Description:
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.
Patient Stories
More Information:
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.
Pseudoarthrosis can also occur in other bones such as the radius, ulna and clavicle. The same treatment without the cross union is effective.
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, 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.
