development of the elbow. In those cases where electromyographic studies have been carried out the activity of the elbow flexors and extensors appears adequate. Despite this the development of full elbow movement is most unusual and in many cases the degree of flexion is less than ninety degrees. This is of obvious importance in deciding on operative correction of the radial club hand deformity, for a child with stiff elbows is only able to get the hand to the face for feeding and toilet if the wrist is deviated.
Fig. 3. Bilateral radial club hand. Note stiff extended elbow position. There is no active or passive elbow movement. Prehension is between ulnar two digits. The natural development of the forearm is of obvious importance. We are indebted to Heikel (1959) for his careful and painstaking study of forty-seven cases. He found in those cases which he followed to maturity and which had not been treated that the ulna was invariably shorter than normal. The lower ulnar epiphysis normally develops at the eighth year and closes at nineteen to twenty-one years and he has found that the epiphysis develops later and closes earlier than normal. This is of very great importance as any operations which are being contemplated m a y cause premature epiphyseal closure and if it is known that this is going to happen anyway and the ulna is going to be shorter than normal then the danger of producing this complication becomes less important. Quite apart from the diminished growth in length of the ulna there is a tendency for it to develop a curvature concave to the radius. In most descriptions of the established radial club hand deformity this bowing of the ulna is accepted as part of the deformity. The writer believes that ir~ most cases this curvature is a secondary one which develops during gr0wth ~/nd is due to the soft tissue tightness on the radial side. He has found in the cases under his supervision that if the soft tissue contracture is prevented by regular splinting then in most cases the ulna will grow straight. In two cases under supervision, however, the ulna has 24
Vol. 4
No. 1
1972
The Treatment of Radial Club Hand--Douglas W. Lamb
been bowed at birth and in these cases it is usually found that there is a tight fibrous band replacing the radius and it is thought that this is responsible for the deformity. If this tight hand is stretched by positive splinting or released by operation then the ulna can be coaxed to grow straight. A S S O C I A T E D DEFECTS
Aplasia or hypoplasia of the radius may be unilateral or bilateral and is often apparently an isolated congenital deficiency. No known cause for its development other than that attributed to Thalidomide bas been discovered, but it is assumed that there is interference with the normal development of the upper limb bud towards the end of its differentiation, i.e. about the fifth or sixth week of embryonic development. On certain occasions, however, other congenital abnormalities have been described in association, e.g. cardiac anomalies, Fanconi syndrome and Thrombocytopenia, while the most common associated skeletal defect has been a dorsal scoliosis. FUNCTION A study of function in this condition is most interesting and important. In the unilateral case the function of the normal arm is such that the deformed and shortened limb is simply used as an aid. In these circumstances the child is usuatl~ perfectly capable of carrying out full self-care activities. In the case of the child with bilateral deformity, however, the function is often much less efficient than would be expected and we have found that many of these children are unable to perform the daily toilet and dressing functions which are necessary for an independent existence. The children tend to use the ulnar two fingers for gripping (Fig. 3) but obviously lack the strength of grip which is provided by normal prehension with a normal thumb. Where the function of the radial two digits is good enough these may also be used for gripping and it is striking how by the age of three or four years there may be an attempt by the index finger to swing round into a position of opposition. This is never, however, sufficient really to get proper opposition effect. The function of the digits does not seem to be affected by the abnormal club hand position. We have tested the function of a group of children both with the deformity uncorrected and then with the hand splinted straight and there is no apparent difference in the actual function of the digits themselves. It must be appreciated that the child with an absent radius has no pronation and supination of the forearm and this function is compensated to a remarkable degree by the radial deviation movement. Any operations which will stiffen the wrist in the straight position will immediately deprive the child of this movement. The importance of retaining this wrist movement when the elbow is stiff has already been stressed. TREATMENT
