Management of Simple Camptodactyly

MANAGEMENT OF SIMPLE CAMPTODACTYLY

J. J. SIEGERT, W. P. COONEYand J. H. DOBYNS Fromthe Section of Surgeryof the Hand,Mayo Clinic and MayoFoundation, Rochester, Minne&ota, U.S.A. Froma reviewof 57 patients with flexion deformityof the fingers (camptodactyly),21 patients with 38 digits treated operatively had 18%good or excellent results, whereas14 patients (41 digits) treated conservativelyhad 66% good or excellent results. Milddeformities respondedwell to splints andstretching. Moderatedeformities treated operatively gained extension but at the loss of finger flexion. Severe deformities hadimprovement in extension (averaging19 ° in° operative cases and 27 in conservative), but there wassignificant loss of flexion in the operative group.Overall, 16 of 21 patients had loss of flexion after operative treatment. Conservativetreatment of camptodactylyis recommendedfor digits with less than 60° lack of extension. Operativetreatment should be reserved for failed conservativetreatment. Early joint raotion post-operativelyappears essential andsurgical proceduresthat immobilisethe P.I.P. joints sl~tould be avoided. Journal of HandSurgery (British Volume, 1990) 15B : 181-189

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 con- fused 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 deformi- Fig. 2 Amild familial deformity of late onset. ties such as syndactyly or combinations of clinodactyly and camptodactyly (Fig. 3). by Millesi (1974) and Courtemanche (1969). Although Surgical treatment for camptodactyly has been rec- McFarlaneet al. (1983) recommendedsurgical treatment, ommendedby a number of authors, although long-term no patient with bilateral deformities whohad undergone objective data to support this are often lacking. In an operation on one hand requested surgery on the other series ~reported by Smith and Kaplan (1968), follow-up hand. Engber and Flatt (1977) also suggested surgical eight Of the nine operative cases was for no more than a treatment for progressive cases, although they noted’that year. Similarly, limited data are available in the reports operative treatment was not uniformly satisfying. Non-operative treatment for camptodactyly has re- cently 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 complexmultiple Fig. 1 Typicalflexion deformity of P.I.P. joint of little finger.Finger deformities of the hand (such as camptodactylyassociated lacksfull extension,both actively and passively1 but has:t’ull activeflexion. with syndactyly and camptodactyly in combination with

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were lost to follow-up and the remaining 21 patients had a meanfollow-up of 6.8 years (range 10 months-20years; 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 symptomswere 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 11. 15 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, 15°-95°).

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 Fig. 3 (a) Early-onset deformity of P.I.P. joints of middle, ring and little fingers, associated with complexclinodactyly of middle hands, the deformity involved the index, middle, ring, and ring fingers. (b) Fixed flexion of ulnar three digits not and little fingers. Five of the 14 conservatively-treated passively correctable. patients had a family history of camptodactyIyy. The major presenting complaints were progressive cosmetic significant rotational deformities) were excluded. This deformities (11 patients) and a combination of progres- study was confined, therefore, to simple camptodactyly. sion and functional loss (three patients). Onepatient with The patients could be grouped into three types (Table 3). cosmetic concerns also complained of occasional pain. Of the 57 patients, 37 were female. 25 were treated Patients in both treatment groups expressed difficulty operatively (Table 1) and 17 conservatively (Table 2). in playing the piano, typing, wearinggloves, writing, and the conservative group, three patients were lost to follow- performing manual labour. up; the other 14 patients had a meanfollow-up period of Associated conditions in the 35 patients were general- 6.5 years (range, 14 months-13.7 years; median, 6.2 ised ligamentous laxity (one patient), severe scoliosis years). In the operatively-treated group, four patients (one), pectus excavatum(one), congenital club foot tibial deficiency syndrome(one), Perthes’ disease (one), and toe contractures (two patients). Table 3~Types of patients Methods No. Operative treatment ~ I Genetic consultation only 15 ~ II Deformity, mild or moderate 17 ~ III Deformity, severeor progressive 25 Of the 21 patients whohad surgical treatment, most had a release of the flexor digitorum superficialis, as recom-

