Table 7.

Treatment of Manifestations in Individuals with Classic Congenital Contractural Arachnodactyly

Manifestation/
Concern		TreatmentConsiderations/Other
Musculoskeletal By orthopedist
  • Contractures may require surgical release.
  • Clubfeet may require casting.
  • Progressive kyphoscoliosis may require bracing &/or surgical intervention.
By physiatrist, OT/PT
  • Early PT in children helps ↑ joint mobility & counteract muscle hypoplasia (usually calf muscles).
  • Camptodactyly & large-joint contractures may spontaneously improve over time.
  • Swimming reinforces the musculature w/out taxing joints.
  • Cycling may benefit those w/patellar hypermobility by ↓ risk for patellar luxation.
Cardiovascular By cardiologist/
cardiovascular surgeon		Aortic root dilatation is managed in a standard manner. See Marfan Syndrome & Milewicz et al [2005] (full text).
Ophthalmologic By ophthalmologist
  • Correction of refractive errors
  • Keratoconus can be treated by scleral contact lenses. It is currently unknown if corneal crosslinking is safe &/or useful in CCA.
Orthodontic By orthodontist/dentist
  • Use of palatal expander may be indicated.
  • Dental crowding may necessitate extraction of molars.

OT = occupational therapy; PT = physical therapy

From: Congenital Contractural Arachnodactyly

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