Congenital Pseudarthrosis of the Tibia Associated with Neurofibromatosis-1: Treatment with Ilizarov's Device

The results of treatment of congenital pseudarthrosis of the tibia (CPT) are frequently unsatisfactory because of the need for multiple operations for recalcitrant nonunion, residual deformities, and limb-length discrepancies (LLD). Although the etiology of CPT is basically unknown, recent reports suggest the periosteum is the primary site for the pathologic processes in CPT. We hypothesized complete excision of the diseased periosteum and the application of a combined approach including free periosteal grafting, bone grafting, and intramedullary (IM) nailing of both the tibia and fibula combined with Ilizarov fixation would improve union rates and reduce refracture rates. We retrospectively reviewed 20 patients at two centers. The minimum followup was 2 years (mean, 4.3 years; range, 2-10

show abstract

“…Boero et al [3] 21 Cystic (4) Hourglass (11) Normotrophic (1) Sclerotic (5) 8.8 (1.3-17) Minimum 2-year followup 6.3 cm (0-19 cm)…”

Section: Npmentioning

confidence: 99%

“…1.2 (1-1.5) Paley et al [33] 15 (16) Andersen [1] Cystic (3) Sclerotic (4) Dysplastic (9) 8 (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17) 4 (2-7)…”

Section: Introductionmentioning

confidence: 99%

Periosteal Grafting for Congenital Pseudarthrosis of the Tibia: A Preliminary Report

Thabet

Paley

Kocaoğlu

et al. 2008

Clinical Orthopaedics &Amp; Related Research

View full text Add to dashboard Cite

show abstract

“…Consequently, there is still room for improvement! [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]. There is, however, consensus that all pseudarthrotic tissue should be resected and bone graft applied to promote healing.…”

Section: Introductionmentioning

confidence: 99%

Preliminary experience with the combined use of recombinant bone morphogenetic protein and bisphosphonates in the treatment of congenital pseudarthrosis of the tibia

Birke

Schindeler

Ramachandran

et al. 2010

Journal of Children's Orthopaedics

View full text Add to dashboard Cite

Purpose Congenital pseudarthrosis of the tibia (CPT) is a rare but serious disorder in children. No single approach has clearly emerged as superior in terms of operative procedure, fixation, optimal time for surgery or adjunctive pharmaceutical intervention. CPT is frequently associated with neurofibromatosis type 1 (NF1), a condition featuring deficient bone anabolism and excessive catabolism. We have therefore combined the use of bone morphogenetic proteins (BMP) with bisphosphonates (BP) as an adjunct to surgical intervention. Methods Between 2002 and 2008 we administered BMP-7 (OP-1) at the time of surgery followed by BP (pamidronate or zoledronic acid) in eight Crawford type IV CPT cases in seven patients (six with a confirmed diagnosis of NF1) with a median age of 7 years (range 2 years 11 months to 12 years) at surgery.

show abstract

“…Resection of the pseudarthrosis site with immediate shortening and compression, with lengthening via a proximal metaphyseal corticotomy, has been successfully advocated [15][16][17]. Immediate shortening and compression would theoretically have the advantage, similar to the A procedure for IM rodding, that no gap to fill with fibrous tissue is allowed to develop, and the ''freshened'' bone ends are immediately coapted, thus having the greatest chance to unite.…”

mentioning

confidence: 99%

A tale of two tibias: A review of treatment options for congenital pseudarthrosis of the tibia

Johnston

Birch

2008

Journal of Children's Orthopaedics

View full text Add to dashboard Cite

Purpose Two common treatment options for congenital pseudarthrosis of the tibia (CPT) are intramedullary fixation following resection/shortening of the pseudarthrosis site and reconstruction with an Ilizarov external fixator following resection. We present in detail a narrative of two cases with similar degrees of tibial dysplasia associated with NF-1 treated using these different methods and followed to completion. Methods Technical issues and details of the treatment methods from case reports are discussed in depth. The eventual profoundly different outcomes are correlated to the technical variations used. Results Treatment with the Charnley–Williams rodding method and aggressive bone grafting supplemented by rh-BMP2 resulted in a normal functioning limb at maturity, while treatment with first, an ineffective version of IM rodding, followed by two sessions of bone transport using an Ilizarov fixator failed to gain useful union and eventually resulted in amputation. Conclusions Technical details, heretofore inadequately reviewed in the literature, are crucial to the success of either of these commonly utilized treatment methods for CPT.

show abstract

Congenital Pseudarthrosis of the Tibia Associated with Neurofibromatosis-1: Treatment with Ilizarov's Device

Cited by 59 publications

References 23 publications

Periosteal Grafting for Congenital Pseudarthrosis of the Tibia: A Preliminary Report

Periosteal Grafting for Congenital Pseudarthrosis of the Tibia: A Preliminary Report

Preliminary experience with the combined use of recombinant bone morphogenetic protein and bisphosphonates in the treatment of congenital pseudarthrosis of the tibia

A tale of two tibias: A review of treatment options for congenital pseudarthrosis of the tibia

Contact Info

Product

Resources

About