Camptodactyly - observation

Historical Overview

• Examination of the extensor tendons is a long-established component of the routine clinical examination of the hand.  The exam is used in the diagnosis of camptodactyly, as well as Kirners deformity¹, Dupuytren’s disease,² certain injuries to the digits,³ rheumatoid arthritis, and a of other diseases, disorders, and conditions.⁴
• The word, camptodactyly is derived from the Greek words “kampto,” which means to bend, and “daktylos,” which means finger. 
• The term “camptodactyly” has been attributed to Louis Landouzy who, in 1906, described a variety of constitutionally crooked digits,⁵ and to RW Tamplin who, in 1846, used the term to describe a flexion deformity of the proximal interphalangeal (PIP) joint of the little finger in an antero-posterior direction.⁶
• Camptodactyly, meaning bent finger, is a congenital flexion deformity of the proximal PIP joint,³ typically of the little finger.

Description

• Camptodactyly, meaning bent finger, is a congenital flexion deformity of the proximal PIP joint,³ typically of the little finger.  Although the term was originally used to describe contracture of the little finger, it is now used to describe the deformity in all fingers.⁷
• As noted camptodactyly is diagnosed primarily by observation of the patient’s little fingers. To compensate for the flexion contracture of the proximal interphalangeal (PIP) joint, the patient’s metacarpophalangeal (MP) joint is usually held in hyperextension.

Pathophysiology

• Campyodactyly can arise from absent extensor tendon function, skin contracture, volar plate anomalies, and articular incontruity.⁸
• It is a congenital deformity of the PIP joint.² Camptodactyly may be sporadic or familial. When it is familial, it follows an autosomal dominant pattern of inherentance.⁵
• Camptodactyly has been associated with ≥70 syndromes² including Trisomy 123, oculodentodigital syndrome, orofaciodigital syndrome, and Aarskog’s syndrome.¹¹
• Camptodactyly may be congenital (type I), acquired (type II), or syndromic (type III).¹⁰ Type II camptodactyly is often observed in girls between the ages of 7 and 11.

Instructions

• Camptodactyly is diagnosed by OBSERVATION. This diagnosis is confirmed by completing a history, physical examination and by performing imaging studies if appropriate but careful observation is the basis of the camptodactyly diagnosis.
• The characteristic lesion is a flexion contracture of the proximal interphalangeal joint⁵
• It almost always affects the little finger^1,2,5
• It is typically painless¹
• Sensory or motor deficits are usually not associated with camptodactyly¹
• There is normal capillary refill of the affected digit¹
• Swelling, erythema, and warmth are not associated with camptodactyly (ie., it is not associated with infection)¹
• Camptodactyly is not associated with trauma⁹

Variations⁵

• The degree of flexion may vary from a very slight deformity to an almost right-angled bend; it is seldom more than this
• The fourth to second fingers, in decreasing order of frequency, may be affected similarly
• The extent of the deformity is progressively less in the more radial fingers
• Often there is hyperextension of the metacarpophalangeal joints of the affected fingers
• Occasionally the proximal interphalangeal joints of some fingers are also hyperextended
• Rarely there is flexion deformity of the terminal interphalangeal joints as well
• Involvement of the thumb is unusual
• In advanced cases, there is ulnar deviation of the affected fingers, with radial rotation of the fifth finger, and the palm becomes cup-shaped

Related Signs and Tests

• Fingers can be bent at the PIP joint due to a variety of diseases, disorders, and conditions, including Kirners deformity¹, Dupuytren’s disease,² injury,³ and rheumatoid arthritis⁴

Diagnostic Performance Characteristics

• The findings of the history and physical define the condition. Therefore, a condition that meets the criteria described in the “Instructions” section, by definition, has a sensitivity (true positive rate; proportion of actual positives that are correctly identified as such) and specificity (true negative rate; proportion of actual negatives that are correctly identified as such) of 100%
• The ability of individual clinicians to apply these criteria will alter the sensitivity and specificity of this diagnostic exam

• A positive result occurs whenthe patient’s little finger shows flexion contracture of the PIP joint and hyperextension of the MP joint.

• A negative result occurs when the patient’s little finger does not show flexion contracture of the PIP joint and hyperextension of the MP joint.

• Because camptodactyly usually affects only the little finger, it often goes unnoticed¹
• Camptodactyly can present in adolescents, as well as infants¹
• It is only rarely associated with significant functional deficit¹
• Operative procedures may improve the camptodactyly deformity, may cause no change, or may worsen the deformity. Therefore, non-operative treatment should always be tried first.¹⁰
• For infants and young children, passive stretching can improve the flexion deformity associated with camptodactyly.¹³
• If surgery is needed, a lateral stiletto-shaped transposition flap with flexor digitorum superficialis tendon release may improve finger aesthetics and function.¹²

1. Frank R. Campodactyly, Orthobullets. Available at http://www.orthobullets.com/hand/6074/camptodactyly. Accessed January 20, 2015
2. Doyle JR. Hand and Wrist (Orthopaedic Surgery Essentials Series). Philadelphia: Lippincott Williams & Wilkins, 2006.
3. Rayan GM and Akelman E. The Hand: Anatomy, Examination, and Diagnosis. Philadelphia: Lippincott Williams & Wilkins, 2012
4. Culp R, Jacoby SM. Musculoskeletal Examination of the Elbow, Wrist, and Hand: Making the Complex Simple. Thorofare: Slack Inc., 2012
5. Gordon H, Davies D, Berman M. Camptodactyly, cleft palate and club foot. A syndrome showing the autosomal-dominant pattern of inheritance. J Med Genet 1969; 6: 266-274. PMID: 5345097
6. Smith P, Grobbelaar A. Camptodactyly: A Unifying theory and approach to surgical treatment. J Hand Surg Am 1998;23:14-19. PMID: 9523948
7. Swanson A, Swanson G, Tada K. A Classification for congenital limb malformation. J Hand Surg Am 1983;8(5 Pt 2):693-702. PMID 6630951
8. eORIF. Campodactyly, available at http://eorif.com/camptodactyly.
9. Siegert J, Cooney W, Dobyn J. Management of simple camptodactyly. J Hand Surg Br 1990;15:181-9. PMID: 2366018
10. Benson LS, Waters PM, Kamil NI, et al. Camptodactyly: classification and results of nonoperative treatment. J PediatrOrthop1994;14(6):814-9. PMID: 7814601
11. Engber WD, Flatt AE. Camptodactyly: an analysis of sixty-six patients and twenty-four operations. J Hand Surg Am1977;2(3):216-24. PMID: 864219
12. Wall LB, Ezaki M, Goldfarb CA. Camptodactyly treatment for the lesser digits. J Hand Surg Am2018;43(9):874. PMID: 29691077
13. Rhee SH, Oh WS, Lee HJ, et al. Effect of passive stretching on simple camptodactyly in children younger than three years of age. J Hand Surg Am2010;35(11):1768-73. PMID:21050962