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CAMPTODACTYLY

Introduction

The word camptodactyly is derived from Greek and translates to mean “bent finger.” In clinical terms, it is used to describe a nontraumatic flexion deformity of the proximal interphalangeal (PIP) joint of the little finger. Camptodactyly describes digital angulation in the anteroposterior plane and is distinct from clinodactyly, which describes a nontraumatic flexion deformity with angulation in the radioulnar plane. Camptodactyly usually shows no improvement or gradually progresses unless treated. Progression will generally stop at the end of growth.4  Benson proposed three categories of camptodactyly: (I) congenital (infant), which appears in infancy and affects boys and girls equally, (II) pre-adolescence (acquired), which appears between age 7 and 11, is progressive and affects mostly pre-teen girls, and (III) syndromic, which involves multiple digits on both hands and can be associated with a variety of syndromes.1

Camptodactyly commonly involves the ulnar PIP joints, especially the PIP joints of the little finger.2 The cause of Camptodactyly is unknown but this deformity represents a combination of PIP joint anomalies secondary to an imbalance of the flexor and extensor tendon systems.

The incidence of Camptodactyly is difficult to assess, but the occurrence has been reported at less than 1%.3 The age of presentation it Is bimodal with the majority of these deformities presenting in infants and young children or in teenagers.2

The differential diagnosis includes:2

  1. Locked trigger fingers
  2. Boutonnière deformities from unrecognized injuries
  3. Dupuytren’s contractures
  4.  Hypoplastic extensor tendons
  5. Delta phalanx
  6. Clinodactyly

Camptodactyly may also be part of a syndrome complex like:

  1. Arthrogryposis
  2. Mucopolysaccharidoses
  3. Marfan’s syndrome
  4. Juvenile rheumatoid arthritis
  5. Scleroderma
  6. Trisomy 123
  7. Congenital collagen disorders
  8. and many others2
ICD-10 Codes

  • CAMPTODACTYLY

    Diagnostic Guide Name

    CAMPTODACTYLY

    ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

    DIAGNOSISSINGLE CODE ONLYLEFTRIGHTBILATERAL (If Available)
    CAMPTODACTYLYQ68.1   

    ICD-10 Reference

    Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
  • Bilateral Fifth Finger Camptodactyly
    Bilateral Fifth Finger Camptodactyly
  • Bilateral Fifth Finger Camptodactyly
    Bilateral Fifth Finger Camptodactyly
  • Camptodactyly right fifth finger
    Camptodactyly right fifth finger
  • Camptodactyly left fifth finger
    Camptodactyly left fifth finger
Symptoms
Bilateral fifth finger deformities with cosmetic concerns
Family history often positive
Deformities often noted at the time of growth spurts
Functional complaints relating to keyboarding, musical instruments, etc
Typical History

A typical patient in the older group is often a teenage female complaining of deformities in both little fingers. The patient is usually experiencing a growth spurt. The cosmetic aspect of the deformity is frequent the primary concern of the patient.  The patient may also complain about difficulty using a keyboard, playing a particular musical instrument, putting on gloves or certain athletic activities. Some parents are vigorously requesting that the deformities be “fixed!”

Positive Tests, Exams or Signs
Work-up Options
Images (X-Ray, MRI, etc.)
  • Lateral X-ray Camptodactyly right fifth finger. Note small head of proximal phalanx and narrow sclerotic neck. (arrow).
    Lateral X-ray Camptodactyly right fifth finger. Note small head of proximal phalanx and narrow sclerotic neck. (arrow).
  • AP X-ray Camptodactyly right fifth finger.
    AP X-ray Camptodactyly right fifth finger.
  • Lateral X-ray Camptodactyly left fifth finger.
    Lateral X-ray Camptodactyly left fifth finger.
  • AP X-ray Camptodactyly left fifth finger.
    AP X-ray Camptodactyly left fifth finger.
  • Fifth finger camptodactyly oblique X-ray
    Fifth finger camptodactyly oblique X-ray
Treatment Options
Treatment Goals
  • Decrease the deformity
  • Accept the deformity
  • Controll pain if present
Conservative
  • Advise patient and family to accept the deformity
  • Passive stretching exercises especially for young patients and young patients with multiple digits involved
  • Static and dynamic splinting or serial plaster casts should be attempted before surgery
Operative
  • Palmar fascia release (up to age 4 y)
  • Fexor tendon release
  • Tendon transfer (aged 13–19 y)
  • Lumbrical release or excision
  • Soft tissue distraction lengthening 
  • Osteotomy of the neck of the proximal phalanx (older patients and those with bony changes)
  • PIP arthrodesis especially for older patients and those with joint pain
Hand Therapy
  • Stretching exercises
  • Night extension splinting
  • Dynamic splinting
Complications
  • Complications genrally are related to surgery
     
