Instituto Nacional de Traumatologia e Ortopedia, Rio de Janeiro, RJ, Brasil
Camptodactyly is a congenital deformity characterized by a flexed posture in the proximal interphalangeal joint. It is gen-erally found in the little finger and may or may not include the other fingers. It is painless and nontraumatic. 1-9
It affects approximately 1% of the population. 8,10 It is bilat-eral in around two thirds of the patients, although the degree of contracture is usually not symmetrical. 8,11,12
The deformity generally increases during growth spurts, especially during the periods of rapid growth from one to four years and from 10 to 14 years of age. 5,11,13
The primary cause of this deformity is still a matter for discussion and there is no consensus in the worldwide literature. 3,4,8,10-13 Although some cases occur sporadically, there is often an autosomal inheritance pattern present. 4,7,8,14 The metacarpophalangeal and distal interphalangeal joints are unaffected, although they may develop compen-satory deformities. 12
According to Siergert et al.,7 camptodactyly can be divided into simple and complex types from a clinical point of view. The simple type consists of flexed contracture of the proximal interphalangeal joint. In complex camptodactyly, there are other associateddeformities suchas syndactyly or a combina-tion of clinodactyly and camptodactyly. 7,10 Glicenstein et al. 15 classified camptodactyly into:
Primitive: when it appears in the first years of life. It affects both sexes in the same proportions and evolves with skele-tal growth. It may also appear close to adolescence, with clear predominance in females. It is frequently bilateral. It is restricted to the little finger and progresses rapidly during the growth spurt.
Secondary: associated with syndromes and other malforma-tions and normally involves more than one finger. The most frequent associations are: radial club hand, oculodentodigi-tal syndrome, Marfan syndrome and arthrogryposis. 15,16
In 1994, Benson et al. 17 classified camptodactyly as follows:
Type I: This is the commonest form and it becomes evident during childhood. It generally affects the little finger alone. It affects boys and girls equally.
Type II: Camptodactylyof adolescence,whichoccurspredom-inantly in females. Clinically, it resembles type I. It develops between the ages of seven and eleven years, starting sub-tly and evolving gradually and progressively. It affects girls more than boys. This type of camptodactyly generally does not improve spontaneously and may evolve to severe flexed deformity.
Type III: This is present from the time of birth. It usually affects several fingers. It is constantlybilateral,withaccentu-ated fixed forms. It is associated with a variety of syndromes and other malformations. 8,12,16,17
The degree of involvement between the hands is often asymmetrical.
In a general manner, the classifications have the aim of grouping different cases of camptodactyly and from this, to establish a treatment protocol.
Main problems and justifications
Several forms of treatment for camptodactyly have already been proposed. Many published studies have emphasized conservative treatment, while others have described surgical procedures.
The problem with this deformity is that several forms of presentation exist, which means that there is no single model for effective treatment (Fig. 1).
Objectives
Theaimof this studywas toretrospectivelyevaluatecases that hadbeen treatedat theHandSurgeryServiceusingapreestab-lishedprotocol, and toconduct acritical analysisonthe results achieved.
Fig. 1 - Camptodactyly in the ring and little fingers.
Materials and methods
Twenty-three patients (40 fingers) who were treated at the Hand Surgery Service, Instituto Nacional de Traumatologia e Ortopedia, Rio de Janeiro, were selected.
All the patients had been treated and followed up by the supervisor in charge of this project and by the co-supervisor of the project since 2004, in conformity with the parameters preestablished in the treatment protocol described below.
We made an initial clinical assessment and divided the cases into reducible forms (flexible) and non-reducible forms (fixed), by means of a physical examination. Among the reducible cases, we divided the patients into two subgroups and proposed the following treatment:
If, through stabilization of the metacarpophalangeal joint, active extension of interphalangeal joint would become pos-sible, we indicated conservative treatment for cases of less than 30 ? of deformity; for deformities greater than 30 ? ,we indicated Zancolli's "lasso" procedure.
If, after correction of the deformity, extension of the inter-phalangeal joint would only be possible passively with flexion of the metacarpophalangeal joint, we instituted con-servative treatment consisting of stretching exercises and useof braces. If conservative treatment failed,wewould indi-cate surgical exploration, in order to search for anomalies in the superficial flexors and/or lumbricals.
