Congenital Pseudarthrosis of the Tibia

DESCRIPTION

Congenital pseudarthrosis of the tibia (CPT) is a rare condition that usually occurs in the first two years of life and is characterized by anterolateral bowing of the tibia that can lead to a fracture that fails to reunite.

CAUSES

Congenital pseudarthrosis of the tibia has been linked to Type 1 neurofibromatosis but the exact cause of CPT is unknown.

SYMPTOMS

The most common symptom of congenital pseudarthrosis of the tibia is progressive bowing of the tibia that is visible at birth or becomes visible in early childhood.

DIAGNOSIS

It is most ideal to diagnose congenital pseudarthrosis of the tibia while the bone is bowing but before the fracture occurs. Early diagnosis increases the possibility of successfully halting and correcting the bowing of the tibia while decreasing the risk of a fracture that will fail to unite. Bowing of the tibia can be found during clinical examinations in the first week of a child’s life. X-rays and MRI scans should be ordered to provide detailed analysis of the tibia and soft tissues.

TREATMENT

Congenital pseudarthrosis of the tibia can be a challenging condition to treat as it poses a major problem in achieving and maintaining union of the tibia. Children with CPT may have poor healing ability and attempts to unite their small bone fragments can cause damage to the tibia and/or ankle joint.

If diagnosed before the child begins to walk and also before a fracture occurs, it may be possible to protect the tibia with an orthosis brace until the child reaches skeletal maturity. However, surgical intervention is required if the fracture has already occurred and fails to heal as bracing alone would be ineffective.

The main goal of surgery is to achieve union of the tibia, correct alignment of the leg, and decrease the risk of additional fractures. CPT fractures in children between the ages of 2 to 4 years are most effectively treated with an external fixator that stabilizes or compresses the fractured tibia. Given the nature of CPT, a child with a fracture may have to undergo multiple procedures to achieve long-lasting union of the tibia.

SOURCES

  1. EJ-Rosasy, M., Paley, D., & Herzenberg, J. (n.d.). Congenital Pseudarthrosis of the Tibia. Limblengtheningdoc.org. Retrieved July 25, 2014, from http://limblengtheningdoc.org/congenital_pseudarthrosis_of_the_tibia_34.pdf
  2. Congenital Pseudarthrosis. (2009, July). Nfauk.org. Retrieved July 25, 2014, from http://www.nfauk.org/assets/downloads/NF1%20and%20Pseudarthrosis.pdf
  3. Congenital Pseudarthrosis of the Tibia. (2011, Nov) Orthopaedics & Traumatology: Surgery & Research, 97(7), 750–761. Retrieved July 25, 2014, from http://www.sciencedirect.com/science/article/pii/S1877056811001885
  4. Congenital Pseudarthrosis of the Tibia: Management and Complications. (2012, Nov-Dec) Indian Journal of Orthopaedics, 46(6), 616–626. Retrieved July 25, 2014, from http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3543877/
  5. Vander Have, K., Hensinger, R., Caird, M., Johnston, C., & Farley, F. (2008, April) Congenital Pseudarthrosis of the Tibia. The Journal of American Academy of Orthopaedic Surgeons, 16(4), 228-236. Retrieved July 25, 2014, from http://www.jaaos.org/content/16/4/228.full
  6. Canale, S., & and Beaty, J. (2013) Congenital Pseudarthrosis of the Fibula and Tibia. Campbell’s Operative Orthopaedics, 12(1), Retrieved July 25, 2014, from https://www.inkling.com/read/campbells-operative-orthopaedics-canale-beaty-12th/chapter-29/congenital-pseudarthrosis-of