Guided Growth in Leg Length Discrepancy in Beckwith-Wiedemann Syndrome: A Consecutive Case Series

Overview

Journal Children (Basel)

Specialty Health Services

Date 2021 Dec 24

PMID 34943348

Citations 2

Authors

Maurizio De Pellegrin

Lorenzo Brogioni

Guy Laskow

Graziano Barera

Roberta Pajno

Sara Osimani

Silvia Russo

Lorenzo Marcucci

Affiliations

Soon will be listed here.

Abstract

Beckwith-Wiedemann Syndrome (BWS) is a rare genetic disorder characterized by overgrowth, macroglossia, abdominal wall defects, neonatal hypoglycemia, predisposition to embryonal tumor, lateralized overgrowth, and leg length discrepancy (LLD), which can affect normal posture and gait. Aim of this study was to evaluate the effects of guided growth (temporary epiphysiodesis technique) as LLD management in BWS patients. Between 2007 and 2021, 22 BWS patients (15 F, 7 M) with a mean age of 7.9 years (2.9-14.4) and a mean LLD at first surgery of 3.65 cm (2-10), underwent temporary proximal tibial (PTE) and distal femur epiphysiodesis (DFE). In 18 patients the first surgical procedure was PTE, in one, DFE, and in 3 cases, PTE and DFE at the same time, respectively. Eleven patients reached equality of leg length after a mean follow-up of 7.7 years (3.7-13.0) and mean age of 13.3 years (12.7-27.5); 10 patients underwent 3 surgical procedures, one 7 procedures. Fifteen patients had no complications. No severe complications, infection, articular stiffness, or neuro-vascular lesions occurred in remaining patients; complications included secondary varus or valgus axial deviation in a total of 6 patients, and two screw breakages in two patients. Guided growth as a minimally invasive procedure seems efficient for LLD treatment with low complication rate in BWS patients.

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References

Boyle J, Makarov M, Podeszwa D, Rodgers J, Jo C, Birch J . Is Proximal Fibula Epiphysiodesis Necessary When Performing a Proximal Tibial Epiphysiodesis?. J Pediatr Orthop. 2020; 40(10):e984-e989. DOI: 10.1097/BPO.0000000000001642. View

Kalish J, Biesecker L, Brioude F, Deardorff M, Di Cesare-Merlone A, Druley T . Nomenclature and definition in asymmetric regional body overgrowth. Am J Med Genet A. 2017; 173(7):1735-1738. PMC: 5932268. DOI: 10.1002/ajmg.a.38266. View

Brioude F, Lacoste A, Netchine I, Vazquez M, Auber F, Audry G . Beckwith-Wiedemann syndrome: growth pattern and tumor risk according to molecular mechanism, and guidelines for tumor surveillance. Horm Res Paediatr. 2013; 80(6):457-65. DOI: 10.1159/000355544. View

Mussa A, Russo S, de Crescenzo A, Freschi A, Calzari L, Maitz S . Fetal growth patterns in Beckwith-Wiedemann syndrome. Clin Genet. 2016; 90(1):21-7. DOI: 10.1111/cge.12759. View

Brioude F, Kalish J, Mussa A, Foster A, Bliek J, Ferrero G . Expert consensus document: Clinical and molecular diagnosis, screening and management of Beckwith-Wiedemann syndrome: an international consensus statement. Nat Rev Endocrinol. 2018; 14(4):229-249. PMC: 6022848. DOI: 10.1038/nrendo.2017.166. View

Gurney B . Leg length discrepancy. Gait Posture. 2002; 15(2):195-206. DOI: 10.1016/s0966-6362(01)00148-5. View

Martin R, Grange D, Zehnbauer B, DeBaun M . LIT1 and H19 methylation defects in isolated hemihyperplasia. Am J Med Genet A. 2005; 134A(2):129-31. DOI: 10.1002/ajmg.a.30578. View

Song K, Kim B, Lee S . Percutaneous leverage reduction for severely displaced radial neck fractures in children. J Pediatr Orthop. 2014; 35(4):e26-30. DOI: 10.1097/BPO.0000000000000305. View

Strong M, Hruska J, Czyrny J, Heffner R, Brody A . Nerve palsy during femoral lengthening: MRI, electrical, and histologic findings in the central and peripheral nervous systems--a canine model. J Pediatr Orthop. 1994; 14(3):347-51. DOI: 10.1097/01241398-199405000-00014. View

10.

Mussa A, Russo S, De Crescenzo A, Freschi A, Calzari L, Maitz S . (Epi)genotype-phenotype correlations in Beckwith-Wiedemann syndrome. Eur J Hum Genet. 2015; 24(2):183-90. PMC: 4717210. DOI: 10.1038/ejhg.2015.88. View

11.

Lee D, Han T, Choi I, Lee C, Chung S . Changes in somatosensory-evoked potentials in limb lengthening. An experimental study on rabbits' tibiae. Clin Orthop Relat Res. 1992; (285):273-9. View

12.

Stevens P . The role of guided growth as it relates to limb lengthening. J Child Orthop. 2016; 10(6):479-486. PMC: 5145829. DOI: 10.1007/s11832-016-0779-8. View

13.

Tenorio J, Romanelli V, Martin-Trujillo A, Fernandez G, Segovia M, Perandones C . Clinical and molecular analyses of Beckwith-Wiedemann syndrome: Comparison between spontaneous conception and assisted reproduction techniques. Am J Med Genet A. 2016; 170(10):2740-9. DOI: 10.1002/ajmg.a.37852. View

14.

McCarthy J, Burke T, McCarthy M . Need for concomitant proximal fibular epiphysiodesis when performing a proximal tibial epiphysiodesis. J Pediatr Orthop. 2002; 23(1):52-4. View

15.

Mussa A, Di Candia S, Russo S, Catania S, De Pellegrin M, Di Luzio L . Recommendations of the Scientific Committee of the Italian Beckwith-Wiedemann Syndrome Association on the diagnosis, management and follow-up of the syndrome. Eur J Med Genet. 2015; 59(1):52-64. DOI: 10.1016/j.ejmg.2015.11.008. View

16.

Dammerer D, Kirschbichler K, Donnan L, Kaufmann G, Krismer M, Biedermann R . Clinical value of the Taylor Spatial Frame: a comparison with the Ilizarov and Orthofix fixators. J Child Orthop. 2012; 5(5):343-9. PMC: 3179531. DOI: 10.1007/s11832-011-0361-3. View

17.

Stevens P . Invalid Comparison Between Methods of Epiphysiodesis. J Pediatr Orthop. 2017; 38(1):e29-e30. DOI: 10.1097/BPO.0000000000001020. View

18.

Mishima K, Kitoh H, Kadono I, Matsushita M, Sugiura H, Hasegawa S . Prediction of Clinically Significant Leg-Length Discrepancy in Congenital Disorders. Orthopedics. 2015; 38(10):e919-24. DOI: 10.3928/01477447-20151002-60. View

19.

Carli D, De Pellegrin M, Franceschi L, Zinali F, Paonessa M, Spolaore S . Evolution over Time of Leg Length Discrepancy in Patients with Syndromic and Isolated Lateralized Overgrowth. J Pediatr. 2021; 234:123-127. DOI: 10.1016/j.jpeds.2021.01.020. View

20.

Sinha R, Weigl D, Mercado E, Becker T, Kedem P, Bar-On E . Eight-plate epiphysiodesis: are we creating an intra-articular deformity?. Bone Joint J. 2018; 100-B(8):1112-1116. DOI: 10.1302/0301-620X.100B8.BJJ-2017-1206.R3. View