Tag Archive for: Arthrogryposis

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

Volume 10 | Issue 2 | May-August 2024 | Page: 00-00 | Ishani P Shah, Sujika Ranmuthuge, Varun Parnami, Anastasios Chytas 

DOI- https://doi.org/10.13107/ijpo.2024.v10.i02.000

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: 


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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): 09-16.
https://doi.org/10.13107/ijpo.2024.v10.i02.184

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Bruck Syndrome (Bone Fragility with Congenital Joint Contractures): A Case Report

Volume 7 | Issue 3 | September-December 2021 | Page: 29-34 | Gaurav Gupta, Qaisur Rabbi, Maulin Shah, Vikas Bohra
DOI-10.13107/ijpo.2021.v07i03.120


Authors: Deepika A. Pinto MS DNB Orth. [1], Sandeep V. Vaidya MS DNB Orth. [1]

[1] Department of Paediatric Orthopaedics, Pinnacle Ortho Center, LBS Road, Thane West, Maharashtra, India.

Address of Correspondence
Dr. Deepika A. Pinto,
Clinical Associate in Paediatric Orthopaedics, Pinnacle Ortho Center, LBS Road, Thane West, Maharashtra, India.
E-mail: deepupinto@gmail.com


Abstract

Background: Bruck syndrome is a disorder in which features of osteogenesis imperfecta and arthrogryposis multiplex congenita coexist. It is an extremely rare condition with less than 40 cases reported worldwide.
Case presentation: We describe the case of a girl child, born of a consanguineous marriage, who, at birth, was noted to have flexion contractures of both knees and elbows as well as right clubfoot. Post-natally, she developed repetitive fractures of both femurs occurring with trivial trauma. She presented to us at the age 8.5 years, with short stature and inability to stand due to the severe knee flexion contractures. She also had flexion contractures at bilateral elbows. Intelligence and fine motor skills were normal. Sclerae, teeth and hearing were also normal. Radiographs revealed osteoporosis, severely deformed femora and vertebral body flattening. A diagnosis of Bruck syndrome was made on the basis of clinical findings. Genetic testing was offered, but declined by the child’s parents. She has since undergone osteotomies and rodding for both femurs, and bilateral distal femoral anterior hemi-epiphysiodesis for gradual correction of knee contractures. She is on cyclical pamidronate therapy to address bone fragility.
Conclusion: In this report, we describe the diagnostic features and management of this rare syndrome, and provide a summary of the existing literature on the disorder.
Keywords: Bruck syndrome, Osteogenesis imperfecta, Arthrogryposis, Congenital joint contractures, Bone fragility


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How to Cite this Article:  Pinto AD, Vaidya SV | Bruck Syndrome (Bone Fragility with Congenital Joint Contractures): A Case Report. | September-December 2021; 7(3): 29-34.

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