Current treatment of congenital pseudarthrosis of the tibia: a systematic review and meta-analysis

Kesireddy, Nithin; Kheireldin, Rowida K; Lu, Alex Y.; Cooper, Jonathan D.; Liu, Jiayong; Ebraheim, Nabil A.

doi:10.1097/bpb.0000000000000524

Cited by 44 publications

(60 citation statements)

References 32 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Unfortunately, he failed to keep brace wearing and refracture occurred after trauma. These results were similar or even superior to other studies [ 16 ]. Dobbs et al reported that trans-ankle Williams rod showed satisfactory long-term outcomes, with initial consolidation obtained in 18 (85.7%) of 21 patients and 12 (23.2%) cases of refracture.…”

Section: Discussionsupporting

confidence: 91%

“…The achievement of skeletal maturity with functional utility and anatomic alignment is the primary goal of treating CPT [ 15 , 16 ]. Due to the high rate of refracture, pediatric surgeons were compelled to focus on successful osteosynthesis rather than the sequelae of CPT, such as joint stiffness and arthritic change led by trans-ankle fixation; metaphyseal irritation and growth failure led by metaphyseal intrusion; and even failure of IM fixation due to inadequate space for nail interlocking in pediatric patients [ 2 , 8 , 10 , 17 – 19 ].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Efficacy of the “Eiffel tower” double titanium elastic nailing in combined management of congenital pseudarthrosis of the tibia: preliminary outcomes of 17 cases with review of literature

Wang

Shi

Zhang

et al. 2021

BMC Musculoskelet Disord

View full text Add to dashboard Cite

Background Difficulty in obtaining union, recurrent fractures, and residual deformities remain the problems challenging the management of congenital pseudarthrosis of the tibia (CPT). We applied the “Eiffel Tower” double titanium elastic nails (TENs) in the existing combined approach, which takes advantages of TEN’s mechanical stability with the protection against refracture, Ilizarov’s high fusion rate with alignment control and the biologic environment provided by bone grafting for bony union. The results of this procedure are presented and discussed. Methods Seventeen patients with CPT treated by combined surgery including pseudarthrosis resection, the “Eiffel Tower” double TENs technique, autogenous iliac bone grafting, and Ilizarov fixation between 2013 and 2019 were retrospectively investigated. Signs of bone union, limb length discrepancy (LLD), rate of refracture, and degree of residual deformities were reviewed. The AOFAS Ankle Hindfoot scale and measurement of ankle motion were used to evaluate ankle function. The mean follow-up time was 40.5 (11 to 91) months. Results The mean age at index surgery was 6.2 (2.5 to 15) years. Union of the pseudarthrosis was achieved in 100% of cases. Among them, 15 (88.2%) patients obtained union of the pseudarthrosis on the first attempt (primary union). The average time to primary union was 3.8 (2 to 6) months. The rest 2 cases achieved union after additional surgeries (secondary union). In terms of complications, refracture occurred in 2 patients (11.8%) and 4 patients (23.5%) developed pin infection. The mean limb length discrepancy at the final follow up was 33.4 (6–141) mm. The average AOFAS score improved from 38.2 (27 to 51) pre-operatively to 77 (63 to 87) post-operatively (p < 0.01). Conclusions The “Eiffel Tower” double TENs technique is an ideal intramedullary fixation method in the surgical treatment of CPT. The combination of TENs technique with bone grafting and Ilizarov fixation has the advantages of early bone union, less injury on metaphysis, and early functional recovery. Level of evidence Level IV.

show abstract

Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Efficacy of the “Eiffel tower” double titanium elastic nailing in combined management of congenital pseudarthrosis of the tibia: preliminary outcomes of 17 cases with review of literature

Wang

Shi

Zhang

et al. 2021

BMC Musculoskelet Disord

View full text Add to dashboard Cite

show abstract

“…CPT patients commonly have a high rate of fracture recurrence. Bone morphogenetic protein (BMP) in treatment has no advantages in improving initial union, and decreasing the duration between union and refracture episodes [25]. Therefore, genetic and molecular factors rather than an environmental factor are more likely contributing to CPT pathogenesis.…”

Section: Discussionmentioning

confidence: 99%

Identification and characterization of NF1 and non-NF1 congenital pseudarthrosis of the tibia based on germline NF1 variants: genetic and clinical analysis of 75 patients