Among thenon-reducible cases, i.e. fixed forms,wedivided the patients into three subgroups and proposed the following treatment:
Deformity less than 30 ? : the treatment was limited to observa-tion, stretching exercises and use of a nighttime brace. Deformity between 30 ? and 60 ? : conservative treatment, with continuous use of a brace and monitoring of the evolution of the deformity. In cases of failure, surgical treatment was instituted.
Deformity greater than 60 ? : in these severe cases, in which it was impossible to adequately fit a brace, we indicated surgi-cal treatment, with en-bloc release of the structures of the volar face of the finger (Fig. 2).
We sought to correlate the altered anatomical structures found in the cases that underwent the surgical procedure; and to report the results from both conservative and conservative treatment.
We analyzed the results using the method of Sierget et al.,7 from the Mayo clinic:
Excellent: Full correctionof extension with < 15 ? loss of flexion of the interphalangeal joint. Good: Correction with loss of up to 20 ? of extension and gain of extension of the interphalangeal joint > 40 ? , with loss of flexion < 30 ? .
Fair: Correction with loss of extension of up to 40 ? and gain of extension of the interphalangeal joint >20 ? , with loss of flexion < 45 ? .
Poor: Correctionwithgainof extensionof the interphalangeal joint < 20 ? , with range of motion < 40 ? . 5,7,8,12
Results Twenty-threepatientswereevaluated: 12 females (52.17%) and 11 males (47.82%) (Fig. 3).
Fig. 3 - Distribution between the sexes.
Treze pacientes (56,52%) apresentavam comprometimento bilateral. Quando unilateral, era mais frequente do lado direito (56,5%) (fig. 4).
O dígito mais acometido foi o dedo mínimo, num total de 34 (85%); e em segundo lugar o dedo anular, em cinco (14,6%) (fig. 5).
Dez pacientes foram classificados como Benson tipo I (42,5%), que ficou evidente durante a infância; oito como tipo II (35%), que se desenvolveu entre sete e 11 anos; e apenas quatro pacientes como tipo III (10%), presente desde o nascimento (fig. 6).
Dos 40 dígitos avaliados, 16 eram redutíveis. Com a estabilizac¸ão da metacarpofalangiana, apenasumdígito apresentou uma extensão ativa da IFP com uma deformidade > 30?. Nesse, indicamos o procedimento do lasso descrito por Zancolli. Durante o peroperatório encontramos inserc¸ão anômala dos lumbricais (fig. 7). O resultado foi excelente, com correc¸ão total da extensão.
Os outros 15 dígitos que eram redutíveis só apresentavam a extensão da interfalangiana, com a flexão da metacarpofalangiana passivamente. Nesses, instituímos o tratamento conservador por meio do uso de órteses (figs. 8A e 8B) e exercícios de estiramento.
Em oito dígitos obtivemos um excelente resultado, emdois um resultado bom, com perda de até 20? da extensão e ganho maior do que 40? de extensão na interfalangiana proximal, com perda menor do que 30? de flexão.
Três pacientes abandonaram o tratamento e dois dígitos tiveram um resultado pobre, com correc¸ão com ganho meno
Fig. 4 - Bilateral versus unilateral impairment.
Fig. 5 - Finger most affected.
Fig. 6 - Distribution according to Benson classification.
Thirteen patients (56.52%) were affected bilaterally. When patients were affected unilaterally, this occurred more fre-quently on the right side (56.5%) (Fig. 4).
The finger most affected was the little finger, with a total of 34 cases (85%); and the ring finger was in second place, in five cases (14.6%) (Fig. 5).
Tenpatientswere classified as Benson type I (42.5%), which was evident during infancy; eight as type II (35%), whichdevel-opedbetweentheagesof sevenandelevenyears; andonly four patients as type III (10%), which had been present since birth (Fig. 6).
Out of the 40 fingers evaluated, 16 were reducible.With sta-bilization of the metacarpophalangeal joint, only one finger presented active extension of the interphalangeal joint, with deformity >30 ? . In this case, we indicated Zancolli's "lasso" procedure. During the operation, we encountered an abnor-mality in the lumbricals (Fig. 7). The result was excellent, with full correction of extension.
