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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 with38 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 splintsand stretching. Moderate deformities treated operatively gained extension but at the loss of fingerflexion. 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 21patients had loss of flexion after operative treatment. Conservative treatment of camptodactyly isrecommended for digits with less than 60° lack of extension. Operative treatment should be reservedfor failed conservative treatment. Early joint raotion post-operatively appears essential and surgicalprocedures that immobilise the P.I.P. joints sl~tould be avoided.Journal of Hand Surgery (British Volume, 1990) 15B : 181-189

The word "camptodactyly" means "bent finger" inGreek; its numerous synonyms are described in thecomprehensive review by Smith and Kaplan (1968). surgical practice, it means a non-traumatic flexioncontracture of the proximal interphalangealjoint, usuallyof the little finger (Fig. 1). Involvement of either thedistal interphalangeal joint or the metacarpo-phalangealjoint suggests a post-traumatic cause rather than camp-todactyly. Likewise, camptodactyly should not be con-fused with Kirner’s deformity or with clinodactyly.

Camptodactyly may be divided into simple andcomplex types. Simple camptodactyly consists only ofthe flexion deformity of the P.I.P. joint (Fig. 2), whereasin complex camptodactyly, there are also other deformi-ties such as syndactyly or combinations of clinodactylyand camptodactyly (Fig. 3).

Surgical treatment for camptodactyly has been rec-ommended by a number of authors, although long-termobjective data to support this are often lacking. Inseries ~reported by Smith and Kaplan (1968), follow-up eight Of the nine operative cases was for no more than ayear. Similarly, limited data are available in the reports

Fig. 2 A mild familial deformity of late onset.

by Millesi (1974) and Courtemanche (1969). AlthoughMcFarlane et al. (1983) recommended surgical treatment,no patient with bilateral deformities who had undergonean operation on one hand requested surgery on the otherhand. Engber and Flatt (1977) also suggested surgicaltreatment for progressive cases, although they noted’thatoperative treatment was not uniformly satisfying.

Non-operative treatment for camptodactyly has re-cently been suggested, but no objective comparison withthe results of surgical treatment ,has been made (Hori etal., 1987).

The purpose of this paper is to review our experiencewith both operative and non-operative treatment ofsimple camptodactyly and to offer recommendations fortreatment based on objective long-term follow-up dataand a proposed clinical grading method.

Material

Fig. 1 Typical flexion deformity of P.I.P. joint of little finger. Fingerlacks full extension, both actively and passively1 but has :t’ullactive flexion.

The clinical records of 57 patients treated at the MayoClinic for camptodactyly from 1966 through 1986 werereviewed (Tables 1 and 2). Patients with complex multipledeformities of the hand (such as camptodactyly associatedwith syndactyly and camptodactyly in combination with

181VOL. 15B No. 2 MAY 1990

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J. J. SIEGERT, W. P. COONEY AND J. H. DOBYNS

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THE JOURNAL OF HAND SURGERY

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VC:i.. I5B No. 2 MAY 1990 183


184

I I I IIII + + ++ + I I


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Fig. 3


were lost to follow-up and the remaining 21 patients hada mean follow-up of 6.8 years (range 10 months-20 years;median, 5.4 years). The patients were also grouped intoeither early-onset deformities (younger than six at timeof onset) or late-onset deformities.

(a) Early-onset deformity of P.I.P. joints of middle, ring andlittle fingers, associated with complex clinodactyly of middleand ring fingers. (b) Fixed flexion of ulnar three digits notpassively correctable.

significant rotational deformities) were excluded. Thisstudy 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 treatedoperatively (Table 1) and 17 conservatively (Table 2). the conservative group, three patients were lost to follow-up; the other 14 patients had a mean follow-up period of6.5 years (range, 14 months-13.7 years; median, 6.2years). In the operatively-treated group, four patients

Table 3~Types of patients

No.

~ I Genetic consultation only 15~ II Deformity, mild or moderate 17~ III Deformity, severeor progressive 25

~. 15B No. 2 MAY 1990

Operative treatment group

In the operatively-treated group of 21 patients (38 digits)with follow-up, 11 had late-onset and 10 had early-onsetdeformities (Table 1). The average age of the entire groupat the time of the first operation was 13.3 years. Presentingsymptoms were cosmetic (progressive deformities) in 17,progressive deformity and functional difficulty in threeand progression of the deformity with pain in one. Therewas a positive family history in 11. 15 of the 21 patientshad bilateral involvement. Six had involvement of onlyone hand, and in only two did the deformity occur in thenon-dominant hand.

38 digits were operated on, 23 of which were littlefingers. All patients had full finger flexion beforeoperation. The complaint was limitation of extension, ofwhich the mean was 53.4° (range, 15°-95°).

