Hip abduction and supported standing affect the ranges of hips extension in spinal muscular atrophy patients
More details
Hide details
Department of Rehabilitation, Józef Piłsudski University of Physical Education, Warsaw, Poland
Center of Functional Rehabilitation Orthos, Warsaw, Poland
Institute of Physiotherapy, Faculty of Health Sciences, The Jan Kochanowski University, Kielce, Poland
Submission date: 2020-07-28
Final revision date: 2020-10-12
Acceptance date: 2020-10-12
Online publication date: 2020-12-09
Corresponding author
Katarzyna Maślanko   

Center of Functional Rehabilitation Orthos, Warsaw, Poland
Pol. Ann. Med. 2021;28(1):50-56
Recommendations for management of spinal muscular atrophy (SMA) do not contain detailed information about the position of lower limbs during support standing. It has been observed that during the measurement of the range of extension in the hip joint (HE) in SMA patients, the examined limb was often naturally abducted.

The main aim of the study was to compare the values of HE in the sagittal plane and in abduction, and to assess the correlation between the duration of supported standing and HE in SMA patients.

Material and methods:
The study group consisted of 75 SMA individuals aged 2–22 years and control group consisted of 202 healthy participants. The measurements were performed with the Rippstein plurimeter and goniometer.

Results and discussion:
Range of HE in SMA patients was larger in abduction than in the sagittal plane. A correlation was noted between the duration of supported standing and HE.

Supported standing with hip joint abduction should be used in SMA patients. The obtained results broaden the knowledge about the biomechanics of hip joints in SMA patients.