Treatment of this condition is extremely difficult. Allowance must be made for the difference between the unilateral and bilateral case. When there is one normal upper limb the function of the affected side becomes less important and operations which may improve the appearance, but not necessarily the function, are permissible. Similarly, in a bilateral case, the side which has less function can often be considered suitable for operation as long as it is realised that the appearance may be better but the function not necessarily so. Vol. 4
No. 1
1972
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The Treatme'nt of Radial Club Hand--Douglas W. Lamb
It is the author's opinion that there is little to be gained by early attempts at operation, provided the deformity is not allowed to become fixed, Soft tissue contracture in the deformed position is prevented by careful splinting. This can be done initially by small plaster, Glassona or plastic splints. When the child is a little older a ratchet type splint which has a positive correcting force has proved of great value (Fig. 4). These splints are simply worn at night and if the hand is left free during the day full function can be encouraged. If contracture of the soft tissues resists stretching by splintage this should be corrected by open operation. Sometimes there is a fibrous band replacing the absent radius which requires excision. Usually division of the soft tissues over the radial side of the carpus will allow correction. Not infrequently the skin has, however, become very tightened and requires a plastic procedure, such as a Z-plastic before it can be corrected. Once the deformity is correctable then splinting is continued. In most cases where the ulna is straight at birth and regular splinting has been used there has been no tendency for curvature of the ulna to develop. Where this has already developed, however, corrective osteotomy should be carried out so that the forearm can be splinted straight and encouraged to grow straight.
Fig, 4. Radial club hand splint. There is a ratchet which allows positive correction of deformity (a) Drawing from photograph of deformity before correction. (b) Drawing from photograph of same child after correction of fixed deformity by gradual distraction. If the elbow is stiff, this is an absolute contra-indication to any surgical operation to straighten or stiffen the wrist. As the child grows and the ulna increases in length it becomes more difficult to prevent further displacement of the carpus in a radial and volar direction. The parents are naturally anxious that something should be done to correct the deformity. However, it must be explained to them that while it is possible to straighten the wrist this often implies reduction in the mobility of the wrist with loss of function. In addition, operations may interfere with any growth potential at the lower ulnar epiphysis. For these reasons it is wise to delay any operative procedure as long as the deformity can be controlled by splinting. Various operations designed to control the displacement of the carpus without limitation of function and without interfering with growth have been carried out in the past but none of these are completely reliable. Start (1945) described some cases where he had attempted to replace the absent radius by transposition of the upper end of a fibula into the forearm. This operation was developed by Riordan (1959) who advocated that it should be done at an early age before the child was a year old. He described six cases and the operation seemed promising. The fibula was placed against the ulna with the upper fibular epiphysis at the wrist. The theory was that the epiphysis might con26
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tinue to grow and encourage growth of the carpus and hand in a normal position. Unfortunately these hopes have not been fulfilled and it is very seldom that any growth occurs in the transplanted epiphysis. An operation which holds out considerable promise in this condition, and seems to be the most successful procedure in a small child, is to centralise the carpus over the end of the ulna. If there is a soft tissue contracture the operation should be done through two separate incisions, one on the radial side to release the contracture and the other on the ulnar side to free the lower end of the ulna and facilitate the placing of the carpus over its end, Great care is necessary with the incision on the radial side as the median nerve is usually found lying closely subjacent to the skin and should always be looked for and retracted carefully aside before freeing the soft tissues. If no soft tissue contracture requires correction then the lower forearm and carpus can be readily exposed through an S-curved incision. It is usually found impossible to hold the carpus satisfactorily over the end of the ulna without resection of a portion of the centre of the carpus and this usually requires removal of the lunate and part or all of the capitate. The lower end of the ulna is then placed in the gap provided (Fig. 5) and care is taken not
