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Treatment of Congenital Pseudarthrosis of the Forearm (CPF) with Vascularized Fibular Graft(VFG). Evaluation of the Results After a Minimum 15 Years of Follow-up - Cohort Study
Rames Mattar Junior, MD, PhD; Tiago Guedes da Motta Mattar, MD; Emygdio Jose Leomil Paula, MD, PhD; Marcelo Rosa Rezende, MD, PhD; Teng Hsiang Wei, MD, PhD; Luciano Ruiz Torres, MD; Luiz Koiti Kimura, MD; Mateus Saito, MD
Department of Orthopedic - Hand and Microsurgery Division, University of Sao Paulo, Sao Paulo, Brazil
Introduction: According Beris et al only 18 patients with CPF were described by the English literature. Moore in 1949 reported a patient with CPF for the first time. The association of Congenital Pseudarthrosis with neurofibromatosis was noted by Gol in 1918. The etiology is still unknown. Patients with CPF present instability, deformity and shortening of the forearm and disability of the upper limb.
Objective: The objective of this study is to present the results of the treatment of 6 patients with CPF with VFG with a minimum 15 years of follow-up.
Material and Methods: Since 1986 6 patients with CPF were treated with VFG, 3 affecting both bones of the forearm and 3 only the radius, 5 male and 1 female. The age at the moment of the surgery ranged from 5 to 11 with an average of 7,7 years old. Surgical procedures occurred between 1986 to 1995 and the follow-up ranged from 15 to 26 years with an average of 17,7 years. All patients presented signs of neurofibromatosis as café-au-lait stains and family history. Patients with CPF affecting only the radius presented deformity similar to congenital radial club hand. Those with disease in both forearm bones presented severe shortening and instability. One patient had 3 previous conventional surgeries without success. Bone shortening previous to surgery ranged from 3 to 10 cm with an average of 5,8 cm. In 3 patients we performed an angiography before surgery. Surgical technique included resection of al pathological tissue and reconstruction with VFG. In two patients with severe shortening the VFG was fixed proximally to the ulna and distally to the radius.
Results: Bone integration and consolidation of the VFG were obtained in all patients. Time for consolidation ranged from 4 to 5 months. Pathological study of the tissue removed revealed fibrous tissue poorly vascularized with myxoid degeneration and inflammatory infiltration. Bone stabilization permitted improvement of the upper limb function. Residual shortening ranged from 2 to 8 cm with an average of 4,5 cm. After a minimum of 15 year of follow-ups all patients use the upper limb for daily life activities.
Discussion: There are few reports about this rare disease. Conventional treatments based in regular cortical cancellous bone graft and bone fixation. The evaluation of the literature and the experience to treat these patients determine that VFG is the best option to treat this rare disease. Parte superior do formulário
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