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Lower Limb Deformity Management in Arthrogryposis – What to Correct and When

Volume 10 | Issue 2 | May-August 2024 | Page: 40-47 | Ishani P Shah, Sujika Ranmuthuge, Varun Parnami, Anastasios Chytas DOI- https://doi.org/10.13107/ijpo.2024.v10.i02.192 Submitted: 18/05/2024; Reviewed: 06/06/2024; Accepted: 16/07/2024; Published: 10/08/2024 Authors: Ishani P Shah DNB Ortho. FRCS (T & O) [1], Sujika Ranmuthuge MD, FRCS (T & O) [1], Varun Parnami DNB Ortho. [1], […]

Management of Limb Deficiencies

Volume 10 | Issue 2 | May-August 2024 | Page: 48-54 | Sakti Prasad Das, Sankar Ganesh, Prateek Behera DOI- https://doi.org/10.13107/ijpo.2024.v10.i02.194 Submitted: 11/03/2024; Reviewed: 08/04/2024; Accepted: 25/06/2024; Published: 10/08/2024 Authors: Sakti Prasad Das MS(Ortho.), DNB(PMR) [1], Sankar Ganesh MPT [2], Prateek Behera MS(Ortho.), DNB(Ortho.) [3] [1] Medical Education & Training, DRIEMS University, Odisha, Tangi, Cuttack, […]

Lower Limb Deformity Management in Arthrogryposis – What to Correct and When

Management of Limb Deficiencies

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Lower Limb Deformity Management in Arthrogryposis – What to Correct and When

Volume 10 | Issue 2 | May-August 2024 | Page: 40-47 | Ishani P Shah, Sujika Ranmuthuge, Varun Parnami, Anastasios Chytas

DOI- https://doi.org/10.13107/ijpo.2024.v10.i02.192

Submitted: 18/05/2024; Reviewed: 06/06/2024; Accepted: 16/07/2024; Published: 10/08/2024

Authors: Ishani P Shah DNB Ortho. FRCS (T & O) [1], Sujika Ranmuthuge MD, FRCS (T & O) [1], Varun Parnami DNB Ortho. [1], Anastasios Chytas MD, MSc [1]

[1] Department of Paediatric Orthopaedic Surgery, Royal Manchester Children’s Hospital, Oxford Road, M13 9WL

Address of Correspondence

Dr. Ishani P Shah,
Consultant Paediatric Orthopaedic Surgeon, Royal Manchester Children’s Hospital, Oxford Road, M13 9WL.
E-mail: ishanipshah@gmail.com

Abstract

Arthrogryposis is a descriptive term involving non – progressive joint contractures of two or more joints at birth. It is associated with more than 300 diseases. Lower limb involvement is seen in about 95% of the cases with variable affection of the foot, knee and hip. Management depends on the severity, affection of number of joints and co-morbidities. Multidisciplinary management is crucial with realistic expectations. Prognosis should be discussed with parents prior to undertaking surgical intervention, especially the risk of recurrence as age advances. Deformities include soft tissue contractures, fibrotic hypoplastic muscles and in older children, deformed articular congruity. Foot is most commonly affected and Ponseti casting is the gold standard first line of treatment. Failed correction or late presentations are treated with soft tissue/bony surgery and/or fixator. Knee contractures can be flexion or extension with or without joint subluxation and patella involvement. Options for management are serial casting, soft tissue release, growth modulation, bony surgery, gradual correction with fixator or a combination of these based on the severity and age at presentation. Pterygium management is difficult due to proximity of neurovascular structures to the skin web and higher risk of recurrence. Hip contractures and dislocation when unilateral should be treated surgically. Treatment of bilateral affection is controversial and should be individualised.
Keywords: Arthrogryposis, Amyoplasia, Arthrogryposis multiplex congenita, Hip contracture, Knee contracture, Lower limb deformity, Atypical clubfoot.