Fig. 5. Tracings from radiographs of a child with radial aplasia. (a) Before operation. (b) After resection of central carpus and insertion of lower ulna into notch created. Position maintained by intra-medullary Kirschner wire. to interfere with the lower ulnar epiphysis and to free its soft tissue attachments as little as possible. The position m a y be held by a Kirschner wire passed across •die carpus and along the third or fourth metacarpal shaft. It is then passed out through the head of the metacarpal and re-introduced in a retrograde manner through the centre of the lower ulnar epiphysis and up the ulnar medullary cavity (Fig. 5). This can be a difficult procedure and should be done under X-ray control. This operation has b e e n very successful in controlling the wrist deformity in fourteen cases and if the Kirschner wire is passed across the centre of the epiphysis there seems little danger of interfering with its growth so that the ulna can develop satisfactorily. The Kirschner wire should be left in as long as possible and cases have been described where it has been left in for several years (Swanson, 1965) but there is a tendency for it to back out within a few months. Following removal of the wire the wrist remains stable with some slight movement, particularly of flexion and extension. Because of the technical difficulty in getting the Kirschner wire in satisfactory position and also because of its tendency to slip out after a few weeks or months, an attempt has been made to stabilise the hand :'vet the ulna without the necessity of pin fixation. In the last four cases the soft tissue over the carpus and wrist has been carefully dissected off bone and retained as a strong flap which could be repaired over the lower end of the ulna and carpus after insertion of the ulna into the notch cut in the carpus. This has proved very satisfactory and has remained as stable as those cases where a wire has been used. Vol. 4
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Provided the soft tissues on the radial side have not been allowed to contract the hand can usually be placed over the ulna without difficulty following resection of an adequate portion of carpus. In some cases, however, the fingers have flexed strongly following this and this has been found to be due to superficial flexor tightness. While this tightness can be overcome sufficiently in some cases by flexion of the elbow to a right angle in others the tightness remains and it is necessary at operation to lengthen or to resect a portion of the superficial tendon. Care must be taken to avoid excessive stretching of the median nerve. Flexion of the elbow usually relieves any undue tightness. The abnormal situation of the median nerve has already been mentioned and in two cases in this series the median nerve divided into two large equal divisions about two inches above the wrist. One division ran through the carpal tunnel, the other deviated radially to the situation normally occupied by the radial nerve. Post operative swelling has been invariable and severe in this series. While it may be limited in extent by careful preservation of dorsal veins and by careful haemostasis after release of the tourniquet the lack of good active mobility of the hand due to the structure of the finger joints and
Fig. 6. Bilateral radial club hand. (a) Before operation. (b) Two years after centralisation of carpus on right side.
Fig. 7. A typical case of radial club hand after operative correction by centralisation of carpus on ulna. (a) Showing digital extension and flexion from volar aspect. (b) Showing digital extension and flexion from the ulnar aspect. 28
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poor flexor muscles makes swelling inevitable. For this reason no post operative plaster is applied. A firm crepe compression bandage is applied and the hand elevated. The swelling is usually settled within a week of operation when a long plaster cast from metacarpal head to upper arm is applied with the elbow at ninety degrees. This plaster should be maintained for at least six weeks following which a plastic splint to hold the wrist in the corrected position is used for three months. The appearance of the hand in this corrected position is much improved (Fig. 6 and 7) and the loss of some of the wrist mobility has not in our cases resulted in any impairment of function. There is no doubt that function can be further improved in suitable cases by transfer of the index finger on a neurovascular bundle as described by Littler and using the techniques of Riordan and Buck-Gramcko. This has been carried out on seventeen occasions in this series. Surgery has been undertaken in this group of children only after very considerable thought and discussion with other surgeons who have had experience in this field. It was thought that the cosmetic