GERY ~. 15B No. 2 MAY1990 185 J. J. SIEGERT, W. P. COONEYAND J. H. DOBYNS mended by Smith and Kaplan (1968). Table 4 summarises Table5--Classification of results the operative treatment in these 21 patients and 38 digits. ° The surgical exposure generally involved either a Z- Excellent: Correction to full extension, with less than 15 loss of P.I.P.joint flexion plasty or a Bruner incision on the palmar aspect of the Good : Correction to within 20° of full P.I.P. joint extension, or little finger, but in a few cases only a small transverse more than 40° increase in P.I.P. joint extension, with incision was made. The flexor digitorum superficialis was less than 30° loss of flexion ° isolated and any abnormality noted. A detailed review of Fair: Correction to within 40 of full P.I.P. joint extension, or more than 20° increase in P.I.P. joint extension, with the surgical records revealed that an abnormal lumbrical less than 45° loss of flexion insertion or flexor tendon anatomy was ,specifically Poor: Less than 20° of improvementin P.I.P. joint extension, or searched for in 17 of the 21 patients. Of these 17, only less than 40° of total P.I.P. joint motion 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 whomalso had unusually tight palmar fascial bands. Three patients Results of treatment had unusually small superficialis tendons, one of whom also had tight fascial bands. Onepatient had only unusual The results were classified as shownin Table 5. palmar fascial bands. In six of the 17 patients, no specific Following operation, there were no excellent and only anomalies were noted during exploration. seven good results; six were fair and 25 poor (Table 1). ¯ After release of the superficialis, a decision was made The° average lack of extension before operation was 53 about the joint capsule. In seven patients, a palmar and afterwards 43°, an°. average improvementof only 10 capsulotomy was performed and in five, the’, collateral Ten of the 21 patients had measurable improvement in ligaments were released. In ten patients, the P.I.P. joint extension. Loss of flexion, however, was quite common, was pinned in extension. In four patients, skin grafts and significant loss of flexion from surgical treatment were applied because of palmar skin deficiency. was measured in ten patients. Another six patients had completeankylosis of the P.I.P. joint, so that 16 of the 2t Conservative treatment patients had less movementafter operation than before (Fig. 4b). Five patients had skin slough at either the Conservative treatment consisted of dynamic and static woundor skin graft sites. One had persistent digital palmar-supportsplints, with the static splints revised, as nerve paraesthesiae. Further surgery, consisting of release possible, to capitalise on any gains madefrom stretching of joint contractures and tendolysis, was required in 43~ the digits toward full extension. In general, static splints of the surgically-treated patients. were worn during the night and dynamic splints during Of the 14 conservatively-treated patients, 12 had the day. Eight patients had a combination of static and improvement in extension and only two had loss of dynamicsplints. Three had only static splint,’;, and two flexion. Amongthe 41 digits, results were good in 27, fair had dynamic splints alone. One patient with mild in eight, and poor in six (Table 2). Patients with more camptodactyly had stretching exercises only, without than one digit involved gained almost full extension, with splinting. In all cases of splinting, stretching exercises improvement of between 25° and 40°. Only three digits were also used. Four patients had complications caused had progressive loss of extension, extension lag changing by pressure from thesplint, including abrasion or from 40°-60° in the first, from 30°-35° in the second, and breakdownfrom the splint and mild skin irritations. from 600-70° 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 improvementof 21° per Table 4~Operative treatment in 21 patients with Camptodactyly(38 joint (Table 2). Digits) In an attempt to clarify the results of treatment, the Number deformities before treatment were divided into three of digits categories on the basis of extension lack: mild (less than 30°), moderate (from 30°-60°), and severe (more Release of: 60°). As shown in Table 6, the mean deformities before Flexor superficialis 34 Capsule 13 treatment in the conservative group were similar to those Skin 6 in the surgical group for both the mild and moderate Fascial bands 5 deformity categories. After treatment, those with a mild Collateral ligaments 13 deformity treated conservatively had almost cornplete Transfer of flexor superficialis 15 correction of the deformity. In contrast, those with mild Skin graft 4 deformity treated operatively had, on average, a doubling

186 THE JOURNAL OF HAND SURGERY MANAGEMENTOF SIMPLE CAMPTODACTYLY Nonoperative Operative 100 100 -"-~ ~,.~ 100 90 80 80 80 80 90~ 70 60 60 60 60 70~ 50 40 40 40 40 50~ 30 2O 20 20 20 30~