Conservative treatment group

In the conservatively treated group of 14 patients (41digits), nine had late-onset and five had early-onsetdeformities (Table 2). The average age of the conservativegroup at the onset of treatment was 14.1 years (range 2-30 years). Most patients were between the ages of 11 and17..years. Five patients had involvement of only onehand, the dominant one in each. In the 23 hands, 41.digits were treated, 23 of which were fifth digits. In sixhands, the deformity involved the index, middle, ring,and little fingers. Five of the 14 conservatively-treatedpatients had a family history of camptodactyIyy. Themajor presenting complaints were progressive cosmeticdeformities (11 patients) and a combination of progres-sion and functional loss (three patients). One patient withcosmetic concerns also complained of occasional pain.

Patients in both treatment groups expressed difficultyin playing the piano, typing, wearing gloves, writing, andperforming manual labour.

Associated conditions in the 35 patients were general-ised 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 hada release of the flexor digitorum superficialis, as recom-

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mended by Smith and Kaplan (1968). Table 4 summarisesthe 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 thelittle finger, but in a few cases only a small transverseincision was made. The flexor digitorum superficialis wasisolated and any abnormality noted. A detailed review ofthe surgical records revealed that an abnormal lumbricalinsertion or flexor tendon anatomy was ,specificallysearched for in 17 of the 21 patients. Of these 17, onlytwo had abnormal lumbrical insertions. One other patienthad an abnormal superficialis insertion and partialdeficiency of the extensor mechanism. Four patients hadextremely tight superficialis tendons, one of whom alsohad unusually tight palmar fascial bands. Three patientshad unusually small superficialis tendons, one of whomalso had tight fascial bands. One patient had only unusualpalmar fascial bands. In six of the 17 patients, no specificanomalies were noted during exploration.¯ After release of the superficialis, a decision was made

about the joint capsule. In seven patients, a palmarcapsulotomy was performed and in five, the’, collateralligaments were released. In ten patients, the P.I.P. jointwas pinned in extension. In four patients, skin graftswere applied because of palmar skin deficiency.

Conservative treatment

Conservative treatment consisted of dynamic and staticpalmar-support splints, with the static splints revised, aspossible, to capitalise on any gains made from stretchingthe digits toward full extension. In general, static splintswere worn during the night and dynamic splints duringthe day. Eight patients had a combination of static anddynamic splints. Three had only static splint,’;, and twohad dynamic splints alone. One patient with mildcamptodactyly had stretching exercises only, withoutsplinting. In all cases of splinting, stretching exerciseswere also used. Four patients had complications causedby pressure from thesplint, including abrasion orbreakdown from the splint and mild skin irritations.

Table 4~Operative treatment in 21 patients with Camptodactyly (38Digits)

Numberof digits

Release of:Flexor superficialis 34Capsule 13Skin 6Fascial bands 5Collateral ligaments 13

Transfer of flexor superficialis 15Skin graft 4

Table 5--Classification of results

Excellent:

Good :

Fair:

Poor:

Correction to full extension, with less than 15° loss ofP.I.P.joint flexion

Correction to within 20° of full P.I.P. joint extension, ormore than 40° increase in P.I.P. joint extension, withless than 30° loss of flexion

Correction to within 40° of full P.I.P. joint extension, ormore than 20° increase in P.I.P. joint extension, withless than 45° loss of flexion

Less than 20° of improvement in P.I.P. joint extension, orless 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 inextension. Loss of flexion, however, was quite common,and significant loss of flexion from surgical treatmentwas measured in ten patients. Another six patients hadcomplete ankylosis of the P.I.P. joint, so that 16 of the 2tpatients had less movement after operation than before(Fig. 4b). Five patients had skin slough at either thewound or skin graft sites. One had persistent digitalnerve paraesthesiae. Further surgery, consisting of releaseof joint contractures and tendolysis, was required in 43~of the surgically-treated patients.

Of the 14 conservatively-treated patients, 12 hadimprovement in extension and only two had loss offlexion. Among the 41 digits, results were good in 27, fairin eight, and poor in six (Table 2). Patients with morethan one digit involved gained almost full extension, withimprovement of between 25° and 40°. Only three digitshad progressive loss of extension, extension lag changingfrom 40°-60° in the first, from 30°-35° in the second, andfrom 600-70° in the third (Fig. 4a). For the entire groupof 14 patients and 41 digits, the overall mean lack ofextension at the P.I.P. joint was 37° before treatment and16° after treatment, an average improvement of 21° perjoint (Table 2).

