Residual Challenges After Healing of Congenital Pseudarthrosis in the Tibia

Kristiansen, Leif Pål; Steen, Harald; Terjesen, Terje

doi:10.1097/01.blo.0000076800.53006.c9

Cited by 37 publications

(41 citation statements)

References 20 publications

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“…Procurvatum in the tibia is a characteristic of the disease, but it seems challenging to adequately address all deformities in CPT, especially when considering the fact that patients often weight bear for a few years on the affected limb before primary surgery with resulting adaptations in the foot. Our findings on the residual deformities in CPT confirm those of other authors [17,26].…”

Section: Discussionsupporting

confidence: 93%

Epidemiology and treatment outcome of congenital pseudarthrosis of the tibia

Horn

Steen

Terjesen

2013

Journal of Children's Orthopaedics

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Purpose Congenital pseudarthrosis of the tibia (CPT) is a rare disease. Epidemiological data are limited, and treatment of the condition is challenging. The purpose of our study was to gain epidemiological data on the incidence of CPT in Norway and to evaluate the treatment outcome of the disease. Methods During the period 1987–2006 22 patients with CPT were born in Norway (11 boys, 11 girls; mean age 15 years, age range 8–24 years) and are included in this study. During the same time period 1,183,380 live-births were registered by the Norwegian Birth Register. Primary surgical treatment was the Ilizarov method in 15 patients, intramedullary nailing in three patients, and plate osteosynthesis in two patients; two patients never developed a fracture and were treated with an orthosis. Results The incidence of CPT based on this period was 1:53,000. The rate of primary healing was 66 % for the Ilizarov group. Primary healing occurred in three patients treated with intramedullary nailing and in none of the patients treated with plate osteosynthesis. However, almost all patients required additional surgery due to refracture or deformity correction. Currently, all 12 skeletally mature patients are considered to be healed, whereas two of the skeletally immature patients are still under treatment. Conclusion The incidence of CPT in Norway seems to be notably higher than that based on epidemiological data from other studies. Primary healing rates are satisfactory when treated either with an Ilizarov device or intramedullary nailing. Refractures must be avoided, and alignment of the leg must be maintained. Healing is usually achieved before skeletal maturity. However, residual deformities are common.

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Section: Discussionsupporting

confidence: 93%

Epidemiology and treatment outcome of congenital pseudarthrosis of the tibia

Horn

Steen

Terjesen

2013

Journal of Children's Orthopaedics

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“…Its review of the 120 published CPT cases using the Ilizarov technique showed satisfactory results with pseudarthrosis healing in 94 patients [6]. However, Kristiansen [14] emphasized the high complication rate, including residual axial deformities and refracture.…”

Section: Discussionmentioning

confidence: 99%

“…Among these procedures, the Ilizarov technique has a high rate of fusion, allowing for the correction of axial malalignment and leg shortening at the same time [1][2][3][4][5]. The drawback of this technique is its lack of protection against refractures after removal of the fixator [14]. To address this issue, we have developed an original surgical technique that combines Ilizarov's fixator with intramedullary nailing of the tibia.…”

Section: Introductionmentioning

confidence: 99%

Association of Ilizarov's technique and intramedullary rodding in the treatment of congenital pseudarthrosis of the tibia

Laurent¹,

Vialle²,

Thévenin-Lemoine³

et al. 2008

Journal of Children's Orthopaedics

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Purpose Many surgical techniques have been published on how to treat congenital pseudoarthrosis of the tibia (CPT). We combined Ilizarov's fixator with intramedullary nailing of the tibia and developed a procedure which combines the advantages of both methods: Ilizarov's high fusion rate with alignment control and the protection against refracture provided by the intramedullary nail. The results of this approach are presented and discussed. Material and methods Seven boys and three girls aged 3-14 years (mean age 8 years 2 months) were treated using our combined technique. In six cases, the CPT was associated with neurofibromatosis. Two strategies were adopted: in six cases, a compression was applied on the bone defect, and in four cases, segmental bone transportation was performed before the compression procedure. The final follow-up (1.2-6.6 years) included a clinical and radiological examination. Results Tibial union was achieved in nine cases without bone grafting. In one case, tibial union still remains uncertain, despite intertibiofibular bone grafting and additional compression procedures. Thirteen overall complications were noted, including three valgus deformity of the ankle. Bone transportation failed to achieve complete limb lengthening in three cases. One deep infection occurred 4 years after removal of the external fixator. The treatment for this included nail removal and antibiotic therapy for 3 months. Despite a permanent protection of orthosis, a refracture occurred 2 years after nail removal, reverting to the initial level of pseudarthrosis. Another surgical attempt using the same method was then performed with a satisfactory result. Conclusions The association of Ilizarov's technique and intramedullary nailing achieved and maintained tibial union in nine of ten patients at final follow-up. It also allowed correction of axial deformities and prevented refracture. Despite the short duration of the follow-up and a high rate of complications in our series, this method can be useful in many cases of CPT in which healing has failed to occur despite many previous surgeries.

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“…Although surgical intervention has achieved substantial improvements in terms of the healing rates of congenital pseudarthrosis of the tibia over recent decades, challenging problems still jeopardize the function of the affected limb 2 .…”

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confidence: 99%