References

1. Bevan, W.P., et al., Arthrogryposis multiplex congenita (amyoplasia): an orthopaedic perspective. J Pediatr Orthop, 2007. 27(5): p. 594-600.
2. Hall, J.G., Arthrogryposis multiplex congenita: etiology, genetics, classification, diagnostic approach, and general aspects. J Pediatr Orthop B, 1997. 6(3): p. 159-66.
3. Sells, J.M., K.M. Jaffe, and J.G. Hall, Amyoplasia, the most common type of arthrogryposis: the potential for good outcome. Pediatrics, 1996. 97(2): p. 225-31.
4. Hall, J.G., K.A. Aldinger, and K.I. Tanaka, Amyoplasia revisited. Am J Med Genet A, 2014. 164A(3): p. 700-30.
5. Hansen-Jaumard, D., et al., A review of the orthopedic interventions and functional outcomes among a cohort of 114 children with arthrogryposis multiplex congenita. J Pediatr Rehabil Med, 2020. 13(3): p. 263-271.
6. Swinyard, C.A. and E.E. Bleck, The etiology of arthrogryposis (multiple congenital contracture). Clin Orthop Relat Res, 1985(194): p. 15-29.
7. Ooishi, T., et al., Congenital dislocation of the knee. Its pathologic features and treatment. Clin Orthop Relat Res, 1993(287): p. 187-92.
8. Hoffer, M.M., et al., Ambulation in severe arthrogryposis. J Pediatr Orthop, 1983. 3(3): p. 293-6.
9. Harold J.P. van Bosse, D.A.Z., The Orthopaedic Management of Arthrogryposis Multiplex Congenita. Journal of the Pediatric Orthopaedic Society of North America, 2021. 3(2): p. 277.
10. Carlson, W.O., et al., Arthrogryposis multiplex congenita. A long-term follow-up study. Clin Orthop Relat Res, 1985(194): p. 115-23.
11. Staheli, L.T., et al., Management of hip dislocations in children with arthrogryposis. J Pediatr Orthop, 1987. 7(6): p. 681-5.
12. St Clair, H.S. and S. Zimbler, A plan of management and treatment results in the arthrogrypotic hip. Clin Orthop Relat Res, 1985(194): p. 74-80.
13. Szoke, G., et al., Medial-approach open reduction of hip dislocation in amyoplasia-type arthrogryposis. J Pediatr Orthop, 1996. 16(1): p. 127-30.
14. van Bosse, H.J.P., et al., Treatment of the Lower Extremity Contracture/Deformities. J Pediatr Orthop, 2017. 37 Suppl 1: p. S16-S23.
15. Wada, A., et al., Surgical treatment of hip dislocation in amyoplasia-type arthrogryposis. J Pediatr Orthop B, 2012. 21(5): p. 381-5.
16. Yau, P.W., et al., Twenty-year follow-up of hip problems in arthrogryposis multiplex congenita. J Pediatr Orthop, 2002. 22(3): p. 359-63.
17. Nema, S.K., et al., Open reduction for hip dislocation in children with arthrogryposis multiplex congenital: Outcomes of a systematic review. J Clin Orthop Trauma, 2024. 53: p. 102434.
18. Sarwark, J.F., G.D. MacEwen, and C.I. Scott, Jr., Amyoplasia (a common form of arthrogryposis). J Bone Joint Surg Am, 1990. 72(3): p. 465-9.
19. Miao, M., et al., Early open reduction of dislocated hips using a modified Smith-Petersen approach in arthrogyposis multiplex congenita. BMC Musculoskelet Disord, 2020. 21(1): p. 144.
20. Hamdy, R.C., et al., Treatment and outcomes of arthrogryposis in the lower extremity. Am J Med Genet C Semin Med Genet, 2019. 181(3): p. 372-384.
21. van Bosse, H.J. and R.E. Saldana, Reorientational Proximal Femoral Osteotomies for Arthrogrypotic Hip Contractures. J Bone Joint Surg Am, 2017. 99(1): p. 55-64.
22. Guidera, K.J., et al., Radiographic changes in arthrogrypotic knees. Skeletal Radiol, 1991. 20(3): p. 193-5.
23. Sodergard, J. and S. Ryoppy, The knee in arthrogryposis multiplex congenita. J Pediatr Orthop, 1990. 10(2): p. 177-82.
24. Murray, C. and J.A. Fixsen, Management of knee deformity in classical arthrogryposis multiplex congenita (amyoplasia congenita). J Pediatr Orthop B, 1997. 6(3): p. 186-91.
25. Brunner, R., F. Hefti, and J.D. Tgetgel, Arthrogrypotic joint contracture at the knee and the foot: correction with a circular frame. J Pediatr Orthop B, 1997. 6(3): p. 192-7.
26. Thomas, B., et al., The knee in arthrogryposis. Clin Orthop Relat Res, 1985(194): p. 87-92.
27. Eriksson, M., et al., Gait in children with arthrogryposis multiplex congenita. J Child Orthop, 2010. 4(1): p. 21-31.
28. Palmer, P.M., et al., Passive motion therapy for infants with arthrogryposis. Clin Orthop Relat Res, 1985(194): p. 54-9.
29. Ponten, E., Management of the knees in arthrogryposis. J Child Orthop, 2015. 9(6): p. 465-72.
30. Drummond DS, S.T., Cruess RL, Management of arthrogryposis multiplex congenita. Instr Course Lect, 1974. 23: p. 79-95.
