Updated: 5/22/2021

Macrodactyly (local gigantism)

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  • Introduction
    • Macrodactyly is nonhereditary congenital digit enlargement.
  • Epidemiology
    • Incidence
      • very rare
    • Anatomic 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
    • Risk factors
      • none known
  • Etiology
    • 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
  • Classification
    • Functional Classification
      Static
      Present at birth and growth is linear with other digits
      Progressive
      Not as noticeable 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
  • Prognosis
    • If static, asymmetry does not worsen
    • If progressive, asymmetry worsens with time

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(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?

QID: 601
FIGURES:
1

Epiphysiodesis now

15%

(470/3232)

2

Epiphysiodesis when the finger reaches adult length of the father

76%

(2454/3232)

3

Compression wrapping until proportional size is achieved

1%

(38/3232)

4

Resection of hypertrophic nerves

3%

(95/3232)

5

Osteotomy at skeletal maturity.

5%

(158/3232)

L 2 D

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