Updated: 10/12/2016

Macrodactyly (local gigantism)

Topic
Review Topic
0
0
Questions
1
0
0
Evidence
2
0
0
https://upload.orthobullets.com/topic/6080/images/1.jpg
https://upload.orthobullets.com/topic/6080/images/macrodactyly.jpg
https://upload.orthobullets.com/topic/6080/images/f21.large.jpg
Introduction
  • Nonhereditary congenital digit enlargement
  • Epidemiology
    • demographics
      • very rare
    • location
      • 90% are unilateral
      • 70% involves more than one digit 
      • index involved most frequently
      • in order of decreasing frequency, the long finger, thumb, ring, and small are also involved
      • can involve digits of the hand or foot
    • 70% involves more than one digit
      index involved most frequently
      in order of decreasing frequency, the long finger, thumb, ring, and small are also involved
      can involve digits of the hand or foot

      \
      90% are unilateral
    • risk factors
      • none known
  • Pathophysiology 
    • etiology unknown
    • no genetic correlations known to date
    • affected digits correspond with neurologic innervation
      • the median nerve being the most common
  • Associated conditions
    • lipfibromatous hamartoma of the median nerve is the adult homolog
    • has been associated with:
      • Proteus syndrome
      • Banayan-Riley-Ruvalcabe's disease
      • Maffucci syndrome
      • Ollier’s disease
      • Milroy’s disease 
  • Prognosis 
    • if static, asymmetry does not worsen
    • if progressive, asymmetry worsens with time
Classification
 
Functional Classification
Static  Present at birth and growth is linear with other digits
Progressive  Not as noticable at birth but shows disproportionate growth over time 
 
Presentation
  • History
    • asymmetry to digits can be present at birth or appearing over time
  • Symptoms
    • pain
    • inability to use digits
    • complaints of cosmetic issues
  • Physical exam 
    • inspection & palpation
      • thick, fibrofatty tissue involving enlarged digits
    • ROM & instability
      • often limited ROM due to soft tissue constraints
Imaging
  • Radiographs 
    • recommended views
      • biplanar hand radiographs
    • findings
      • enlarged phalanges to involved digits  
      • may see malalignment of joints or angled phalanges
  • CT, MRI
    • not typically needed
Studies
  • Angiography
    • only needed if used for surgical planning
Treatment
  • Nonoperative
    • observation
      • in mild cases
  • Operative
    • epiphysiodesis   
      • indications
        •  single digit
        • perform once digit reaches adult length of same sex parent
        • most common approach
      • postoperative care
        • soft tissue care
        • early ROM
    • osteotomies and shortening procedures
      • indications
        • thumb involvement
        • multiple digit involvement
        • severe deformity
      • postoperative care
        • local soft tissue care
        • early ROM
    • amputations
      • indications
        • severe involvement of digit
        • non-reconstructable digit
Complications
  • Digital stiffness
  • Chronic digital pain or edema
 

Please rate topic.

Average 4.6 of 17 Ratings

Thank you for rating! Please vote below and help us build the most advanced adaptive learning platform in medicine

The complexity of this topic is appropriate for?
How important is this topic for board examinations?
How important is this topic for clinical practice?
Questions (1)

(OBQ08.215) An 8-year-old boy's parents are concerned about the appearance of the child's middle finger. The child denies pain and his digital neurovascular status is normal. A clinical photograph and radiograph are provided in figures A and B. For children with this condition, which of the following is the best intervention to achieve a finger that is proportional to the rest of the hand? Review Topic

QID: 601
FIGURES:
1

Epiphysiodesis now

14%

(379/2660)

2

Epiphysiodesis when the finger reaches adult length of the father

76%

(2026/2660)

3

Compression wrapping until proportional size is achieved

1%

(28/2660)

4

Resection of hypertrophic nerves

3%

(82/2660)

5

Osteotomy at skeletal maturity.

5%

(130/2660)

ML 2

Select Answer to see Preferred Response

PREFERRED RESPONSE 2
ARTICLES (2)
Topic COMMENTS (1)
Private Note