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.

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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

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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

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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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Management of Complex Foot Deformities in Children

Volume 10 | Issue 2 | May-August 2024 | Page: 34-39 | Sagar Umerjikar, Abhishek V. Mundargi, Koushik N. Subramanyam

DOI- https://doi.org/10.13107/ijpo.2024.v10.i02.190

Submitted: 25/05/2024; Reviewed: 24/06/2024; Accepted: 21/07/2024; Published: 10/08/2024

Authors: Sagar Umerjikar MS Ortho [1], Abhishek V. Mundargi MS Ortho [1], Koushik N. Subramanyam MS Ortho [1]

[1] Department of Orthopaedics, Sri Sathya Sai Institute of Higher Medical Sciences, Prasanthigram, Puttaparthi, Andhra Pradesh, India.

Address of Correspondence

Dr. Koushik Narayan Subramanyam ,
Department of Orthopaedics, Sri Sathya Sai Institute of Higher Medical Sciences, Prasanthigram, Puttaparthi, Andhra Pradesh, India.
E-mail: koushik.n@sssihms.org.in

Abstract

Aim: The aim of this article is to understand the intricacies of various complex foot deformities in children and non-operative and operative strategies in managing them by reviewing data from published literature.
Methods: A review of information pertaining to complex foot deformities in children was assimilated for holistic understanding of the condition along with their management from various sources from PubMed and Google Scholar.
Conclusion: Treatment of pediatric foot and ankle deformities is complex and must be individualized. The inter-individual phenotypic variability demands a personalized planning of each foot to be operated. Factors to consider are age at presentation, primary pathology causing the deformity and the underlying complex pathoanatomy. Treatment must also assess the possibility of relapse of the deformity and measures must be incorporated to prevent the same.

Keywords: 

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16. Horsch A, Petzinger L, Ghandour M, Putz C, Renkawitz T, Götze M. Defining Equinus Foot in Cerebral Palsy. Children (Basel). 2022 Jun 25;9(7):956. doi: 10.3390/children9070956. PMID: 35883940; PMCID: PMC9320304.
17. Kadhim M, Holmes L Jr, Church C, Henley J, Miller F. Pes planovalgus deformity surgical correction in ambulatory children with cerebral palsy. J Child Orthop. 2012 Jul;6(3):217-27. doi: 10.1007/s11832-012-0413-3. Epub 2012 Jun 20. PMID: 23814622; PMCID: PMC3400002.
18. Hochstetter-Owen J, Stott S, Williams SA. The efficacy of split tibial tendon transfers on functional gait outcomes for children and youth with cerebral palsy and spastic equinovarus foot deformities. Bone Jt Open. 2023 May 1;4(5):283-298. doi: 10.1302/2633-1462.45.BJO-2023-0005.R1. PMID: 37121581; PMCID: PMC10149292.
19. Wirth T. Congenital Vertical Talus. Foot Ankle Clin. 2021 Dec;26(4):903-913. doi: 10.1016/j.fcl.2021.08.002. Epub 2021 Oct 7. PMID: 34752243.
20. Altaf KA, Shah SBS, Ahmad S, Mumtaz U, Mantoo SA. Results of JESS (Joshi’s External Stabilizing System) in Relapsed, Neglected and Neurogenic Clubfoot in an Age Group of 2-10 Years. Ortop Traumatol Rehabil. 2020 Apr 30;22(2):121-129. doi: 10.5604/01.3001.0014.1170. PMID: 32468991.

How to Cite this Article: Umerjikar S, Mundargi AV, Subramanyam KN | Management  of Complex Foot Deformities in Children | International Journal of Paediatric Orthopaedics | May-August 2024; 10(2): 34-39.

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Radiological Assessment and Planning of Deformities

Volume 10 | Issue 2 | May-August 2024 | Page: 12-17 | Sudhanshu Bansal, Gaurav Gupta, Deepak Khurana 

DOI- https://doi.org/10.13107/ijpo.2024.v10.i02.184

Submitted: 05/03/2024; Reviewed: 02/04/2024; Accepted: 15/05/2024; Published: 10/08/2024

Authors: Sudhanshu Bansal MS Ortho [1], Gaurav Gupta MS Ortho [2], Deepak Khurana MS Ortho [3]

[1] Department of Paediatric Orthopaedic Surgery, CODS Clinic, Ludhiana, Amandeep Hospital, Amritsar, Punjab, India.
[2] Department of Paediatric Orthopaedic Surgery, Child Ortho Clinic, Delhi-NCR, India.
[3] Department of Paediatric Orthopaedic Surgery, JCPODS, Jeevan Rekha hospital, Jaipur, Rajasthan, India.