The other 15 fingers that were reducible only presented extension of the interphalangeal joint, with passive flexion of the metacarpophalangeal joint. In these cases, we insti-tuted conservative treatment using braces (Fig. 8A and B) and stretching exercises.
In eight fingers, we obtained an excellent result and in two, a god result with loss of extension of not more than 20 ? and a gain of extension of the proximal interphalangeal joint of more than 40? , with loss of flexion of less than 30 ? .
Three patients abandoned the treatment and two fingers hadpoor results,withcorrectionproducingagainof extension of the proximal interphalangeal joint of less than 20 ? , with a range of motion of less than 40 ? . In these cases, we indicated surgical exploration. In both cases, we performed Zancolli's procedure. In one case, abnormal insertion of the lumbricals was found. One finger presented an excellent result, with full correctionof extension, and theother evolvedwithscar retrac-tion during the postoperative period.
The other 23 fingerswere irreducible. One presented defor-mity of less than 30? and an excellent result was obtained through conservative treatment.
Fourteen patients presented deformities of between 30 ? and 60 ? . We instituted continuous use of braces and followed up the cases. Eight cases of deformity evolved with excel-lent results; two patients abandoned the treatment; and four evolved with a poor result and surgical exploration was indi-cated. Among these, the volar release technique was used in three cases,whichproducedexcellent results inone case,with loss of flexion of the interphalangeal joint of less than 15 ? and full extension; and good results in two cases, with loss of flexion of the interphalangeal joint of less than 30 ? .
Fig. 7 - "Lasso" procedure.
Fig. 8 - Brace.
During the surgical procedure, we found one case of abnor-mal insertion of the lumbricals and one with a hypoplastic superficial flexor.
Eight fingers presented deformities greater than 60 ? and en-bloc release of the structures of the volar face of the finger was indicated (Figs. 9 and 10).
In three fingers, abnormal insertion of the lumbricals was encountered. We obtained an excellent result in three cases of deformity and a good result in one finger. Three patients abandoned the treatment, even before the surgery, and one abandoned it after the surgical procedure.
Fig. 9 - Volar access.
Fig. 10 - Release of volar structures.
underestimated, because aggressive stretching could cause pain and tissue damage. 5
At a later stage, to avoid recurrence, the brace is used for shorter periods during the day. However, nighttime use is maintaineduntil the end of the skeletal growthperiod.8,12,13,16 Surgical treatment is reserved for specific cases and in cases of failure of conservative treatment. 3,5,7,8,13,16
Bone abnormalities are not a contraindication for surgery, but the result expected will be greatly diminished. 12
The surgical procedures can be described as those that attempt to identify a primary cause; those that attempt to rebalance the interphalangeal joint through transferring flex-ion force to the extensor surface; those that provide en-bloc release of all of the structures of the volar face in order to achieve correction; and bone procedures with dorsal-angle osteotomy of the neck of the proximal phalanx. 3,16
If active extension is possible, with correction of the defor-mityandplacement of themetacarpophalangeal joint inslight flexion, the problemis found in stabilizing the metacarpopha-langeal joint, inanalogy withanulnar claw. These caseswould gain effective benefit from the "lasso" surgical procedure that was described by Zancolli apud Adams 8 and McFarlane et al. 19
If the deformity can only be reduced passively, with place-ment of the wrist or metacarpophalangeal joint in flexion, it can be assumed that the structure responsible for contraction crosses the joints above theflexor surface. Thepossibilities are that the lumbricalmusclehas anabnormal originor insertion, or that the superficial flexor is abnormally fixed.
Surgery to treat camptodactyly, especially incasesof severe contracture, has several complication, such as lesions of neu-rovascular structures, scar tension during extension and loss of flexion.
Incomplete extension is better tolerated than deficient flexion. Early mobilization should be instituted in order to promote restoration of flexion. 8,10
The return of the set of movements of the deep flexor of the fingers and the proximal interphalangeal joint is slow and may take six to twelve months in patients who are treated surgically. 8
Conclusion
According to our observations from outpatient review consul-tations, we concluded that the cases of camptodactyly in the little finger alone, in the flexible form(>60 ? ), whichunderwent surgical treatment in a uniform manner, presented excellent results.