Zhu

Zheng

Liu

et al. 2019

Orphanet J Rare Dis

View full text Add to dashboard Cite

Background Congenital pseudarthrosis of the tibia (CPT) is a rare disease. Some patients present neurofibromatosis type 1 (NF1), while some others do not manifest NF1 (non-NF1). The etiology of CPT, particularly non-NF1 CPT, is not well understood. Here we screened germline variants of 75 CPT cases, including 55 NF1 and 20 non-NF1. Clinical data were classified and analyzed based on NF1 gene variations to investigate the genotype-phenotype relations of the two types of patients. Results Using whole-exome sequencing and Multiplex Ligation-Dependent Probe Amplification, 44 out of 55 NF1 CPT patients (80.0%) were identified as carrying pathogenic variants of the NF1 gene. Twenty-five variants were novel; 53.5% of variants were de novo, and a higher proportion of their carriers presented bone fractures compared to inherited variant carriers. No NF1 pathogenic variants were found in all 20 non-NF1 patients. Clinical features comparing NF1 CPT to non-NF1 CPT did not show significant differences in bowing or fracture onset, lateralization, tissue pathogenical results, abnormality of the proximal tibial epiphysis, and follow-up tibial union after surgery. A considerably higher proportion of non-NF1 patients have cystic lesion (Crawford type III) and used braces after surgery. Conclusions We analyzed a large cohort of non-NF1 and NF1 CPT patients and provided a new perspective for genotype-phenotype features related to germline NF1 variants. Non-NF1 CPT in general had similar clinical features of the tibia as NF1 CPT. Germline NF1 pathogenic variants could differentiate NF1 from non-NF1 CPT but could not explain the CPT heterogeneity of NF1 patients. Our results suggested that non-NF1 CPT was probably not caused by germline NF1 pathogenic variants. In addition to NF1, other genetic variants could also contribute to CPT pathogenesis. Our findings would facilitate the interpretation of NF1 pathogenic variants in CPT genetic counseling.

show abstract

“…The achievement of skeletal maturity with functional utility and anatomic alignment of the bone is the primary goal of treating CPT (3,4). Among various surgical techniques that have been developed to optimize the outcomes in treating CPT, the Ilizarov technique has been proved to be valuable in promoting high rate of union through compression, dealing with limb length discrepancy via diaphyseal transfer and addressing angular deformities concomitantly.…”

Section: Discussionmentioning

confidence: 99%

Prebent Titanium Elastic Nailing, Ilizarov Fixation and Bone Grafting for Treating Congenital Pseudarthrosis of the Tibia: Preliminary Outcomes of 15 Cases

Wang

Zhang

et al. 2020

Preprint

View full text Add to dashboard Cite

Background: To report the preliminary outcome of treating congenital pseudarthrosis of the tibia (CPT) by using titanium elastic nails (TENs) combined with other surgical procedures including pseudarthrosis resection, autogenous iliac bone grafting and Ilizarov technique, with a mean follow-up time of 4.5 (3.3 to 6.3) years.Methods: 15 patients with CPT treated by combined surgery including pseudarthrosis resection, titanium elastic nails (TENs) technique, autogenous iliac bone grafting and Ilizarov fixation between 2013 and 2017 were retrospectively investigated. Signs of bone union, limb length discrepancy (LLD), rate of refracture and degree of residual deformities were reviewed. The AOFAS Ankle Hindfoot scale and measurement of ankle movement were used to evaluate ankle function. Results: The mean age at index surgery was 110 (24 to 285) months. 13 (86.7 %) patients obtained union of the pseudarthrosis on the first attempt (primary union). The average time to primary union was 4.8 (3 to 7) months. The rest 2 cases achieved union after additional surgeries (secondary union). In terms of complications, refracture occurred in 2 patients (13.3%) and 5 (33.3%) patients developed pin infection. The mean limb length discrepancy at the final follow up was 14.6 (4–41) mm. 3 patients (20 %) had ankle valgus deformities with a mean LDTA of 74.3° (range, 68 to 81°). The average AOFAS score improved from 40.3 (27 to 51) pre-operatively to 76.1 (63 to 87) post-operatively. For the patients who received proximal tibial lengthening, the average healing index (HI) was 65.1 (57.3 to 77.8) days/cm.Conclusions: Titanium elastic nails (TENs) technique is an ideal intramedullary fixation method in the surgical treatment of CPT. The combination of TENs technique with bone grafting and Ilizarov fixation has the advantages of early bone union, less injury on metaphyseal and early functional recovery.

show abstract

Current treatment of congenital pseudarthrosis of the tibia: a systematic review and meta-analysis

Cited by 44 publications

References 32 publications

Efficacy of the “Eiffel tower” double titanium elastic nailing in combined management of congenital pseudarthrosis of the tibia: preliminary outcomes of 17 cases with review of literature

Efficacy of the “Eiffel tower” double titanium elastic nailing in combined management of congenital pseudarthrosis of the tibia: preliminary outcomes of 17 cases with review of literature

Identification and characterization of NF1 and non-NF1 congenital pseudarthrosis of the tibia based on germline NF1 variants: genetic and clinical analysis of 75 patients

Prebent Titanium Elastic Nailing, Ilizarov Fixation and Bone Grafting for Treating Congenital Pseudarthrosis of the Tibia: Preliminary Outcomes of 15 Cases

Contact Info

Product

Resources

About