0 0 0 PIPjoint b PIPjoint a flexion stance flexion stance Fig. 4 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 improvementin extension, ° ormore, (b) Operative trea tment: 10 of 21 patients (48%) had improvementin extension. in the flexion contracture from a mean of 19° to a mean Discussion of 39° after operation. In the moderate deformities, both methods resulted in Camptodactyly appears superficially to be a simple a mean improvement, but those treated conservatively problem. In reality, however, it is a long-term and had greater improvement overall than those treated by frustrating problem to both patient and doctor. The cause operation (23° and 14°, respectively). In the severe is unknown, but the deformity often appears to have a deformities, there was improvementin extension of the genetic basis (Littman et al., 1968). Although somecases P.I.P. joint after both types of treatment, but the original occur sporadically, frequently an autosomal dominant deformities in this severe category were, on average, pattern of inheritance is present, with a high degree of greate~ in those treated surgically; also, very few patients penetrance but various types of phenotypic expression with severe deformity were treated conservatively (Table (Gordon et al., 1969). In our total study group of 6). Thus, the ’results of treatment for severe deformities patients, 23 had a family history of camptodactyly. reater than 60° extension .lag)are not directly compa- Numerousreports have associated it with other anomal- rable. ies, and a variety of syndromes ..have been described An attempt was also made to compare the age at onset (Nevin et al., 1966; Welch and Telntamy, 1966; Gordon of the deformity, and the timing of treatment with the et al., 1969; Goodmanet al., 1976; Baraitser, 1982; end results, but no correlations were apparent. Baraitser et al., 1983). Because of this strong genetic

Table 6--Meanlack of full P.I.P.joint extension before and after Operativeand Conservativetreatment in relation to severity of deformity

Conservative treatment Deformity , : Operative treatment No. of Lack of extension (degrees) No. of " Lack of extension (degrees) digits digits Before After Before ~. After treatment treatment treatment treatment

Mild°) (< 30 6 19 39 14 1.8 < 1 Moderate(30 ° to°) 60 17 43 29 " 23 43 20 Severe°) ( > 60 15 79 60 4 71 44

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component, we believe that the so-called congenital and adolescence may account for the apparent bimodal adolescent types are merely manifestations of the same distribution of presentation..Smith and Kaplan (1968) condition. Thus, we agree with Ba~inka (1964) that the have pointed out that virtually every structure about the terms "early" and "delayed" (or "late") rather than the base of the finger has been implicated as the major terms "congenital" and "adolescent" should be used for deforming factor. McFarlaneet al. (1983) suggested that these two age clusters. In our study, the age at onset and lumbrical abnormalities are the major deforming force. the age at which the operation was performed were not However,in 17 of our patients lumbrical anomalies were deciding factors for the surgical findings or the prognosis specifically looked for at operation, but only two had from surgery. Perhaps the only importance of identifying such an abnormality. Anomalies of the superficialis the early type is to raise the possibility of other tendons were more commonin our patients, four having musculoskeletal anomalies which the physician should abnormally tight tendons, three having unusually small look for. tendons, and one having an abnormal insertion. Extensor The pathological anatomy in camptodactyly is also tendon abnormalities did not appear to be of significance unclear. Webelieve that the deformity maybe the result in our patients. Thus, in contrast to McFarlane et al. .of the inab!lity of palmar soft tissues to growand lengthen (1983) and on the basis of our surgical findings, we agree in proportion to the adjacent skeleton. Thus, the normal with Engber and Flatt’s (1977) statement that there rapid periods of skeletal growth during infancy and probably no single cause for the deformity. Although simple release of the flexor digitorum superficialis has been recommendedas 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 proce- dures 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 outcomesis the markeddifference in follow-up time (1 year or less comparedwith 6.8 years). A commonfinding 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 recommendationwasbased on only three patients with little or no follow-up (a meanof 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 one- third of the patients whounderwent 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~. Weare unable to explain whysuch Fig. 5 (a) Late-onset deformity of left little finger with recurrence measures were more successful in our patients, except to P.I.P. contracture after surgical correction. (b) Radiographic suggest that patient complianceis important in determin-. appearance with secondary changes in articular surfaces of ing the.outcomeof splintage (Hori et al., 1987). P.I.P. joint: widenedbase of middle phalanx and flattening of Progressive flexion contractures of the P.I.P. joints in articular condyle of proximal phalanx. a child are a difficult problem. Conservative treatment

