Crawford Type IV Congenital Pseudarthrosis of the Tibia: Treatment With Vascularized Fibular Grafting and Outcome at Skeletal Maturity. 2021

Tarek A El-Gammal, and Amr El-Sayed, and Mohamed M Kotb, and Waleed R Saleh, and Yasser F Ragheb, and Omar A Refai, and Mohamed M Morsy, and Yousif T El-Gammal
Department of Orthopedics and Traumatology, Reconstructive Microsurgery Unit, Assiut University Hospitals and School of Medicine, Assiut, Egypt.

BACKGROUND Vascularized fibular grafting (VFG) is currently accepted as one of the best treatments for congenital tibial pseudoarthrosis (CPT). However, with longer follow-up, functional results deteriorate, and some problems become evident. METHODS Thirty-nine patients with CPT were treated with VFG. Their age at surgery averaged 5.2 years. Twenty-one cases aged 3 years or less at the time of surgery. All cases were Crawford type IV affected by neurofibromatosis-1. Fourteen patients had 1 to 7 previous surgeries with an average of 4.2 procedures. The length of the fibula used averaged 9.9 cm, and the fibula was fixed by intramedullary wires in 26 cases, and transfixion screws in 11 cases. RESULTS Follow-up averaged 8.1 years. Seventeen cases (43.6%) reached skeletal maturity before the final follow-up. Primary bone union was achieved in 37 cases (96%) after an average of 3.2 months. Stress fracture occurred in 21 cases (51.3%) and was recurrent in 11 cases. Twenty-one cases (53.8%) developed tibial shaft deformities with an average valgus angle of 13.8 degrees and procurvatum angle of 18.8 degrees. The overall final limb length discrepancy (LLD) averaged 2.2 cm. Ipsilateral ankle valgus deformity occurred in 21 cases (53.8%) and averaged 10 degrees. A total of 17 cases required an average of 2 secondary procedures. The tibial deformity did not appear to remodel with age, but the LLD and the angle of ipsilateral ankle valgus deformity continued to significantly increase until skeletal maturity. CONCLUSIONS This study reports the largest single center experience of CPT cases treated with VFG. Free VFG is an excellent treatment option for CPT. Best results are achieved when the procedure is performed at or before the age of 3 years, in the absence of previous surgeries, and with the use of intramedullary fixation. The resulting LLD and the degree of ipsilateral ankle valgus increase with age until skeletal maturity, however, tibial deformity does not remodel and should be corrected at any age if it is excessive. Donor side mild ankle valgus may occur despite sound tibiofibular synostosis. METHODS Level IV.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D009456 Neurofibromatosis 1 An autosomal dominant inherited disorder (with a high frequency of spontaneous mutations) that features developmental changes in the nervous system, muscles, bones, and skin, most notably in tissue derived from the embryonic NEURAL CREST. Multiple hyperpigmented skin lesions and subcutaneous tumors are the hallmark of this disease. Peripheral and central nervous system neoplasms occur frequently, especially OPTIC NERVE GLIOMA and NEUROFIBROSARCOMA. NF1 is caused by mutations which inactivate the NF1 gene (GENES, NEUROFIBROMATOSIS 1) on chromosome 17q. The incidence of learning disabilities is also elevated in this condition. (From Adams et al., Principles of Neurology, 6th ed, pp1014-18) There is overlap of clinical features with NOONAN SYNDROME in a syndrome called neurofibromatosis-Noonan syndrome. Both the PTPN11 and NF1 gene products are involved in the SIGNAL TRANSDUCTION pathway of Ras (RAS PROTEINS). Peripheral Neurofibromatosis,Recklinghausen Disease of Nerve,von Recklinghausen Disease,Cafe-au-Lait Spots with Pulmonic Stenosis,Molluscum Fibrosum,NF1 (Neurofibromatosis 1),Neurofibromatosis I,Neurofibromatosis Type 1,Neurofibromatosis Type I,Neurofibromatosis, Peripheral Type,Neurofibromatosis, Peripheral, NF 1,Neurofibromatosis, Peripheral, NF1,Neurofibromatosis, Type 1,Neurofibromatosis, Type I,Pulmonic Stenosis with Cafe-au-Lait Spots,Recklinghausen Disease, Nerve,Recklinghausen's Disease of Nerve,Recklinghausens Disease of Nerve,Watson Syndrome,von Recklinghausen's Disease,Cafe au Lait Spots with Pulmonic Stenosis,Neurofibromatoses, Peripheral,Neurofibromatoses, Type I,Neurofibromatosis, Peripheral,Peripheral Neurofibromatoses,Pulmonic Stenosis with Cafe au Lait Spots,Syndrome, Watson,Type 1 Neurofibromatosis,Type 1, Neurofibromatosis,Type I Neurofibromatoses,Type I, Neurofibromatosis,von Recklinghausens Disease
D011542 Pseudarthrosis A pathologic entity characterized by deossification of a weight-bearing long bone, followed by bending and pathologic fracture, with inability to form normal BONY CALLUS leading to existence of the "false joint" that gives the condition its name. (Dorland, 27th ed) Pseudoarthrosis,Pseudarthroses,Pseudoarthroses
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005260 Female Females
D005360 Fibula The bone of the lower leg lateral to and smaller than the tibia. In proportion to its length, it is the most slender of the long bones. Fibulas
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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