Etiological And Pathological Investigations Of Congenital Pseudarthrosis Of Tibia With A Reference To The New Protocol Of Surgical Treatment

Congenital pseudarthrosis of the tibia (CPT) is a rare disease with its incidence as one in 250,000 newborns. Yet its etiology and pathology have not been universally understood. Its occurrence was considered to be associated with the presence of neurofibromatosis. However, little evidence was described about the lesion pathology in the literature. Because of poor understanding of CPT etiology and pathology, it still remains one of the most difficult conditions to treat in orthopedic surgery. The author carried out the pathological and mmunohistochemical studies on a larger number of CPT specimens. Objective: To investigate its etiology and pathology. These studies were supposed to provide a scientific base for the reasonable protocol of treatment. Methods: Sixty-three specimens were taken from 21 CPT patients, who underwent a surgery in Xi-Jing Hospital between 1982 and 1997. This research project was undertaken by means of macroscopy, microscopy, electronic microscopy and immunohistochemistry. The specimens were taken from (1) CPT periosteum and the surrounding soft tissues; (2) the soft tissues interposed between the CPT bone ends; (3) the bone ends at the lesion. The antibodies, which were used for the immunohistochemical studies, were produced against (1) NF-200 or S-100 protein; (2) Type I or III collagen; (3) PCNA; (4) TGF-(1 and (5) Estrogen...