Results and Complications of Bilateral Limb Lengthening in Achondroplasia: a Retrospective Analysis

Overview

Journal Front Pediatr

Specialty Pediatrics

Date 2023 Nov 29

PMID 38027309

Authors

Fabio Verdoni

Riccardo Giorgino

Camilla Virgilio

Alessandra Nannini

Marco Vigano

Domenico Curci

Giuseppe Michele Peretti

Laura Mangiavini

Affiliations

Soon will be listed here.

Abstract

Background: Achondroplasia is one of the main causes of disharmonic dwarfism. Patients with achondroplasia might have physical and psychological limitations due to their disproportionate stature. Surgical limb lengthening is the only practical option available to achieve a stature comparable to normal population range. The purpose of this study is to analyze results and complications of our lengthening protocol.

Methods: A retrospective analysis was performed on 33 patients with achondroplasia (21 females and 12 males) undergoing simultaneous bilateral tibia or femur lengthening in four surgical stages from 2017 to 2021 (46 lengthening procedures, with a total of 56 tibias and 36 femurs). For each patient, patients' characteristics and antero-posterior and lateral radiographs were obtained. The following parameters were analyzed: duration of lengthening with external fixator, amount of lengthening, complications or events that influenced outcomes and the healing index (HI).

Results: The average tibial and femoral gain was 7.9 cm and 6.9 cm, respectively. The tibiae achieved better results than the femurs ( = 0.005). Nineteen complications were reported for 92 segments (20.7%), and the variables influencing complications were: step ( = 0.002) and fixation duration ( = 0.061).

Conclusions: Bilateral parallel lower limb lengthening in four surgical steps may be a viable technique in patients with achondroplasia.

References

Venkatesh K, Modi H, Devmurari K, Yoon J, Anupama B, Song H . Femoral lengthening in achondroplasia: magnitude of lengthening in relation to patterns of callus, stiffness of adjacent joints and fracture. J Bone Joint Surg Br. 2009; 91(12):1612-7. DOI: 10.1302/0301-620X.91B12.22418. View

Dossanov B, Trofimchuk V, Lozovoy V, Khmyzov S, Dossanova A, Zhukenov O . Evaluating the results of long tubular bone distraction with an advanced rod monolateral external fixator for achondroplasia. Sci Rep. 2021; 11(1):14727. PMC: 8290032. DOI: 10.1038/s41598-021-94146-z. View

Kim S, Cielo Balce G, Agashe M, Song S, Song H . Is bilateral lower limb lengthening appropriate for achondroplasia?: midterm analysis of the complications and quality of life. Clin Orthop Relat Res. 2011; 470(2):616-21. PMC: 3254769. DOI: 10.1007/s11999-011-1983-y. View

Kazmers N, Fragomen A, Rozbruch S . Prevention of pin site infection in external fixation: a review of the literature. Strategies Trauma Limb Reconstr. 2016; 11(2):75-85. PMC: 4960058. DOI: 10.1007/s11751-016-0256-4. View

Aldegheri R, DallOca C . Limb lengthening in short stature patients. J Pediatr Orthop B. 2001; 10(3):238-47. View

Cormier-Daire V, AlSayed M, Ben-Omran T, de Sousa S, Boero S, Fredwall S . The first European consensus on principles of management for achondroplasia. Orphanet J Rare Dis. 2021; 16(1):333. PMC: 8325831. DOI: 10.1186/s13023-021-01971-6. View

Park K, Garcia R, Rejuso C, Choi J, Song H . Limb Lengthening in Patients with Achondroplasia. Yonsei Med J. 2015; 56(6):1656-62. PMC: 4630057. DOI: 10.3349/ymj.2015.56.6.1656. View

Shiang R, Thompson L, Zhu Y, Church D, Fielder T, Bocian M . Mutations in the transmembrane domain of FGFR3 cause the most common genetic form of dwarfism, achondroplasia. Cell. 1994; 78(2):335-42. DOI: 10.1016/0092-8674(94)90302-6. View

Castelein S, Docquier P . Complications associated with bone lengthening of the lower limb by callotasis. Acta Orthop Belg. 2017; 82(4):806-813. View

10.

Tjernstrom B, Thoumas K, PECH P . Bone remodeling after leg lengthening: evaluation with plain radiographs, and computed tomography and magnetic resonance imaging scans. J Pediatr Orthop. 1992; 12(6):751-5. View

11.

Vaidya S, Song H, Lee S, Suh S, Keny S, Telang S . Bifocal tibial corrective osteotomy with lengthening in achondroplasia: an analysis of results and complications. J Pediatr Orthop. 2006; 26(6):788-93. DOI: 10.1097/01.bpo.0000242429.83866.97. View

12.

Donaldson J, Aftab S, Bradish C . Achondroplasia and limb lengthening: Results in a UK cohort and review of the literature. J Orthop. 2015; 12(1):31-4. PMC: 4353991. DOI: 10.1016/j.jor.2015.01.001. View

13.

Peretti G, Memeo A, Paronzini A, Marzorati S . Staged lengthening in the prevention of dwarfism in achondroplastic children: a preliminary report. J Pediatr Orthop B. 1995; 4(1):58-64. DOI: 10.1097/01202412-199504010-00009. View

14.

Chan M, Qi Y, Larimore K, Cherukuri A, Seid L, Jayaram K . Pharmacokinetics and Exposure-Response of Vosoritide in Children with Achondroplasia. Clin Pharmacokinet. 2021; 61(2):263-280. PMC: 8813707. DOI: 10.1007/s40262-021-01059-1. View

15.

Song S, Kim S, Agashe M, Lee H, Refai M, Park Y . Growth disturbance after lengthening of the lower limb and quantitative assessment of physeal closure in skeletally immature patients with achondroplasia. J Bone Joint Surg Br. 2012; 94(4):556-63. DOI: 10.1302/0301-620X.94B4.28375. View

16.

Batibay S, Balci H, Bayram S, Chodza M, Goksoy S, Hurmeydan O . Quality of Life Evaluation Following Limb Lengthening Surgery in Patients with Achondroplasia. Indian J Orthop. 2020; 54(Suppl 1):39-46. PMC: 7474039. DOI: 10.1007/s43465-020-00127-1. View

17.

Pauli R . Achondroplasia: a comprehensive clinical review. Orphanet J Rare Dis. 2019; 14(1):1. PMC: 6318916. DOI: 10.1186/s13023-018-0972-6. View

18.

Griffith S, McCarthy J, Davidson R . Comparison of the complication rates between first and second (repeated) lengthening in the same limb segment. J Pediatr Orthop. 2006; 26(4):534-6. DOI: 10.1097/01.bpo.0000226275.70706.d5. View

19.

Paley D . Current techniques of limb lengthening. J Pediatr Orthop. 1988; 8(1):73-92. DOI: 10.1097/01241398-198801000-00018. View

20.

Paley D . Extensive Limb Lengthening for Achondroplasia and Hypochondroplasia. Children (Basel). 2021; 8(7). PMC: 8305967. DOI: 10.3390/children8070540. View