Ilizarov bone transport as a treatment of congenital pseudarthrosis of the tibia: a long-term follow-up study

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Abstract

Purpose

Most studies on congenital pseudarthrosis of the tibia (CPT) report on the short-term union rate and refracture rate but do not take into account the long-term outcome. This review includes patients treated with an Ilizarov bone transport, who all reached skeletal maturity. It describes long-term results and highlights any prognostic factors that could predict the final outcome.

Methods

The records of patients with CPT treated with an Ilizarov bone transport in our institution were retrospectively evaluated.

Results

A total of 12 consecutive patients were studied. The mean follow-up was 24.5 years (range 6–39 years). Primary consolidation was seen in ten patients (83 %). Half of these patients had a refracture. At final follow-up, eight patients experienced union and four remained un-united, of whom one had an amputation.

Conclusions

The present data confirm a good primary healing rate. However, tibial union at final follow-up was only seen in 67 %, indicating that refracture is the main issue. United bone is often of inferior biological and mechanical quality, so lifetime protection with intramedullary devices, braces or a combination of both is recommended.

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Most cited references 27

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Neurofibromatosis update.

Alvin Crawford, Elizabeth Schorry (2015)

Type 1 neurofibromatosis (NF-1), also known as von Recklinghausen disease, is one of the most common human single-gene disorders, affecting at least 1 million persons throughout the world. It encompasses a spectrum of multifacted disorders and may present with a wide range of clinical manifestations, including abnormalities of the skin, nervous tissue, bones, and soft tissues. The condition can be conclusively diagnosed when 2 of 7 criteria established by the National Institutes of Health Consensus Development Conference are met. Most children with NF-1 have no major orthopedic problems. For those with musculoskeletal involvement, the most important issue is early recognition. Spinal deformity, congenital tibial dysplasia (congenital bowing and pseudarthrosis), and disorders of excessive bone and soft-tissue growth are the three types of musculoskeletal manifestitations that require evaluation. Statistics gathered from the Cincinnati Children's Hospital Neurofibromatosis Center database of 588 patients show the incidence of spinal deformity in children with NF-1 to be 21%; pectus deformity, 4.3%; limb-length inequality, 7.1%; congenital tibial dysplasia, 5%; hemihypertrophy, 1.4%; and plexiform neurofibromas, 25%. The orthopedic complications can be managed, but only rarely are they cured. Current developments in molecular genetics are exciting and give hope to more positive outcomes.

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Treatment approaches for congenital pseudarthrosis of tibia: results of the EPOS multicenter study. European Paediatric Orthopaedic Society (EPOS).

C. Tudisco, J Fixsen, B Romanus … (2000)

This study was designed to analyze the different therapeutic methods used by European Paediatric Orthopaedic Society members from 13 countries for congenital pseudarthrosis of tibia. The treatment data of 340 patients who underwent 1287 procedures for this condition were analyzed. The essential findings were that the method of choice needed to approach the biological problem with the aims of: (1) resecting the pseudarthrosis to provide stability, the basic requirement for bony consolidation; (2) correcting length discrepancy and axial deformity; (3) achieving fusion; and (4) solving the additional problems around the main deformity such as alignment, leg length discrepancy and ankle valgus. The Ilizarov technique emerged as being the optimal method, having the highest rate of fusion (75.5%) of pseudarthrosis and rate of success in correction of the additional deformities. There was also consensus that surgery should be avoided before the third year of life.

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Pathology and natural history of congenital pseudarthrosis of the tibia.

John Boyd (1982)

Of the six types of congenital pseudarthrosis, Type II is the most common and lends to poorest prognosis. The basic pathology is an aggressive osteolytic fibromatosis. Failures in these patients are due to recurrence of the osteolytic fibromatosis, which can remove living bone or a dead bone graft. Type III cases have a better prognosis. The gross pathology is a bone cyst, and the microscopic findings resemble fibrous dysplasia. After surgical treatment, generally, the older the child the better the prognosis. Recurrences are rare after skeletal maturity.

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Author and article information

Contributors

Jan Vanderstappen: +32 16 33 88 27 , jan.vanderstappen@uzleuven.be

Journal

Journal ID (nlm-ta): J Child Orthop

Journal ID (iso-abbrev): J Child Orthop

Title: Journal of Children's Orthopaedics

Publisher: Springer Berlin Heidelberg (Berlin/Heidelberg )

ISSN (Print): 1863-2521

ISSN (Electronic): 1863-2548

Publication date (Electronic): 13 August 2015

Publication date PMC-release: 13 August 2015

Publication date (Print): August 2015

Volume: 9

Issue: 4

Pages: 319-324

Affiliations

[ ]Department of Orthopaedic Surgery, University of Leuven, Leuven, Belgium

[ ]Ilizarov Department, Department of Orthopaedic Surgery, University of Leuven, Leuven, Belgium

[ ]Department of Pediatric Orthopaedics, Department of Orthopaedic Surgery, University of Brussels, Brussels, Belgium

Article

Publisher ID: 675

DOI: 10.1007/s11832-015-0675-7

PMC ID: 4549345

PubMed ID: 26266468

SO-VID: 0b94c518-d95a-498a-a509-512916bd11af

License:

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

History

Date received : 19 April 2015

Date accepted : 29 July 2015

Custom metadata

ScienceOpen disciplines: Orthopedics

Keywords: congenital pseudarthrosis of the tibia,ilizarov bone transport,neurofibromatosis

Data availability:

ScienceOpen disciplines: Orthopedics

Keywords: congenital pseudarthrosis of the tibia, ilizarov bone transport, neurofibromatosis

Ilizarov bone transport as a treatment of congenital pseudarthrosis of the tibia: a long-term follow-up study

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Abstract

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Orthopedics

Most cited references 27

Neurofibromatosis update.

Treatment approaches for congenital pseudarthrosis of tibia: results of the EPOS multicenter study. European Paediatric Orthopaedic Society (EPOS).

Pathology and natural history of congenital pseudarthrosis of the tibia.

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