Address of Correspondence

Dr. Sudhanshu Bansal,
Consultant, Paediatric Orthopaedic Surgeon, CODS Clinic, Ludhiana, Amandeep Hospital, Amritsar, Punjab, India.
E-mail: drbansalsudhanshu@gmail.com

Abstract

Deformity correction is a fundamental aspect of orthopedic surgery, requiring a precise radiological assessment and systematic planning. This article provides an indepth overview of the radiological modalities available for deformity assessment, including X-rays, scannograms, computed tomography (CT), and magnetic resonance imaging (MRI). Proper radiographic techniques, such as standing fulllength X-rays and scannograms, are critical for accurate limb alignment assessment. Advanced imaging modalities, including CT and MRI, are necessary in cases of complex deformities or rotational abnormalities. Furthermore, various software applications are available for preoperative deformity planning, enabling precise correction strategies. This article also addresses approaches for managing deformities in limited-resource settings, emphasizing cost-effective and accessible imaging techniques. The objective is to enhance clinical decisionmaking and optimize surgical outcomes in deformity management.
Keywords: Alignment, Anatomic tibiofemoral angle, Deformity, Knee, Lower limb alignment, Mechanical axis angle, Radiography

References

1. Sabharwal, S.; Zhao, C.; McKeon, J.; Melaghari, T.; Blacksin, M.; Wenekor, C. Reliability analysis for radiographic measurement of limb length discrepancy: Full-length standing anteroposterior radiograph versus scanogram. J. Pediatr. Orthop. 2006, 27, 46–50.
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12. Solomin, L.N.; Utekhin, A.I.; Vilenskiy, V.A. Reference values of the femur and tibia mechanical axes and angles in the sagittal plane, determined on the basis of three-dimensional modeling. J. Limb Lengthen Reconstr. 2020, 6, 116–120. 
13. Guggenberger, R.; Pfirrmann, C.W.; Koch, P.P.; Buck, F.M. Assessment of lower limb length and alignment by biplanar linear radiography: Comparison with supine CT and upright fulllength radiography. AJR Am. J. Roentgenol. 2014, 202, W161–W167 
14. Fürmetz, J.; Sass, J.; Ferreira, T.; Jalali, J.; Kovacs, L.; Mück, F.; Degen, N.; Thaller, P.H. Three-dimensional assessment of lower limb alignment: Accuracy and reliability. Knee 2019, 26, 185–193. 
15. Ahrend, M.D.; Baumgartner, H.; Ihle, C.; Histing, T.; Schröter, S.; Finger, F. Influence of axial limb rotation on radiographic lower limb alignment: A systematic review. Arch. Orthop. Trauma Surg. 2022, 142, 3349–3366. 
16. Buck FM, Guggenberger R, Koch pp, Pfirrmann CWA. Femoral and tibial torsion measurements with 3D models based on low-dose biplanar radiographs in comparison with standard CT measurements. AJR Am J Roentgenol 2012;199:W607–W612.
17. Kuiper, R.J.A.; Seevinck, P.R.; Viergever, M.A.; Weinans, H.; Sakkers, R.J.B. Automatic Assessment of Lower-Limb Alignment from Computed Tomography. J. Bone Jt. Surg. Am. 2023, 105, 700–712. 
18. Simon, S.; Schwarz, G.M.; Aichmair, A.; Frank, B.J.H.; Hummer, A.; DiFranco, M.D.; Dominkus, M.; Hofstaetter, J.G. Fully automated deep learning for knee alignment assessment in lower extremity radiographs: A cross-sectional diagnostic study. Skeletal Radiol. 2022, 51, 1249– 1259. 

How to Cite this Article:  Bansal S, Gupta G, Khurana D | Radiological Assessment and Planning of Deformities | International Journal of Paediatric Orthopaedics | May-August 2024; 10(2): 12-17. https://doi.org/10.13107/ijpo.2024.v10.i02.184

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Basics of Paediatric Limb Reconstruction Surgeries

Volume 10 | Issue 2 | May-August 2024 | Page: 2-11 | Prateek Rastogi, Nitish Arora, Yogesh Patel 

DOI- https://doi.org/10.13107/ijpo.2024.v10.i02.182

Submitted: 18/05/2024; Reviewed: 14/06/2024; Accepted: 19/07/2024; Published: 10/08/2024

Authors: Prateek Rastogi MS Ortho [1], Nitish Arora MS Ortho [2], Yogesh Patel MS Ortho [3]

[1] Department of Orthopaedics, Sharda Hospital, Greater Noida, Uttar Pradesh, India.
[2] Department of Orthopaedics, Medicover Hospital, Khargar, Navi Mumbai, Maharashtra, India.
[3] Department of Orthopaedics, Sagar Multispeciality Hospital, Bhopal, Madhya Pradesh, India.