  • Neurovascular damage especially if the PIP joint is pinned in extension without checking capillary refill
  • Skin loss
  • Tendon exposure
  • Decrease in range of motion (flexion in PIP) owing to scar formation within the tendon sheath or ligaments of the PIP joint.
Outcomes
  • Conservative treatment with static and dynamic splinting will improve mild deformities and prevent some moderate deformities from progressing.
  • Surgical treatment can improve the deformity and range of motion but the result can be no change and in some cases a worsening of the deformity and/or motion.
Video
The Bouvier Manuever suggests that the central slip is intact and that the PIP joint may be held in flexion by an abnormal insertion of the lumbrical or the FDS tendon.
Key Educational Points
  • Infants and young children comprise seventy-five to eighty percent of the patients with camptodactyly. The rest of the patients are usually adolescents. 
  • Adolescents often present with the complaint that their deformity is worsening. Some camptodactyly contractures do worsen in this age group but some flexion contractures are actually unchanged but appear worse because the involved digit(s) are experiencing a growth spurt making the digit longer which makes the unchanged contracture more evident.  Additional, the increased awareness of self-image may play a role in this age group. 
  • In camptodactyly X-ray changes are best seen in the lateral view. Radiographic abnormalities include bony erosions, proximal phalanx head deformity (flattening or hypoplasia), and/or angulation of the neck of the proximal phalanx.
  • Camptodactyly inheritance follows an autosomal dominant with incomplete penetrance inheritance pattern.  The majority occur bilaterally.  
  • In adolescent camptodactyly soft tissue surgery may be an option if the PIP flexion contracture can be corrected fully and actively by keeping the MP joint in flexion especially if an MRI can identify a specific abnormality like an abnormal lumbrical or flexor digitorum superficialis insertion.
  • If a simple correctable tendon imbalance can be identified or discovered at surgery, the soft tissue procedures may work.  Typical the surgical plan will be a combined lumbrical release or excision with a FDS release and transfer.  The patient, parents and surgeon must accept the fact that the surgical outcome may not always be ideal despite technically excellent surgery.
  • If the PIP contracture is greater than 60 degrees or if there are bony abnormalities, then soft tissue procedures are unlikely to help. If the PIP joint is painful then arthrodesis should be recommended, and its limitations thoroughly review during patient and parent education.
  • Surgical treatment can produce unpredictable results.  Recommended procedures may improve the deformity, cause no change, or worsen the problem.  Therefore, non-operative treatment should always be tried first and accepting the deformity is often wise.
References

Cited

  1. Benson LS, et al. Camptodactyly: classification and results of nonoperative treatment. J Pediatr Orthop 1994;14(6):814-9. PMID: 7814601
  2. Engber WD and Flatt AE. Camptodactyly: an analysis of sixty-six patients and twenty-four operations. J Hand Surg 1977: 2A(#3): 216-224. PMID: 864219
  3. Jones KG, Marmour L, Lankford LL. An overview on new procedures in hand surgery.  Clin Orthop1974: 99:154-167.  PMID: 4368405  
  4. 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 2010: 35A: 1768-1773. PMID: 21050962

New Articles

  1. Hamilton KL, Netscher DT. Evaluation of a stepwise approach to camptodactyly. Plast Reconstr Surg 2015;135(3):568e-576e. PMID: 25719721
  2. Kakinoki R, et al. Simultaneous reconstruction of a palmar skin defect and the digital artery with an arterialized venous flap after correction of camptodactyly with severe flexion deformity. Hand (N Y) 2011;6(4):445-9. PMID: 23204975

Reviews

  1. Ty JM, James MA. Failure of differentiation: Part II (arthrogryposis, camptodactyly, clinodactyly, madelung deformity, trigger finger, and trigger thumb). Hand Clin 2009;25(2):195-213. PMID: 19380060
  2. McFarlane RM, et al. The anatomy and treatment of camptodactyly of the small finger. J Hand Surg Am 1992;17(1):35-44. PMID: 1538110

Classics

  1. Schaff B, Schafer PW. Camptodactyly. Arch Surg 1948;57(5):633-6. PMID: 18107182
  2. 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(3):266-74. PMID: 5345097
  3. Engber WD and Flatt AE. Camptodactyly: an analysis of sixty-six patients and twenty-four operations. J Hand Surg 1977: 2A(#3): 216-224. PMID: 864219
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