We are thankful to all the participants and their parents for participating in the research. We want to express our thanks to the SMA Foundation Poland for cooperation and help in organizing the project.
The work (costs of translation) was supported by the Ministry of Science and Higher Education in 2017 as a research project of the University of Physical Education in Warsaw (DS-258).
The authors declare that there is no conflict of interest.
Lefebvre S, Buirglen L, Reboullet S, et al. Identification and characterization of a spinal muscular atrophy – determining gene. Cell. 1995;80(1):155–165.
D’ Amico A, Mercuri E, Tiziano F, Bertini E. Spinal muscular atrophy. Orphanet J Rare Dis. 2011;6:71.
Johnson ER, Fowler WM Jr, Lieberman JS. Contractures in neuromuscular disease. Arch Phys Med Rehabil. 1992;73(9):807–810.
Russman BS, Buncher CR, White M, Samaha FJ, Iannaccone ST. Function changes in spinal muscular atrophy II and III. The DCN/SMA Group. Neurology. 1996;47(4):973–976.
Wang CH, Finkel RS, Bertini ES, et al. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol. 2007;22(8):1027–1049.
Mullender MG, Blom NA, De Kleuver M, et al. A Dutch guideline for the treatment of scoliosis in neuromuscular disorders. Scoliosis. 2008;3:14.
Canavese F, Sussman MD. Strategies of hip management in neuromuscular disorders: Duchenne Muscular Dystrophy, Spinal Muscular Atrophy, Charcot-Marie-Tooth Disease and Arthrogryposis Multiplex Congenita. Hip Int. 2009;19(Suppl 6):46–52.
Fujak A, Kopschina C, Gras F, Forst R, Forst J. Contractures of the lower extremities in spinal muscular atrophy type II. Descriptive clinical study with retrospective data collection. Ortop Traumatol Rehabil. 2011;13(1):27–36.
Fujak A, Raab W, Schuh A, Richter S, Forst R, Forst J. Natural course of scoliosis in proximal spinal muscular atrophy type II and IIIa: descriptive clinical study with retrospective data collection of 126 patients. BMC Musculoskelet Disord. 2013;14:283.
Mercuri E, Finkel RS, Muntoni F, et al. Diagnosis and management of spinal muscular atrophy: Part 1: Recommendations for diagnosis, rehabilitation orthopedic and nutritional care. Neuromuscul Disord. 2018;28(2):103–115.
Wang HY, Ju YH, Chen SM, Lo SK, Jong YJ. Joint range of motion limitations in children and young adults with spinal muscular atrophy. Arch Phys Med Rehabil. 2004;85:1689–1693.
Granata C, Magni E, Merlini L, Cervellati S. Hip dislocation in spinal muscular atrophy. Chir Organi Mov. 1990;75(2):177–184.
Thompson CE, Larsen LJ. Recurrent hip dislocation in intermediate spinal atrophy. J Pediatr Orthop. 1990;10(5):638–641.
Sporer SM, Smith BG. Hip dislocation in patients with spinal muscular atrophy. J Pediatr Orthop. 2003;23(1):10–14.
Zenios M, Sampath J, Cole C, Khan T, Galasko CSB. Operative treatment for hip subluxation in spinal muscular atrophy. J Bone Joint Surg Br. 2005;87B(11):1541–1544.
McDonald CM, Abresch RT, Carter GT, Fowler JW, Johnson ER, Kilmer DD. Profiles of neuromuscular diseases. Duchenne muscular dystrophy. Am J Phys Med Rehabil. 1995;74(5 Suppl):70–92.
Chiriboga CA. Nusinersen for the treatment of spinal muscular atrophy. Expert Rev Neurother. 2017;17(10):955–962.
Farrar MA, Park SB, Vucic S, et al. Emerging therapies and challenges in spinal muscular atrophy. Ann Neurol. 2017;81(3):355–368.
Maharshi V, Hasan S. Nusinersen: The first option beyond supportive care for spinal muscular atrophy. Clin Drug Investig. 2017;37(9):807–817.
Paleg GS, Smith BA, Glickman LB. Systematic review and evidence-based clinical recommendations for dosing of pediatric supported standing programs. Pediatr Phys Ther. 2013;25(3):232–247.
Macias-Merlo L, Bagur-Calafat C, Girabent-Farrés M, Stuberg WA. Effects of the standing program with hip abduction on hip acetabular development in children with spastic diplegia cerebral palsy. Disabil Rehabil. 2016;38(11):1075–1081.
Norkin CC, White DJ. Measurement of Joint Motion: A Guide to Goniometry. 2nd ed. Philadelphia: FA Davis; 1995.
Stępień A, Jędrzejowska M, Guzek K, Rekowski W, Stępowska J. Reliability of four tests to assess body posture and the range of selected movements in individuals with spinal muscular atrophy. BMC Musculoskelet Disord. 2019;20(1):54.
Carter GT, Abresch RT, Fowler WM, Johnson ER, Kilmer DD, McDonald CM. Profiles of neuromuscular diseases. Spinal muscular atrophy. Am J Phys Med Rehabil. 1995;74(5 Suppl):S150–159.
Michalski T, Michalik P, Dąbrowska-Galas M, Król T, Rutkowska M, Hadała M. Efficacy of stretching in physiotherapy and sports. Pol Ann Med. 2018;25(2):277–282.
Iannaccone ST, Browne RH, Samaha FJ, Buncher CR. Prospective study of spinal muscular atrophy before age 6 years. Pediatr Neurol. 1993;9(3):187–193.
Skalsky AJ, McDonald CM. Prevention and management of limb contractures in neuromuscular diseases. Phys Med Rehabil Clin N Am. 2012;23(3):675–687.
Heckmatt JZ, Dubowitz V, Hyde SA, Florence J, Gabain AC, Thompson N. Prolongation of walking in Duchenne muscular dystrophy with lightweight orthoses: review of 57 cases. Dev Med Child Neurol. 1985;27(2):149–154.
Pardo AC, Do T, Ryder T, Meyer A, Miles L, Wong BL. Combination of steroids and ischial weight-bearing knee ankle foot orthoses in Duchenne’s muscular dystrophy prolongs ambulation past 20 years of age – a case report. Neuromuscul Disord. 2011;21(11):800–802.
Berven S, Bradford DS. Neuromuscular scoliosis: causes of deformity and principles for evaluation and management. Semin Neurol. 2002;22(2):167–178.
Mercuri E, Bertini E, Iannaccone ST. Childhood spinal muscular atrophy: controversies and challenges. Lancet Neurol. 2012;11(5):443–452.
Granata C, Merlini L, Magni E, Marini ML, Stagni SB. Spinal muscular atrophy: Natural history and orthopaedic treatment of scoliosis. Spine. 1989;14:760–762.
Journals System - logo
Scroll to top