31. Ho, C.A. and L.A. Karol, The utility of knee releases in arthrogryposis. J Pediatr Orthop, 2008. 28(3): p. 307-13.
32. Palocaren, T., et al., Anterior distal femoral stapling for correcting knee flexion contracture in children with arthrogryposis–preliminary results. J Pediatr Orthop, 2010. 30(2): p. 169-73.
33. Klatt, J. and P.M. Stevens, Guided growth for fixed knee flexion deformity. J Pediatr Orthop, 2008. 28(6): p. 626-31.
34. Herzenberg, J.E., et al., Mechanical distraction for treatment of severe knee flexion contractures. Clin Orthop Relat Res, 1994(301): p. 80-8.
35. DelBello, D.A. and H.G. Watts, Distal femoral extension osteotomy for knee flexion contracture in patients with arthrogryposis. J Pediatr Orthop, 1996. 16(1): p. 122-6.
36. Yang, S.S., et al., Ambulation gains after knee surgery in children with arthrogryposis. J Pediatr Orthop, 2010. 30(8): p. 863-9.
37. Feldman, D.S., T.J. Rand, and A.J. Huser, Novel Approach to Improving Knee Range of Motion in Arthrogryposis with a New Working Classification. Children (Basel), 2021. 8(7).
38. van Bosse, H.J., et al., Treatment of knee flexion contractures in patients with arthrogryposis. J Pediatr Orthop, 2007. 27(8): p. 930-7.
39. Aman, M., et al., Comparative analysis of surgical treatment modalities for a popliteal pterygium: a meta-analysis. Arch Orthop Trauma Surg, 2024. 144(5): p. 2449-2459.
40. Curtis, B.H. and R.L. Fisher, Congenital hyperextension with anterior subluxation of the knee. Surgical treatment and long-term observations. J Bone Joint Surg Am, 1969. 51(2): p. 255-69.
41. Oetgen, M.E., et al., Functional results after surgical treatment for congenital knee dislocation. J Pediatr Orthop, 2010. 30(3): p. 216-23.
42. Shah, N.R., N. Limpaphayom, and M.B. Dobbs, A minimally invasive treatment protocol for the congenital dislocation of the knee. J Pediatr Orthop, 2009. 29(7): p. 720-5.
43. Johnson, E., R. Audell, and W.L. Oppenheim, Congenital dislocation of the knee. J Pediatr Orthop, 1987. 7(2): p. 194-200.
44. Roy, D.R. and A.H. Crawford, Percutaneous quadriceps recession: a technique for management of congenital hyperextension deformities of the knee in the neonate. J Pediatr Orthop, 1989. 9(6): p. 717-9.
45. Fucs, P.M., et al., Quadricepsplasty in arthrogryposis (amyoplasia): long-term follow-up. J Pediatr Orthop B, 2005. 14(3): p. 219-24.
46. Kowalczyk, B. and J. Felus, Arthrogryposis: an update on clinical aspects, etiology, and treatment strategies. Arch Med Sci, 2016. 12(1): p. 10-24.
47. Morcuende, J.A., M.B. Dobbs, and S.L. Frick, Results of the Ponseti method in patients with clubfoot associated with arthrogryposis. Iowa Orthop J, 2008. 28: p. 22-6.
48. Matar, H.E., P. Beirne, and N. Garg, The effectiveness of the Ponseti method for treating clubfoot associated with arthrogryposis: up to 8 years follow-up. J Child Orthop, 2016. 10(1): p. 15-8.
49. van Bosse, H.J.P., Challenging clubfeet: the arthrogrypotic clubfoot and the complex clubfoot. J Child Orthop, 2019. 13(3): p. 271-281.
50. Drummond, D.S. and R.L. Cruess, The management of the foot and ankle in arthrogryposis multiplex congenita. J Bone Joint Surg Br, 1978. 60(1): p. 96-9.
51. Niki, H., L.T. Staheli, and V.S. Mosca, Management of clubfoot deformity in amyoplasia. J Pediatr Orthop, 1997. 17(6): p. 803-7.
52. Guidera, K.J. and J.C. Drennan, Foot and ankle deformities in arthrogryposis multiplex congenita. Clin Orthop Relat Res, 1985(194): p. 93-8.
53. Chergui, S., et al., Talectomy for arthrogrypotic foot deformities: A systematic review. Foot Ankle Surg, 2023. 29(1): p. 15-21.
54. Segal, L.S., et al., Equinovarus deformity in arthrogryposis and myelomeningocele: evaluation of primary talectomy. Foot Ankle, 1989. 10(1): p. 12-6.
55. Eidelman, M. and A. Katzman, Treatment of arthrogrypotic foot deformities with the Taylor Spatial Frame. J Pediatr Orthop, 2011. 31(4): p. 429-34.
56. El Barbary, H., H. Abdel Ghani, and M. Hegazy, Correction of relapsed or neglected clubfoot using a simple Ilizarov frame. Int Orthop, 2004. 28(3): p. 183-6.
57. Chalayon, O., A. Adams, and M.B. Dobbs, Minimally invasive approach for the treatment of non-isolated congenital vertical talus. J Bone Joint Surg Am, 2012. 94(11): p. e73.
58. Bouchard, M., An Algorithmic Approach to the Congenital Vertical Talus. Journal of the Pediatric Orthopaedic Society of North America, 2022. 4(1): p. 398.

