Appendix
← Clinical Reader
Orthopedics

Congenital Tibial Pseudarthrosis

Congenital pseudarthrosis of the tibia is a rare dysplastic pathological condition of the lower limbs leading to significant disability. The etiology of this condition is not clear, but an association has been seen with neurofibromatosis type 1. It may be seen at birth or manifest at the walking age with pathological fractures leading to deformity and limb length discrepancy. The treatment of this condition is mainly surgical, with various options aimed at achieving bone union, correcting limb d

v1 · Reviewed Nov 7, 2026 by Automated Clinical Reviewer (Strict Verbatim v1)
  • Congenital pseudarthrosis of the tibia is a rare dysplastic pathological condition of the lower limbs leading to significant disability.
  • It may be seen at birth or manifest at the walking age with pathological fractures leading to deformity and limb length discrepancy.
  • Patients with congenital pseudarthrosis of the tibia should be promptly evaluated and adequately treated, as it can lead to significant long-term disability when neglected.
  • Standard roentgenogram should be advised as the primary investigation, which can show anterolateral bowing, heterogeneous lesions, resorption of bone at the ends of the fracture responsible for significant loss of bone substance.
  • In cystic forms bowing appears before the first year, the medullary canal is narrow, and deformity gradually worsens, ultimately resulting in transverse fracture.
  • In dysplastic forms bowing can be seen since birth, narrow tibia with an hourglass appearance, and partially or completely obstructed medullary cavity.
  • The fibula is commonly affected in such forms.
  • The role of magnetic resonance imaging (MRI) has recently emerged as it helps provide a more detailed analysis of soft tissue lesions, mainly the periosteum, which lies in the vicinity of pseudarthrosis.
  • Bone perfusion sequences in MRI can help visualize the vascularization defects in the bone, which ultimately helps in finalizing the limits of resection preoperatively.
  • A multimodality approach including genetic counseling is needed to screen for neurofibromatosis in all patients diagnosed with CPT.
  • A complete dermatological and neurological examination is also done in cases associated with NF-1, which is an autosomal dominant, multisystemic neurocutaneous disease.
Crawford classification
Type 1: Presence of anterior bowing along with increased cortical density and narrow medulla Type 2: Presence of anterior bowing with sclerotic bone and narrow medulla Type 3: Presence of anterior bowing along with cyst or pre-fracture signs Type 4: Presence of anterior bowing with an evident fracture along with pseudoarthrosis of tibia and fibula
  • The management of congenital pseudarthrosis of the tibia is mainly surgical as with the progression of time, and it leads to more worsening, shortening, and deformity of the condition.
  • The role of prophylactic bracing in a child who has just started walking can help delay or limit the deformity associated with this condition.
  • The goal of surgical management is restricted to achieving the union in cases of CPT and includes restoring the limb's satisfactory alignment and correcting the limb length discrepancies while preserving the normal articular function.
  • Various surgical techniques described in the literature include intramedullary nailing along with a bone graft, Ilizarov technique, and vascularized fibular transfer.
  • Multiple treatment options are available for this disease, including both operative and non-operative options based on the severity of the condition.
  • Recent advances in the management of congenital pseudarthrosis of the tibia include the use of bone morphogenic proteins, induced membrane and spongy autologous grafts, periosteal grafts, and electrostimulation.
  • The contralateral fibula is used classically for this procedure, although an ipsilateral fibula can also be used.
  • The common complications associated with this condition include recurrent fractures, limb length discrepancies, valgus deformity of the ankle, tibiotarsal instability, and ankle stiffness.
  • The prognostic factors associated with poor prognosis include significant limb length discrepancy, atrophy of the bone ends, narrow medullary canal with extensive sclerosis, and involvement of the fibular bone and tibial bowing.
  • Consultation with a pediatrician is required to evaluate the other manifestations of neurofibromatosis type 1, which is often associated with this disease.
  • Also, dermatology referral is necessary for the evaluation of skin manifestations.
  • The role of physical therapy and rehabilitation cannot be understated in getting the best functional outcomes; hence physiotherapists should be informed about the rehab protocol after each case.
  • Interprofessional healthcare teamwork with open communication and collaborative action are crucial to successful patient outcomes.
  • review
    Congenital Tibial Pseudarthrosis. StatPearls [Internet] — Udit Agrawal, Vivek Tiwari — StatPearls Publishing, 2023

Decision support only. Verify against local protocols and current labeling. Source: www.ncbi.nlm.nih.gov