MANAGEMENT OF SIMPLE CAMPTODACTYLY

Transcription

1 MANAGEMENT OF SIMPLE CAMPTODACTYLY J. J. SIEGERT, W. P. COONEY and J. H. DOBYNS From the Section of Surgery of the Hand, Mayo Clinic and Mayo Foundation, Rochester, Minne&ota, U.S.A. From a review of 57 patients with flexion deformity of the fingers (camptodactyly), 21 patients with 38 digits treated operatively had 18% good or excellent results, whereas 14 patients (41 digits) treated conservatively had 66% good or excellent results. Mild deformities responded well to splints and stretching. Moderate deformities treated operatively gained extension but at the loss of finger flexion. Severe deformities had improvement in extension (averaging 19 in operative cases and 27 in conservative), but there was significant loss of flexion in the operative group. Overall, 16 of 21 patients had loss of flexion after operative treatment. Conservative treatment of camptodactyly is recommended for digits with less than 60 lack of extension. Operative treatment should be reserved for failed conservative treatment. Early joint raotion post-operatively appears essential and surgical procedures that immobilise the P.I.P. joints sl~tould be avoided. Journal of Hand Surgery (British Volume, 1990) 15B : The word "camptodactyly" means "bent finger" in Greek; its numerous synonyms are described in the comprehensive review by Smith and Kaplan (1968). surgical practice, it means a non-traumatic flexion contracture of the proximal interphalangealjoint, usually of the little finger (Fig. 1). Involvement of either the distal interphalangeal joint or the metacarpo-phalangeal joint suggests a post-traumatic cause rather than camptodactyly. Likewise, camptodactyly should not be confused with Kirner s deformity or with clinodactyly. Camptodactyly may be divided into simple and complex types. Simple camptodactyly consists only of the flexion deformity of the P.I.P. joint (Fig. 2), whereas in complex camptodactyly, there are also other deformities such as syndactyly or combinations of clinodactyly and camptodactyly (Fig. 3). Surgical treatment for camptodactyly has been recommended by a number of authors, although long-term objective data to support this are often lacking. In series ~reported by Smith and Kaplan (1968), follow-up eight Of the nine operative cases was for no more than a year. Similarly, limited data are available in the reports Fig. 1 Typical flexion deformity of P.I.P. joint of little finger. Finger lacks full extension, both actively and passively1 but has :t ull active flexion. VOL. 15B No. 2 MAY 1990 Fig. 2 A mild familial deformity of late onset. by Millesi (1974) and Courtemanche (1969). Although McFarlane et al. (1983) recommended surgical treatment, no patient with bilateral deformities who had undergone an operation on one hand requested surgery on the other hand. Engber and Flatt (1977) also suggested surgical treatment for progressive cases, although they noted that operative treatment was not uniformly satisfying. Non-operative treatment for camptodactyly has recently been suggested, but no objective comparison with the results of surgical treatment,has been made (Hori et al., 1987). The purpose of this paper is to review our experience with both operative and non-operative treatment of simple camptodactyly and to offer recommendations for treatment based on objective long-term follow-up data and a proposed clinical grading method. Material The clinical records of 57 patients treated at the Mayo Clinic for camptodactyly from 1966 through 1986 were reviewed (Tables 1 and 2). Patients with complex multiple deformities of the hand (such as camptodactyly associated with syndactyly and camptodactyly in combination with 181

2 J. J. SIEGERT, W. P. COONEY AND J. H. DOBYNS + + I t + I 182 THE JOURNAL OF HAND SURGERY

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4 J. J. SIEGERT, W. P. COONEY AND J. H. DOBYNS I I I IIII I I 184 THE JOURNAL OF HAND SURGERY