How to Cite this Article: Shah IP, Ranmuthuge S, Parnami V, Chytas A | Lower Limb Deformity Management in Arthrogryposis – What to Correct and When | International Journal of Paediatric Orthopaedics | May-August 2024; 10(2): 40-47.
https://doi.org/10.13107/ijpo.2024.v10.i02.192

(Article Text HTML) (Full Text PDF)

Management of Limb Deficiencies

Volume 10 | Issue 2 | May-August 2024 | Page: 48-54 | Sakti Prasad Das, Sankar Ganesh, Prateek Behera

DOI- https://doi.org/10.13107/ijpo.2024.v10.i02.194

Submitted: 11/03/2024; Reviewed: 08/04/2024; Accepted: 25/06/2024; Published: 10/08/2024

Authors: Sakti Prasad Das MS(Ortho.), DNB(PMR) [1], Sankar Ganesh MPT [2], Prateek Behera MS(Ortho.), DNB(Ortho.) [3]

[1] Medical Education & Training, DRIEMS University, Odisha, Tangi, Cuttack, Odisha, India.
[2] Department of Physiotherapy, Composite Regional Centre, Lucknow, Uttar Pradesh, India.
[3] Department of Orthopaedics, AIIMS Bhopal, Madhya Pradesh, India.

Address of Correspondence

Dr. Sakti Prasad Das,
Director, Medical Education & Training, DRIEMS University, Odisha, Tangi, Cuttack, Odisha, India.
E-mail: sakti2663@yahoo.com

Abstract

Limb deficiency disorders encompass a wide variety of congenital anomalies that have a significant underdevelopment or even complete absence of bones in the limbs. Treatment of these conditions must be holistic with the child at the centre. This article provides a review of the current understanding of the management of such conditions. Surgical treatment offers a practical and effective solution for treating many variants of congenital limb abnormalities. Although novel surgical treatments may expand the range of disorders that can be treated, it is crucial for both the surgeon and the family to be aware of the careful prognosis associated with the methods used. Additionally, the importance of an amputation as an option should always be kept under consideration.
Keywords: Amputation, Congenital Abnormalities, Deformity correction, Limb reconstruction, Pediatric skeletal deficiencies, Skeletal dysplasia