In an attempt to clarify the results of treatment, thedeformities before treatment were divided into threecategories on the basis of extension lack: mild (less than30°), moderate (from 30°-60°), and severe (more 60°). As shown in Table 6, the mean deformities beforetreatment in the conservative group were similar to thosein the surgical group for both the mild and moderatedeformity categories. After treatment, those with a milddeformity treated conservatively had almost cornpletecorrection of the deformity. In contrast, those with milddeformity treated operatively had, on average, a doubling

186 THE JOURNAL OF HAND SURGERY


Fig. 4

100

80

60

40

2O

Nonoperative Operative

100 -"-~ ~,.~ 10090

90~80 80 80

70

70~60 60 60

50

50~40 40 40

30

30~20 20 20

0 0 0PIP joint b PIP joint

a flexion stance flexion stanceResults of treatment. Each line represents one patient, with the pre-operative flexion stance on the left and the post-operative positionon the right. (a) Conservative treatment: 12 of 14 patients (86%) had improvement in extension, ° ormore, (b) Operative treatment:10 of 21 patients (48%) had improvement in extension.

in the flexion contracture from a mean of 19° to a meanof 39° after operation.

In the moderate deformities, both methods resulted ina mean improvement, but those treated conservativelyhad greater improvement overall than those treated byoperation (23° and 14°, respectively). In the severedeformities, there was improvement in extension of theP.I.P. joint after both types of treatment, but the originaldeformities in this severe category were, on average,greate~ in those treated surgically; also, very few patientswith severe deformity were treated conservatively (Table6). Thus, the ’results of treatment for severe deformities

reater than 60° extension .lag)are not directly compa-rable.

An attempt was also made to compare the age at onsetof the deformity, and the timing of treatment with theend results, but no correlations were apparent.

Discussion

Camptodactyly appears superficially to be a simpleproblem. In reality, however, it is a long-term andfrustrating problem to both patient and doctor. The causeis unknown, but the deformity often appears to have agenetic basis (Littman et al., 1968). Although some casesoccur sporadically, frequently an autosomal dominantpattern of inheritance is present, with a high degree ofpenetrance 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 anomal-ies, and a variety of syndromes ..have been described(Nevin et al., 1966; Welch and Telntamy, 1966; Gordonet 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 digits

Before After Before ~. Aftertreatment treatment treatment treatment

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

-. 15B No. 2 MAY 1990 187


Fig. 5

component, we believe that the so-called congenital andadolescent types are merely manifestations of the samecondition. Thus, we agree with Ba~inka (1964) that theterms "early" and "delayed" (or "late") rather than theterms "congenital" and "adolescent" should be used forthese two age clusters. In our study, the age at onset andthe age at which the operation was performed were notdeciding factors for the surgical findings or the prognosisfrom surgery. Perhaps the only importance of identifyingthe early type is to raise the possibility of othermusculoskeletal anomalies which the physician shouldlook for.

The pathological anatomy in camptodactyly is alsounclear. We believe that the deformity may be the result.of the inab!lity of palmar soft tissues to grow and lengthenin proportion to the adjacent skeleton. Thus, the normalrapid periods of skeletal growth during infancy and

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(a) Late-onset deformity of left little finger with recurrence P.I.P. contracture after surgical correction. (b) Radiographicappearance with secondary changes in articular surfaces ofP.I.P. joint: widened base of middle phalanx and flattening ofarticular condyle of proximal phalanx.

adolescence may account for the apparent bimodaldistribution of presentation..Smith and Kaplan (1968)have pointed out that virtually every structure about thebase of the finger has been implicated as the majordeforming factor. McFarlane et al. (1983) suggested thatlumbrical abnormalities are the major deforming force.However, in 17 of our patients lumbrical anomalies werespecifically looked for at operation, but only two hadsuch an abnormality. Anomalies of the superficialistendons were more common in our patients, four havingabnormally tight tendons, three having unusually smalltendons, and one having an abnormal insertion. Extensortendon abnormalities did not appear to be of significancein our patients. Thus, in contrast to McFarlane et al.(1983) and on the basis of our surgical findings, we agreewith Engber and Flatt’s (1977) statement that there probably no single cause for the deformity.

Although simple release of the flexor digitorumsuperficialis has been recommended as the preferredtreatment for this condition, we believe that the deformityalso involves the other tissues on the palmar half of thefinger. Release of the superficialis alone did not providelong-term improvement in most of our patients, butsuperficialis release combined with lengthening proce-dures to the skin, capsule, or fascia also proveddisappointing.

The operative treatment of many of our patients wassimilar to that described by Smith and Kaplan (1968),although our results were far worse. A likely explanationfor this disparity in outcomes is the marked difference infollow-up time (1 year or less compared with 6.8 years).A common finding among our patients was that resultssobn after surgery were encouraging, similar to thosereported by Smith and Kaplan (1968), but that after year, they deteriorated to unsatisfactory outcomes (Fig.5). .~

Courtemanche (1969) also advocated operative treat-ment, but once again, the recommendationwas based ononly three patients with little or no follow-up (a mean of9.5 months).