5 MANAGEMENT OF SIMPLE CAMPTODACTYLY were lost to follow-up and the remaining 21 patients had a mean follow-up of 6.8 years (range 10 months-20 years; median, 5.4 years). The patients were also grouped into either early-onset deformities (younger than six at time of onset) or late-onset deformities. Operative treatment group In the operatively-treated group of 21 patients (38 digits) with follow-up, 11 had late-onset and 10 had early-onset deformities (Table 1). The average age of the entire group at the time of the first operation was 13.3 years. Presenting symptoms were cosmetic (progressive deformities) in 17, progressive deformity and functional difficulty in three and progression of the deformity with pain in one. There was a positive family history in of the 21 patients had bilateral involvement. Six had involvement of only one hand, and in only two did the deformity occur in the non-dominant hand. 38 digits were operated on, 23 of which were little fingers. All patients had full finger flexion before operation. The complaint was limitation of extension, of which the mean was 53.4 (range, ). GERY Fig. 3 (a) Early-onset deformity of P.I.P. joints of middle, ring and little fingers, associated with complex clinodactyly of middle and ring fingers. (b) Fixed flexion of ulnar three digits not passively correctable. significant rotational deformities) were excluded. This study was confined, therefore, to simple camptodactyly. The patients could be grouped into three types (Table 3). Of the 57 patients, 37 were female. 25 were treated operatively (Table 1) and 17 conservatively (Table 2). the conservative group, three patients were lost to followup; the other 14 patients had a mean follow-up period of 6.5 years (range, 14 months-13.7 years; median, 6.2 years). In the operatively-treated group, four patients Table 3~Types of patients ~ I Genetic consultation only 15 II Deformity, mild or moderate 17 ~ III Deformity, severeor progressive 25 ~. 15B No. 2 MAY 1990 No. Conservative treatment group In the conservatively treated group of 14 patients (41 digits), nine had late-onset and five had early-onset deformities (Table 2). The average age of the conservative group at the onset of treatment was 14.1 years (range 2-30 years). Most patients were between the ages of 11 and 17..years. Five patients had involvement of only one hand, the dominant one in each. In the 23 hands, 41. digits were treated, 23 of which were fifth digits. In six hands, the deformity involved the index, middle, ring, and little fingers. Five of the 14 conservatively-treated patients had a family history of camptodactyiyy. The major presenting complaints were progressive cosmetic deformities (11 patients) and a combination of progression and functional loss (three patients). One patient with cosmetic concerns also complained of occasional pain. Patients in both treatment groups expressed difficulty in playing the piano, typing, wearing gloves, writing, and performing manual labour. Associated conditions in the 35 patients were generalised ligamentous laxity (one patient), severe scoliosis (one), pectus excavatum (one), congenital club foot tibial deficiency syndrome (one), Perthes disease (one), and toe contractures (two patients). Methods Operative treatment Of the 21 patients who had surgical treatment, most had a release of the flexor digitorum superficialis, as recom- 185