References

1. WHO. Congenital disorders. Geneva: WHO, 2023. Available: https://www.who.int/news-room/fact-sheets/detail/birth-defects
2. Moges N, Anley DT, Zemene MA, Adella GA, Solomon Y, Bantie B, et al. Congenital anomalies and risk factors in Africa: a systematic review and meta-analysis. BMJ paediatrics open, 2023;7(1), e002022. https://doi.org/10.1136/bmjpo-2023-002022
3. WHO. International statistical classification of diseases and related health problems (ICD)-11. Geneva WHO. 2023. Available: https://www.who.int/classifications/classification-of-diseases
4. Epps CH Jr. Proximal femoral focal deficiency. J Bone Joint Surg Am 1983; 65: 867–70.
5. Kakarla S. Proximal femoral focal deficiency (PFFD) imaging spectrum. J Med Sci Res. 2015;3(2):90–93. doi: 10.17727/JMSR.2015/3-018
6. Paley D, Guardo F. Lengthening reconstruction surgery for congenital deficiency. In: Kocaoglu M, Tsuchiya H, Eralp L, editors. Advanced techniques in limb reconstruction surgery. 2014. pp. 245–298.
7. Gupta SK, Alassaf N, Harrop AR, Kiefer GN. Principles of rotationplasty. J Am Acad Orthop Surg. 2012;20:657–667. doi: 10.5435/JAAOS-20-10-657
8. Ackman J, Altiok H, Flanagan A, Peer M, Graf A, Krzak J, et al. Long-term follow-up of Van Nes rotationplasty in patients with congenital proximal focal femoral deficiency. Bone Joint J. 2013;95-B(2):192–198. doi: 10.1302/0301-620X.95B2.30853
9. Jones D, Barnes J, Lloyd-Roberts GC. Congenital aplasia and dysplasia of the tibia with intact fibula. Classification and management. J Bone Joint Surg Br. 1978;60(1):31-39. doi:10.1302/0301-620X.60B1.627576
10. Kalamchi A., Dawe R.W. Congenital deficiency of the tibia. J. Bone Jt. Surg. Br. 1985;67:581–584. doi: 10.1302/0301-620X.67B4.4030854
11. Weber M. New classification and score for tibia hemimelia. J Child Orthop. 2008;2:169–175.
12. Fernandez-Palazzi F, Bendahan J, Rivas S. Congenital deficiency of the tibia: a report on 22 cases. J Pediatr Orthop B. 1998;7:298–302.
13. Epps C.H., Jr., Schneider P. Treatment of hemimelias of the lower extremity. Long–term results. J. Bone Jt. Surg. Am. Vol. 1989;71:273–277. doi: 10.2106/00004623-198971020-00015
14. Putti V. The treatment of congenital absence of the tibia or fibula. Chir. Org. Mov. 1929;7:513.
15. Paley D. Surgical reconstruction for fibular hemimelia. J. Child. Orthop. 2016;10:557–583. doi: 10.1007/s11832-016-0790-0
16. Paley D., Robbins C. Fibular hemimelia Paley type 3. In: Rozbruch S.R., Hamdy R., editors. Limb Lengthening and Reconstruction Surgery Case Atlas. 1st ed. Springer International Publishing; Cham, Switzerland: 2015. pp. 1–8.
17. Johnson CE, Haideri NF. Comparison of functional outcome in fibular deficiency treated by limb salvage versus Syme’s amputation. In: Herring JA, Birch JG, eds. The Child With a Limb Deficiency. Rosemont: American Academy of Orthopaedic Surgeons; 1998: 173–177.
18. Herzenberg J., Shabtai L, Standard SC. Fibular hemimelia: Principles and techniques of management. In: Sabharwal S., editor. Pediatric Lower Limb Deformities, Principles and Techniques of Management. 1st ed. Springer International Publishing; Cham, Switzerland: 2016. pp. 427–454.