Address of Correspondence

Dr. Prateek Rastogi,
Paediatric Orthopaedics and Limb Reconstruction Surgeon, Department of Orthopaedics, Sharda Hospital, Greater Noida, Uttar Pradesh, India.
E-mail: prateek.rastogi12@gmail.com

Abstract

Paediatric limb reconstruction surgeries play a pivotal role in managing congenital and acquired deformities, limb length discrepancies, and complex musculoskeletal disorders in children. These procedures aim to restore alignment, function, and length while preserving growth potential and minimizing long-term disability. Unlike adult cases, paediatric reconstructions demand unique considerations due to ongoing skeletal development, necessitating precise planning to avoid growth plate damage. This review outlines the evolving indications for reconstruction—including congenital conditions like various hemimelia and bony deficiency, as well as acquired deformities from trauma, infection, and tumors. Foundational principles such as anatomical and mechanical axes and their deviation, CORA (Center of Rotation of Angulation), and ACA (Angulation Correction Axis) are discussed alongside osteotomy planning and execution. Techniques of gradual deformity correction such as growth modulation, and distraction osteogenesis are examined in depth, highlighting the roles of devices like Ilizarov fixators, hexapods, and intramedullary lengthening nails. Recent advancements in imaging, surgical planning, and implant design have significantly improved outcomes, although complications such as joint stiffness, infection, and secondary deformities persist. With increasing precision and a growing array of tools, paediatric limb reconstruction continues to evolve, offering promising outcomes and functional restoration to affected children.
Keywords: Paediatric limb reconstruction, Deformity Correction, Limb Lengthening, Growth Modulation, Distraction Osteogenesis, Osteotomy Techniques

References

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12. Boero S, Riganti S, Marrè Brunenghi G, Nasto LA. Hexapod External Fixators in Paediatric Deformities. In: Massobrio M, Mora R, eds. Hexapod External Fixator Systems. Springer International Publishing; 2021:133-152. doi:10.1007/978-3-030-40667-7_8
13. Georgiadis AG, Rossow JK, Laine JC, Iobst CA, Dahl MT. Plate-assisted Lengthening of the Femur and Tibia in Pediatric Patients. Journal of Pediatric Orthopaedics. 2017;37(7):473-478. doi:10.1097/BPO.0000000000000645
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How to Cite this Article:  Rastogi P, Arora N, Patel Y| Basics of Paediatric Limb Reconstruction Surgeries| International Journal of Paediatric Orthopaedics | May-August 2024; 10(2): 02-11. https://doi.org/10.13107/ijpo.2024.v10.i02.182

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Editorial

Volume 10 | Issue 2 | May-August 2024 | Page: 01 | Jayanth S. Sampath

DOI- https://doi.org/10.13107/ijpo.2024.v10.i02.180

Authors: Jayanth S. Sampath FRCSEd (Tr & Orth) [1]

[1] Department of Orthopaedics, Rainbow Children’s Hospital, Bangalore, Karnataka, India.

Address of Correspondence

Dr. Jayanth S. Sampath,
Rainbow Children’s Hospital, Bangalore, Karnataka, India.
E-mail: editor.posi.ijpo@gmail.com

Editorial

Limb reconstruction surgery in children has evolved into the treatment of choice for complex deformities of the limb and foot. The power of gradual correction with stable fixation devices of varying complexity have overcome the limitations of traditional open surgery. It is an important and potent skill in the hands of the paediatric orthopaedic surgeon.
This issue of IJPO presents an overview of the principles and practice in modern limb reconstruction surgery. We start with an article on basic deformity correction principles followed by a detailed description of radiological assessment of deformity. The subsequent articles provide a comprehensive overview of the management of Blount disease, hip instability, complex foot deformities, arthrogryposis and limb deficiencies. We are proud that the authors are from institutions across the world, each offering a unique perspective to the management of these difficult problems. Please share the articles with your trainees and fellows. IJPO issues are easily downloadable free of charge and in full-text format from our website www.ijpoonline.com
We invite your suggestions and comments for any improvements to the journal. Kindly write to us editor.ijpo@gmail.com or editor@posi.org.in
It is my pleasure to acknowledge the contributions of authors, reviewers, editors, and the backend team who have been instrumental in bringing out this issue. The cover page artwork by Dr Easwar T R, POSI Webmaster illustrates the correction of a child with tibia vara using an external fixator. It emphasises that the most successful treatments in our armamentarium are not necessarily complicated but are based on a firm understanding of the pathological anatomy of deformity and the application of a standardised method in practice.

Sincerely


Dr Jayanth S Sampath
Editor,

International Journal of Paediatric Orthopaedics

 

 

How to Cite this Article:  Sampat JS | Editorial | International Journal of Paediatric Orthopaedics | May-August 2024;10(2): 01. https://doi.org/10.13107/ijpo.2024.v10.i02.180

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