6 J. J. SIEGERT, W. P. COONEY AND J. H. DOBYNS mended by Smith and Kaplan (1968). Table 4 summarises the operative treatment in these 21 patients and 38 digits. The surgical exposure generally involved either a Z- plasty or a Bruner incision on the palmar aspect of the little finger, but in a few cases only a small transverse incision was made. The flexor digitorum superficialis was isolated and any abnormality noted. A detailed review of the surgical records revealed that an abnormal lumbrical insertion or flexor tendon anatomy was,specifically searched for in 17 of the 21 patients. Of these 17, only two had abnormal lumbrical insertions. One other patient had an abnormal superficialis insertion and partial deficiency of the extensor mechanism. Four patients had extremely tight superficialis tendons, one of whom also had unusually tight palmar fascial bands. Three patients had unusually small superficialis tendons, one of whom also had tight fascial bands. One patient had only unusual palmar fascial bands. In six of the 17 patients, no specific anomalies were noted during exploration. After release of the superficialis, a decision was made about the joint capsule. In seven patients, a palmar capsulotomy was performed and in five, the, collateral ligaments were released. In ten patients, the P.I.P. joint was pinned in extension. In four patients, skin grafts were applied because of palmar skin deficiency. Conservative treatment Conservative treatment consisted of dynamic and static palmar-support splints, with the static splints revised, as possible, to capitalise on any gains made from stretching the digits toward full extension. In general, static splints were worn during the night and dynamic splints during the day. Eight patients had a combination of static and dynamic splints. Three had only static splint, ;, and two had dynamic splints alone. One patient with mild camptodactyly had stretching exercises only, without splinting. In all cases of splinting, stretching exercises were also used. Four patients had complications caused by pressure from thesplint, including abrasion or breakdown from the splint and mild skin irritations. Table 4~Operative treatment in 21 patients with Camptodactyly (38 Digits) Number of digits Release of: Flexor superficialis 34 Capsule 13 Skin 6 Fascial bands 5 Collateral ligaments 13 Transfer of flexor superficialis 15 Skin graft 4 Table 5--Classification of results Excellent: Good : Fair: Poor: Correction to full extension, with less than 15 loss of P.I.P.joint flexion Correction to within 20 of full P.I.P. joint extension, or more than 40 increase in P.I.P. joint extension, with less than 30 loss of flexion Correction to within 40 of full P.I.P. joint extension, or more than 20 increase in P.I.P. joint extension, with less than 45 loss of flexion Less than 20 of improvement in P.I.P. joint extension, or less than 40 of total P.I.P. joint motion Results of treatment The results were classified as shown in Table 5. Following operation, there were no excellent and only seven good results; six were fair and 25 poor (Table 1). The average lack of extension before operation was 53 and afterwards 43, an. average improvement of only 10 Ten of the 21 patients had measurable improvement in extension. Loss of flexion, however, was quite common, and significant loss of flexion from surgical treatment was measured in ten patients. Another six patients had complete ankylosis of the P.I.P. joint, so that 16 of the 2t patients had less movement after operation than before (Fig. 4b). Five patients had skin slough at either the wound or skin graft sites. One had persistent digital nerve paraesthesiae. Further surgery, consisting of release of joint contractures and tendolysis, was required in 43~ of the surgically-treated patients. Of the 14 conservatively-treated patients, 12 had improvement in extension and only two had loss of flexion. Among the 41 digits, results were good in 27, fair in eight, and poor in six (Table 2). Patients with more than one digit involved gained almost full extension, with improvement of between 25 and 40. Only three digits had progressive loss of extension, extension lag changing from in the first, from in the second, and from in the third (Fig. 4a). For the entire group of 14 patients and 41 digits, the overall mean lack of extension at the P.I.P. joint was 37 before treatment and 16 after treatment, an average improvement of 21 per joint (Table 2). In an attempt to clarify the results of treatment, the deformities before treatment were divided into three categories on the basis of extension lack: mild (less than 30 ), moderate (from ), and severe (more 60 ). As shown in Table 6, the mean deformities before treatment in the conservative group were similar to those in the surgical group for both the mild and moderate deformity categories. After treatment, those with a mild deformity treated conservatively had almost cornplete correction of the deformity. In contrast, those with mild deformity treated operatively had, on average, a doubling 186 THE JOURNAL OF HAND SURGERY

7 O Nonoperative MANAGEMENT OF SIMPLE CAMPTODACTYLY 90 Operative 100 -"-~ ~,.~ ~ ~ ~ ~ Fig PIP joint b PIP joint a flexion stance flexion stance Results of treatment. Each line represents one patient, with the pre-operative flexion stance on the left and the post-operative position on the right. (a) Conservative treatment: 12 of 14 patients (86%) had improvement in extension, ormore, (b) Operative trea tment: 10 of 21 patients (48%) had improvement in extension. in the flexion contracture from a mean of 19 to a mean of 39 after operation. In the moderate deformities, both methods resulted in a mean improvement, but those treated conservatively had greater improvement overall than those treated by operation (23 and 14, respectively). In the severe deformities, there was improvement in extension of the P.I.P. joint after both types of treatment, but the original deformities in this severe category were, on average, greate~ in those treated surgically; also, very few patients with severe deformity were treated conservatively (Table 6). Thus, the results of treatment for severe deformities reater than 60 extension.lag)are not directly comparable. An attempt was also made to compare the age at onset of the deformity, and the timing of treatment with the end results, but no correlations were apparent. Discussion Camptodactyly appears superficially to be a simple problem. In reality, however, it is a long-term and frustrating problem to both patient and doctor. The cause is unknown, but the deformity often appears to have a genetic basis (Littman et al., 1968). Although some cases occur sporadically, frequently an autosomal dominant pattern of inheritance is present, with a high degree of penetrance but various types of phenotypic expression (Gordon et al., 1969). In our total study group of patients, 23 had a family history of camptodactyly. Numerous reports have associated it with other anomalies, and a variety of syndromes..have been described (Nevin et al., 1966; Welch and Telntamy, 1966; Gordon et al., 1969; Goodman et al., 1976; Baraitser, 1982; Baraitser et al., 1983). Because of this strong genetic Table 6--Mean lack of full P.I.P.joint extension before and after Operative and Conservative treatment in relation to severity of deformity Deformity, : Operative treatment Conservative treatment No. of Lack of extension (degrees) No. of " Lack of extension (degrees) digits Before After digits Before ~. After treatment treatment treatment treatment Mild ) (< < 1 Moderate (30 to ) " Severe ) ( > B No. 2 MAY