appearance would be definitely enhanced but there was considerable division of opinion regarding the effect on function. In the light of experience and following careful pre- and post-operative assessment of function by a skilled occupational therapist it became evident that function was not impaired. Once the wrist had been stabilised the advantages of providing a thumb for prehension became apparent and in most cases following pollicisation there has been a significant increase in function. It may take several months for the child to become orientated to using the radial side of the hand in preference to the ulnar digits which have predominated previously. In the light of this experience and in an attempt to provide an early pattern of radial prehension there would appear to be good indications for early operation (within the first year of life) to stabilise the carpus by centralisation and pollicisation. SUMMARY
1. A series of forty-one children with radial club hand deformity is described. In twenty-two this was bilateral. 2. The importance of early splintage to prevent soft tissue contracture is stressed. A simple ratchet type of splint, which has proved effective, is described. Splintage is only required at night. 3. Operation by centralisation of the carpus over the ulna has proved satisfactory in correcting the deformity and producing wrist stability. A limited range of wrist movement is still possible and so far there does not appear to have been any detrimental effect on the growth of the lower ulnar epiphysis. A satisfactory correction can be maintained by a soft tissue periosteal flap. 4. While the expected improvement in appearance has been achieved the loss of function which had been anticipated has not materialised. 5. Surgical correction of the wrist deformity should not be contemplated unless there is active flexion of the elbow to about ninety degrees. In many cases where the elbow is stiff in extension at birth there is a tendency to gradual loosening of the elbow during the first year or two. Where this does not occur a posterior release of the elbow capsule (as carried out in arthrogryphosis) can be successful. Vol. 4
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. Pollicisation of the i n d e x finger should be seriously considered. The result will depend to a considerable extent on the structure a n d f u n c t i o n of the index finger. 7. I n order to get the best possible f u n c t i o n a l result early operation should be considered. ACKNOWLEDGEMENTS
I wish to thank all my colleagues who have referred patients. I am particularly indebted to the surgeons in many countries who have generously given advice from their experience of the management of this difficult condition. The help given by Miss Aline Macnaughtan, Head Occupational Therapist, with her careful assessment of the functional capabilities of these children has been invaluable. I am indebted to Miss Anne Sim for her willing secretarial assistance. Figures 4 and 5 are reproduced by kind permission of Butterworth & Co. (Publishers) Ltd., from Operative Surgery Second Edition, Vol. 11, The Hand.
REFERENCES
BARSKY, A. J. (1958) Congenital Anomalies of the Hand and their Surgical Treatment. Springfield, Illinois. Charles C. Thomas. BROWN, J. J. M. (1962) Surgery of Childhood. London. Edward Arnold. BUCK-GRAMCKO, D. Personal Communication. BUNNELL, S. (1964) Surgery of the Hand. Editor J. Boyes. 4th Edition, Philadelphia, J. B. Lippincott Company. HEIKEL, H. V. A. (1959) Aplasia and Hypoplasia of the Radius. Acta Orthopaedica Scandinavica, Supplement 39. JAMES, J. I. P. and LAMB, D. W. (1963) Congenital Abnormalities of the Limbs. The Practitioner, 191, 159. KATO, K. (1924) Congenital Absence of Radius. Journal of Bone and Joint Surgery, 6: 589. LAMB, D. W. (1970) Club Hand: Absent Radius. Operative Surgery, 2nd Edition. Editors C. Rob and R. Smith. London. Butterworths. ¥ol. 11, 12-16. LIDGE, Ralph. Quoted by A. B. Swanson. LITTLER, J. W. (1953) The Neurovascular Pedicle Method of Digital Transposition for Reconstruction of the Thumb. Plastic and Reconstructive Surgery. 12, 303. LLOYD-ROBERTS, G. C. (1963) Orthopaedic Abnormalities in "Congenital Abnormalities in Infancy". Edited by A. P. Norman, Oxford. Blackwell Scientific Publication. MERCER, W. and DUTHIE, R. B. (1964) Orthopaedic Surgery. 6th Edition. London. Edward Arnold. PULVERTAFT, R. G. and REID, D. A. C. (1963) Surgery of the Hand in Great Britain. British Journal of Surgery. 50: 673. RIORDAN, D. C. (1955) Congenital Absence of Radius. Journal of Bone and Joint Surgery. 37A, 1129. RIORDAN, D. C. (1966) Inter-clinic Information Bulletin. Vol. 4, No. 6. STARR, D. E. (1945)Congenital Absence of Radius. A Method of Surgical Correction. Journal of Bone and Joint Surgery. 27, 572. SWANSON, A. B. (1965) The Treatment of Congenital Limb Malformations. Kobe Journal Medical Sciences. Vol. 11. Supplement p. 41. 30
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