In the series by Engber arid Flatt (1977), averagesurgical follow-up was exactly one-half as long as that inour series. Their surgical results were less satisfactorythan Smith and Kaplan’s (1968): approximately one-third of the patients who underwent corrective operationswere improved, one-third unchanged, and one-thirdworse. Unfortunately, post-operative loss of flexion wasnot specifically discussed or included in their grading ofresults. For their patients treated conservatively, Engberand Flatt found that splinting and stretching wereeffective in only 20~. We are unable to explain why suchmeasures were more successful in our patients, except tosuggest 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 ina child are a difficult problem. Conservative treatment



by splinting for mild and moderate deformities appearsto be better than operative treatment, because the loss of!exion that we commonly found after surgical treatmentdoes not occur. More than 50~ of our patients stated thattheir hands were worse after operative treatment andthat the loss of flexion was a significant concern. For thisreason, post-operative loss of flexion was incorporatedinto our grading method. In addition to loss of flexion,two patients were greatly concerned about the residualsurgical scar.

From our data, it appears that operative treatmentshould be limited to progressive deformities in patientswith moderate-to-severe involvement and only after"onservative treatment has failed. The operative treat-.nent should include releases of tight tissues (usually theflexor digitorum superficialis, skin, and fascia) withoutcomplicated transfers to the extensor mechanism. Suchtransfers require post-operative immobilisation of thefinger joints which is likely to lead to loss of flexion oreven 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 simplecamptodactyly, particularly those with extension loss of60° or less. If conservative measures have failed andoperative treatment is undertaken, we recommend aprogramme of early post-operative mobilisation, togetherwith static and dynamic splinting to minimise the loss offlexion.

References

BARAITSER, M. (1982). A new camptodactyly syndrome. Journal of MedicalGenetics, 19 : 40-43.

.... . 15B No. 2 MAY 1990

BARAITSER, M., BURN, J. and FIXSEN, J. (1983). recessively in heritedwindmill-vane camptodactyly/ichthyosis syndrome. Journal of MedicalGenetics, 20: 125-127.

BAt~INKA, L. (1964). Campylodactylia (A Preliminary Communication). Chirnrgiae Plasticae (Prague), 6:1 : 54-60.

COURTEMANCHE, A. D. (1969). Campylodactyly: etiology and management.Plastic and Reconstructive Surgery, 44:5 : 451-454.

ENGBER, W. D. and FLATT, A. E. (1977). Camptodactyly: An analysis ofsixty-six patients and twenty-four operations. Journal of Hand Surgery, 2: 3 :216-224.

GOODMAN, R. M., KATZNELSON, M. B.-M., HERTZ, M. and KATZNEL-SON, A. (1976). Camptodactyly, with muscular hypoplasia, skeletaldysplasia, and abnormal palmar creases. Tel Hashomer camptodactylysynd rome. Journal of Medical Genetics, 13 : t 36.141.

GORDON, H., DAVIES, D. and BERMAN, M. (1969). Camptodactyly, CleftPalate, and Club Foot: A Syndrome Showing the Autosomal-dominantPattern of Inheritance. Journal of Medical Genetics, 6: 266.274.

HORI, M., NAKAMURA, R., INOUE, G., IMAMURA, T., HORRII, E.,TANAKA, Y. and MIURA, T. (1987). Nonoperative treatment ofcamptodactyly. Journal of Hand Surgery. 12A : 6: 1061 - 1065.

LITTMAN, A., YATES, J. W. and TREGER, A. (1968). Camptodactyly: Kindred Study. Journal of the American Medical Association, 206: 1565-1567.

McFARLANE, R. M., CURRY, G. I. and EVANS, H. B. (1983). Anomalies ofthe intrinsic muscles in camptodactyly. Journal of Hand Surgery, 8: 5(1):531-544.

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). FamilialCamptodactyly with Taurinuria. Journal of Medical Genetics, 3 : 265-268.

SMITH, R. J. and KAPLAN, E. B. (1968). Camptodactyly and SimilarAtraumatic Flexion Deformities of the Proximal Interphalangeal Joints ofthe Fingers: A Study of Thirty-One Cases. Journal of Bone and Joint Surgery,50A: 6:1187-1203.

WELCH, J. P. and TEMTAMY, S. A. (1966). Hereditary Contractures of theFingers (Camptodactyly). Journal of Medical Genetics, 3 : 104-113.

Accepted: 2~July, 1989William P. Cooney, M.D., Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905,U.S.A.

© 1990 The British Society for Surgery of the Hand

0266-7681/90/0015-0181/$10.0~

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