8 component, we believe that the so-called congenital and adolescent types are merely manifestations of the same condition. Thus, we agree with Ba~inka (1964) that the terms "early" and "delayed" (or "late") rather than the terms "congenital" and "adolescent" should be used for these two age clusters. In our study, the age at onset and the age at which the operation was performed were not deciding factors for the surgical findings or the prognosis from surgery. Perhaps the only importance of identifying the early type is to raise the possibility of other musculoskeletal anomalies which the physician should look for. The pathological anatomy in camptodactyly is also unclear. We believe that the deformity may be the result.of the inab!lity of palmar soft tissues to grow and lengthen in proportion to the adjacent skeleton. Thus, the normal rapid periods of skeletal growth during infancy and Fig. 5 (a) Late-onset deformity of left little finger with recurrence P.I.P. contracture after surgical correction. (b) Radiographic appearance with secondary changes in articular surfaces of P.I.P. joint: widened base of middle phalanx and flattening of articular condyle of proximal phalanx. J. J. SIEGERT, W. P. COONEY AND J. H. DOBYNS adolescence may account for the apparent bimodal distribution of presentation..smith and Kaplan (1968) have pointed out that virtually every structure about the base of the finger has been implicated as the major deforming factor. McFarlane et al. (1983) suggested that lumbrical abnormalities are the major deforming force. However, in 17 of our patients lumbrical anomalies were specifically looked for at operation, but only two had such an abnormality. Anomalies of the superficialis tendons were more common in our patients, four having abnormally tight tendons, three having unusually small tendons, and one having an abnormal insertion. Extensor tendon abnormalities did not appear to be of significance in our patients. Thus, in contrast to McFarlane et al. (1983) and on the basis of our surgical findings, we agree with Engber and Flatt s (1977) statement that there probably no single cause for the deformity. Although simple release of the flexor digitorum superficialis has been recommended as the preferred treatment for this condition, we believe that the deformity also involves the other tissues on the palmar half of the finger. Release of the superficialis alone did not provide long-term improvement in most of our patients, but superficialis release combined with lengthening procedures to the skin, capsule, or fascia also proved disappointing. The operative treatment of many of our patients was similar to that described by Smith and Kaplan (1968), although our results were far worse. A likely explanation for this disparity in outcomes is the marked difference in follow-up time (1 year or less compared with 6.8 years). A common finding among our patients was that results sobn after surgery were encouraging, similar to those reported by Smith and Kaplan (1968), but that after year, they deteriorated to unsatisfactory outcomes (Fig. 5)..~ Courtemanche (1969) also advocated operative treatment, but once again, the recommendationwas based on only three patients with little or no follow-up (a mean of 9.5 months). In the series by Engber arid Flatt (1977), average surgical follow-up was exactly one-half as long as that in our series. Their surgical results were less satisfactory than Smith and Kaplan s (1968): approximately onethird of the patients who underwent corrective operations were improved, one-third unchanged, and one-third worse. Unfortunately, post-operative loss of flexion was not specifically discussed or included in their grading of results. For their patients treated conservatively, Engber and Flatt found that splinting and stretching were effective in only 20~. We are unable to explain why such measures were more successful in our patients, except to suggest that patient compliance is important in determin-. ing the.outcome of splintage (Hori et al., 1987). Progressive flexion contractures of the P.I.P. joints in a child are a difficult problem. Conservative treatment 188 THE JOURNAL OF HAND SURGERY