In the rigid forms, our observations indicated that there were benefits relating to gains of extension and correction of the deformity. However, the range of motion with active flexion of the proximal interphalangeal joint was always par-tial, i.e. even in the cases with excellent results, there was an average loss of flexion of 15 ? .
Over time, some cases evolved to present some loss of the gain that hadpreviouslybeenachieved,whichemphasizes the need for continual follow-up monitoring, with systematic use of braces, until the final phase of skeletal growth has been reached.
Conflicts of interest
The authors declare no conflicts of interest.
1. Foucher G, Loréa P, Khouri RK, Medina J, Pivato G. Camptodactyly as a spectrum of congenital deficiencies: a treatment algorithm based on clinical examination. Plast Reconstr Surg. 2006;117(6):1897-905.
2. Ekblom AG, Laurell T, Arner M. Epidemiology of congenital upper limb anomalies in 562 children born in 1997-2007: a total population study from Stockholm, Sweden. J Hand Surg Am. 2010;35(11):1742-54.
3. Medina J, Pajardi G. An algorithm for treatment of camptodactyly based on a review of 135 fingers treated in 109 patients. J Hand Surg Br. 1994;28:36.
4. Minami A, Sakai T. Camptodactyly caused by abnormal insertion and origin of lumbrical muscle. J Hand Surg Br. 1993;18(3):310-1.
5. Rhee SH, Oh WS, Lee HJ, Roh YH, Lee JO, Baek GH. Effect of passive stretching on simple camptodactyly in children younger than three years of age. J Hand Surg Am. 2010;35(11):1768-73.
6. Goldfarb CA. Congenital hand differences. J Hand Surg Am. 2009;34(7):1351-6.
7. Siegert JJ, Cooney WP, Dobyns JH. Management of simple camptodactyly. J Hand Surg Br. 1990;15(2):181-9.
8. Adams BD. Congenital contracture. In: Wolfe SW, Hotchkiss RN, Pederson WC, Kozin SH, editors. Green's operative hand surgery. 6th ed. Philadelphia: Churchill Livingstone/Elsevier; 2011. p. 1443-51.
9. Reichert B, Brenner P, Berger A. Considerations on etiology, correction and treatment of camptodactyly. J Hand Surg Br. 1994;19:9.
10. Smith PJ, Grobbelaar AO. Camptodactyly: a unifying theory and approach to surgical treatment. J Hand Surg Am. 1998;23(1):14-9.
11. Gupta A, Burke FD. Correction of camptodactyly. Preliminary results of extensor indicis transfer. J Hand Surg Br. 1990;15(2):168-70.
12. Scott BD. Camptodactyly and clinodactyly in hand surgery. In: Berger RA, Weiss AP, editors. Hand surgery. Philadelphia: Lippincott Williams & Wilkins; 2004. p. 1478-91.
13. Ogino T, Kato H. Operative findings in camptodactyly of the little finger. J Hand Surg Br. 1992;17(6):661-4.
14. Oberg KC, Feenstra JM, Manske PR, Tonkin MA. Developmental biology and classification of congenital anomalies of the hand and upper extremity. J Hand Surg Am. 2010;35(12):2066-76.
15. Monteiro AV, Almeida SF. Deformidades congênitas do membro superior. In: Pardini AG, Freitas A, editors. Cirurgia da mão: lesões não traumáticas. 2a. ed. Rio de Janeiro: Medbook; 2008. p. 206-8.
16. Glicenstein J, Haddad R, Guero S. Surgical treatment of camptodactyly. Ann Chir Main Memb Super. 1995;14(6):264-71.
17. Benson LS, Waters PM, Kamil NI, Simmons BP, Upton 3rd J. Camptodactyly: classification and results of nonoperative treatment. J Pediatr Orthop. 1994;14(6):814-9.
18. Miura T, Nakamura R, Tamura Y. Long-standing extended dynamic splintage and release of an abnormal restraining structure in camptodactyly. J Hand Surg Br. 1992;17(6):665-72.
19. McFarlane RM, Classen DA, Porte AM, Botz JS. The anatomy and treatment of camptodactyly of the small finger. J Hand Surg Am. 1992;17(1):35-44.
