Congenital Pseudarthrosis of the Tibia

A deformed and/or non-healing tibia bone, often associated with Neurofibromatosis type 1 (NF1).

My Approach

Congenital Pseudarthrosis of the tibia (CPT) is a rare disorder in which a child is born with a specific bowing of the tibia (anterolateral) and/or a break of the tibia at birth. I firmly believe that every limb with this disorder can be treated, healed and not re-fracture by utilizing a Cross Union Technique. As a result, this condition should never undergo amputation. During the over twenty years I have been in practice, I have seen this condition evolve from one with a nearly 30-40% amputation rate to now where amputation should be a never occurrence.



From My Patients

More Information on Congenital Pseudarthrosis of the Tibia

CPT, which causes a deformity of the leg, often shortening of the leg and a non-healing fracture, has been troublesome for surgeons to treat. Oftentimes patients have undergone multiple procedures in childhood which has left the leg quite damaged and the ankle not functional. As a result, amputation is offered to the families of these children. This has all changed over the past several years with combining treatment in the Cross Union Technique.



Cause

Most (>80%) children with congenital pseudarthrosis of the tibia suffer from Neurofibromatosis Type 1.



Diagnosis Methods

CPT may be diagnosed on plain x-rays based on a very specific deformity of the tibia. NF1 may be diagnosed with a genetic blood test.



Treatments

Utilizing pre-surgical Zolendronic Acid intravenously, combined with a surgery that includes: excising all the tissue causing the non-healing bone, freshening the bone edges, placing a very large bone graft from the patient’s pelvis (a unique and difficult procedure) between the tibia and fibula, utilizing Bone Morphogenic protein, and a periosteal graft from the pelvic lining combined with a growing rod in the bone and a plate to hold it for 6 months. All of this has allowed for complete healing of the tibia in this population. The surgery also allows the ankle to move and preserves the ankle function.



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