19. Birch JG, Lincoln TL, Mack PW, et al. Congenital fibular deficiency: a review of thirty years’ experience at one institution and a proposed classification system based on clinical deformity. J Bone Joint Surg Am. 2011;93:1144–1151.
20. Ali S, Kaplan S, Kaufman T, Fenerty S, Kozin S, Zlotolow DA. Madelung deformity and Madelung-type deformities: a review of the clinical and radiological characteristics. Pediatr Radiol. 2015;45(12):1856-1863. doi:10.1007/s00247-015-3390-0
21. Nielsen JB. Madelung’s deformity. A follow-up study of 26 cases and a review of the literature. Acta Orthop Scand. 1977;48(4):379-384. doi:10.3109/17453677708992012
22. Farr S, Martinez-Alvarez S, Little KJ et al (2021) The prevalence of Vickers’ ligament in Madelung’s deformity: a retrospective multicentre study of 75 surgical cases. J Hand Surg Eur. https://doi. org/ 10. 1177/ 17531 93420 981522
23. Vickers D, Nielsen G. Madelung deformity: surgical prophylaxis (physiolysis) during the late growth period by resection of the dyschondrosteosis lesion. J Hand Surg Br. 1992;17(4):401-407.
24. Laffosse JM, Abid A, Accadbled F, Knör G, de Gauzy JS, Cahuzac JP. Surgical correction of Madelung’s deformity by combined corrective radioulnar osteotomy: 14 cases with four-year minimum follow-up. IntOrthop. 2009; 33: 1655-1661.
25. Steinman S, Oishi S, Mills J, Bush P, Wheeler L, Ezaki M. Volar ligament release and distal radial dome osteotomy for the correction of Madelung deformity: long-term follow-up. J Bone Joint Surg Am. 2013;95(13):1198-1204. doi:10.2106/JBJS.L.00714
26. Colen DL, Lin IC, Levin LS, Chang B. Radial longitudinal deficiency: recent developments, controversies, and an evidence-based guide to treatment. J Hand Surg Am. 2017 Jul;42(7):546-563. https://doi.org/10.1016/j.jhsa.2017.04.012
27. Bednar MS, James MA, Light TR. Congenital longitudinal deficiency. J Hand Surg Am. 2009 Nov;34(9):1739-1747. https://doi.org/10.1016/j.jhsa.2009.09.002
28. Stutz C, Oishi S. Radial longitudinal deficiency: radius hypoplasia. In: Laub Jr DR, ed. Congenital Anomalies of the Upper Extremity. New York: Springer; 2015:85-94.
29. Takagi T, Seki A, Mochida J, Takayama S. Bone lengthening of the radius with temporary external fixation of the wrist for mild radial club hand. J Plast Reconstr Aesthetic Surg. 2014;67:1688e1693. https://doi.org/10.1016/ j.bjps.2019.05.044
30. Buck-Gramcko D. Radialization as a new treatment for radial club hand. J. Hand Surg. Am. 1985;10(6 Pt 2):964–968. doi: 10.1016/S0363-5023(85)80013-7
31. Wall LB, Ezaki M, Oishi SN. Management of congenital radial longitudinal deficiency: controversies and current concepts. Plast Reconstr Surg. 2013 Jul;132(1):122-128.
32. Cole RJ, Manske PR .Classification of ulnar deficiency according to the thumb and first web.. J Hand Surg Am. 1997;22:479–488.

How to Cite this Article: Das SP, Ganesh S, Behera P | Management of Limb Deficiencies | International Journal of Paediatric Orthopaedics | May-August 2024; 10(2): 48-54. https://doi.org/10.13107/ijpo.2024.v10.i02.194

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Lower Limb Deformity Management in Arthrogryposis – What to Correct and When

Management of Limb Deficiencies

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Management of Limb Deficiencies

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