188 THE JOURNAL OF HAND SURGERY MANAGEMENT OF SIMPLE CAMPTODACTYLY by splinting for mild and moderatedeformities appears BARAITSER,M., BURN,J. and FIXSEN, J. (1983). recessively in herited windmill-vane camptodactyly/ichthyosis syndrome. Journal of Medical to be better than operative treatment, becausethe loss of Genetics, 20: 125-127. !exion that we commonlyfound after surgical treatment BAt~INKA,L. (1964). Campylodactylia (A Preliminary Communication). does not occur. Morethan 50~ of our patients stated that Chirnrgiae Plasticae (Prague), 6:1 : 54-60. COURTEMANCHE,A. D. (1969). Campylodactyly: etiology and management. their hands were worse after operative treatment and Plastic and Reconstructive Surgery, 44:5 : 451-454. that the loss of flexion wasa significant concern.For this ENGBER,W. D. and FLATT, A. E. (1977). Camptodactyly: An analysis of sixty-six patients andtwenty-four operations. Journalof HandSurgery, 2: 3 : reason, post-operative loss of flexion was incorporated 216-224. into our grading method.In addition to loss of flexion, GOODMAN,R. M., KATZNELSON,M. B.-M., HERTZ, M. and KATZNEL- two patients were greatly concerned about the residual SON, A. (1976). Camptodactyly, with muscular hypoplasia, skeletal dysplasia, and abnormal palmar creases. Tel Hashomer camptodactyly surgical scar. synd rome. Journal of Medical Genetics, 13 : t 36.141. Fromour data, it appears that operative treatment GORDON,H., DAVIES, D. and BERMAN,M. (1969). Camptodactyly, Cleft Palate, and Club Foot: A Syndrome Showing the Autosomal-dominant should be limited to progressive deformities in patients Pattern of Inheritance. Journal of Medical Genetics, 6: 266.274. with moderate-to-severe involvement and only after HORI, M., NAKAMURA,R., INOUE, G., IMAMURA, T., HORRII, E., "onservative treatment has failed. The operative treat- TANAKA,Y. and MIURA, T. (1987). Nonoperative treatment of camptodactyly. Journal of HandSurgery. 12A: 6: 1061 - 1065. .nent shouldinclude releases of tight tissues (usually the LITTMAN,A., YATES, J. W. and TREGER, A. (1968). Camptodactyly: flexor digitorumsuperficialis, skin, and fascia) without Kindred Study. Journal of the American Medical Association, 206: 1565- complicated transfers to the extensor mechanism.Such 1567. McFARLANE,R. M., CURRY,G. I. and EVANS,H. B. (1983). Anomalies of transfers require post-operative immobilisation of the the intrinsic muscles in camptodactyly. Journal of Hand Surgery, 8: 5(1): finger joints whichis likely to lead to loss of flexion or 531-544. MILLESI,H. Camptodacyly. In: Littler, J. W., Cramer, L. M. and Smith, J. W. even ankylosis. (Eds.). Symposiumon 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 Conclusion Camptodactylywith Taurinuria. Journal of Medical Genetics, 3 : 265-268. SMITH, R. J. and KAPLAN,E. B. (1968). Camptodactyly and Similar This study showsthat camptodactytyis difficult to treat Atraumatic Flexion Deformities of the Proximal Interphalangeal Joints of :.lccessfully. Fromour results, we recommendthat the Fingers: A Study of Thirty-One Cases. Journal of Bone and Joint Surgery, 50A: 6:1187-1203. .:perative treatment be avoided in most cases of simple WELCH,J. P. and TEMTAMY,S. A. (1966). Hereditary Contractures of the camptodactyly,particularly those with extension loss of Fingers (Camptodactyly). Journal of Medical Genetics, 3 : 104-113. 60° or less. If conservative measures have failed and operative treatment is undertaken, we recommenda programmeof early post-operative mobilisation, together with static and dynamicsplinting to minimisethe loss of flexion. Accepted:2~July, 1989 WilliamP. Cooney,M.D., MayoClinic, 200 First Street SW,Rochester, Minnesota 55905, U.S.A. References ©1990 The British Societyfor Surgeryof the Hand BARAITSER,M. (1982). A new camptodactyly syndrome. Journal of Medical Genetics, 19 : 40-43. 0266-7681/90/0015-0181/$10.0~

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