9 MANAGEMENT OF SIMPLE CAMPTODACTYLY by splinting for mild and moderate deformities appears to be better than operative treatment, because the loss of!exion that we commonly found after surgical treatment does not occur. More than 50~ of our patients stated that their hands were worse after operative treatment and that the loss of flexion was a significant concern. For this reason, post-operative loss of flexion was incorporated into our grading method. In addition to loss of flexion, two patients were greatly concerned about the residual surgical scar. From our data, it appears that operative treatment should be limited to progressive deformities in patients with moderate-to-severe involvement and only after "onservative treatment has failed. The operative treat-.nent should include releases of tight tissues (usually the flexor digitorum superficialis, skin, and fascia) without complicated transfers to the extensor mechanism. Such transfers require post-operative immobilisation of the finger joints which is likely to lead to loss of flexion or even ankylosis. Conclusion This study shows that camptodactyty is difficult to treat :.lccessfully. From our results, we recommend that.:perative treatment be avoided in most cases of simple camptodactyly, particularly those with extension loss of 60 or less. If conservative measures have failed and operative treatment is undertaken, we recommend a programme of early post-operative mobilisation, together with static and dynamic splinting to minimise the loss of flexion. References BARAITSER, M. (1982). A new camptodactyly syndrome. Journal of Medical Genetics, 19 : BARAITSER, M., BURN, J. and FIXSEN, J. (1983). recessively in herited windmill-vane camptodactyly/ichthyosis syndrome. Journal of Medical Genetics, 20: BAt~INKA, L. (1964). Campylodactylia (A Preliminary Communication). Chirnrgiae Plasticae (Prague), 6:1 : COURTEMANCHE, A. D. (1969). Campylodactyly: etiology and management. Plastic and Reconstructive Surgery, 44:5 : ENGBER, W. D. and FLATT, A. E. (1977). Camptodactyly: An analysis of sixty-six patients and twenty-four operations. Journal of Hand Surgery, 2: 3 : GOODMAN, R. M., KATZNELSON, M. B.-M., HERTZ, M. and KATZNEL- SON, A. (1976). Camptodactyly, with muscular hypoplasia, skeletal dysplasia, and abnormal palmar creases. Tel Hashomer camptodactyly synd rome. Journal of Medical Genetics, 13 : t GORDON, H., DAVIES, D. and BERMAN, M. (1969). Camptodactyly, Cleft Palate, and Club Foot: A Syndrome Showing the Autosomal-dominant Pattern of Inheritance. Journal of Medical Genetics, 6: HORI, M., NAKAMURA, R., INOUE, G., IMAMURA, T., HORRII, E., TANAKA, Y. and MIURA, T. (1987). Nonoperative treatment of camptodactyly. Journal of Hand Surgery. 12A : 6: LITTMAN, A., YATES, J. W. and TREGER, A. (1968). Camptodactyly: Kindred Study. Journal of the American Medical Association, 206: McFARLANE, R. M., CURRY, G. I. and EVANS, H. B. (1983). Anomalies of the intrinsic muscles in camptodactyly. Journal of Hand Surgery, 8: 5(1): MILLESI, H. Camptodacyly. In: Littler, J. W., Cramer, L. M. and Smith, J. W. (Eds.). Symposium on Reconstructive Hand Surgery. St. Louis, C. V. Mosby, 1974: Vol. 9: 175-I77. NEVIN, N. C., HURWITZ, L. J. and NEILL, D. W. (1966). Familial Camptodactyly with Taurinuria. Journal of Medical Genetics, 3 : SMITH, R. J. and KAPLAN, E. B. (1968). Camptodactyly and Similar Atraumatic Flexion Deformities of the Proximal Interphalangeal Joints of the Fingers: A Study of Thirty-One Cases. Journal of Bone and Joint Surgery, 50A: 6: WELCH, J. P. and TEMTAMY, S. A. (1966). Hereditary Contractures of the Fingers (Camptodactyly). Journal of Medical Genetics, 3 : Accepted: 2~July, 1989 William P. Cooney, M.D., Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905, U.S.A The British Society for Surgery of the Hand /90/ /$10.0~ B No. 2 MAY