Management of Limb Deformities in Skeletal Dysplasia

Volume 11 | Issue 1 | Januar-April 2025 | Page: 44-50 | Binu T Kurian, Aditi Pinto, James A Fernandes

DOI- https://doi.org/10.13107/ijpo.2025.v11.i01.222

Open Access License: CC BY-NC 4.0

Copyright Statement: Copyright © 2025; The Author(s).

Submitted: 29/01/2025; Reviewed: 22/02/2025; Accepted: 17/03/2025; Published: 10/04/2025

Authors: Binu T Kurian MS Ortho [1], Aditi Pinto MS Ortho [1], James A Fernandes FRCS Tr & Ortho [2]

[1] Department of Orthopaedics, St. John’s Medical College, Hospital, Bangalore, Karnataka, India.
[2] Department of Orthopaedics, Sheffield Children’s Hospital NHS Trust Sheffield S10 4 NH United Kingdom

Address of Correspondence

Dr. Binu T Kurian,
Department of Orthopaedics St. John’s Medical College, Hospital, Bangalore, Karnataka, India.
E-mail: binu.k@stjohns.in

Abstract

Skeletal Dysplasia encompasses a diverse group of genetic conditions that predominantly affect bone and cartilage formation. The clinical assessment of these conditions requires a detailed family history to assess the genetic inheritance patterns and physical examination of the limb length discrepancies, joint laxity, spinal alignment, and gait abnormalities to identify deviations from normal skeletal development. Pharmacological therapy consists of Bisphosphonates to improve the bone density in conditions like osteogenesis imperfecta. Surgical procedures include Growth modulation techniques, limb lengthening procedures, corrective osteotomies and joint reconstructions. A Detailed genetic counselling is key to parents with a history of skeletal dysplasia in the family to assess recurrence risks, explore reproductive options, and guide personalized treatment strategies. With advancements in gene therapy, 3D navigation and patient specific implants and prosthesis there is a promising future in the management of limb deformities in Skeletal dysplasia
Keywords: Skeletal Dysplasia, Lower limb deformity, Guided Growth, Limb Lengthening

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How to Cite this Article:  Kurian BT, Pinto A, Fernandes JA. Management of Limb Deformities in Skeletal Dysplasia. International Journal of Paediatric Orthopaedics. January-April 2025; 11(1): 44-50 .

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Radiological Diagnosis of Skeletal Dysplasias in Children

Volume 11 | Issue 1 | Januar-April 2025 | Page: 6-16 | Sangeet Gangadharan, Samantha Low, James Fernandes, Ishandeep Singh

DOI- https://doi.org/10.13107/ijpo.2025.v11.i01.214

Open Access License: CC BY-NC 4.0

Copyright Statement: Copyright © 2025; The Author(s).

Submitted: Submitted: 02/01/2025; Reviewed: 29/01/2025; Accepted: 23/03/2025; Published: 10/04/2025

Authors: Sangeet Gangadharan MBBS, DNB Orth [1], Samantha Low MBBS, FRCR [2], James Fernandes MBBS, FRCS (Tr & Orth) [3], Ishandeep Singh BSc [4]

[1] Department of Paediatric Orthopaedics, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK
[2] Department of Radiology, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK
[3] Department of Trauma and Orthopaedics, Sheffield Children’s Hospital NHS Foundation Trust, Sheffield, UK
[4] Norwich Medical School, University of East Anglia, Norwich, UK

Address of Correspondence

Dr. Sangeet Gangadharan,
Department of Paediatric Orthopaedics, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK
E-mail: drsangeetgangadharan@gmail.com

Abstract

Radiological diagnosis of skeletal dysplasias in children relies on early recognition of disproportionate growth and characteristic imaging patterns across the skeleton. Skeletal dysplasias are a heterogenous group of genetic disorders of bone and cartilage with a combined birth prevalence of around 1 in 5,000 births. Early radiographic identification guides surveillance for spinal stenosis, craniovertebral anomalies, hip dysplasia and blood disorders. It also supports genetic counselling and, in some conditions, allows targeted therapies such as enzyme replacement or growth-modifying treatment. This chapter outlines key clinical clues that should alert paediatric orthopaedic surgeons to an underlying skeletal dysplasia and summarises indications and techniques for a dedicated dysplasia skeletal survey, including bone age assessment and targeted supplementary views. Furthermore, core radiological terminology and measurements used to describe disproportions and dysplastic change, are received to support systematic pattern recognition. The chapter then describes the principal clinical and radiological hallmarks of common dysplasias relevant to paediatric orthopaedic practice, including achondroplasia and hypochondroplasia, pseudoachondroplasia and multiple epiphyseal dysplasia, spondyloepiphyseal dysplasia congenita, diastrophic dysplasia, osteogenesis imperfecta, sclerosing bone dysplasias, mucopolysaccharidoses and metaphyseal chondrodysplasias, highlighting key differential diagnoses and red flag complications. The role of advanced imaging, particularly at the craniovertebral junction, cervical spine and hips is emphasised where there is risk of cord compression, atlantoaxial instability or early degenerative change. Finally, the importance of multidisciplinary assessment, integrating clinical genetics, endocrinology, radiology, anesthesia and surgical specialties is stressed to minimise misdiagnosis and optimise long-term functional outcomes. Prenatal ultrasound and cross sectional imaging features that raise suspicion of skeletal dysplasia are summarised, underscoring opportunities for early counselling, delivery planning and postnatal evaluation.
Keywords: Skeletal Dysplasia Radiology, Achondroplasia, Hypochondroplasia, MED, Storage disorders Radiology

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How to Cite this Article:  Gangadharan S, Low S, Fernandes J, Singh I. Radiological Diagnosis of Skeletal Dysplasias in Children. International Journal of Paediatric Orthopaedics. January-April 2025; 11(1): 06-16.

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A Case of Osteofibrous Dysplasia Treated with a Hybrid Approach: Clinical and Radiological Outcomes

Volume 10 | Issue 3 | September-December 2024 | Page: 33-38 | Vivek Madankar, K. Ramkumar Reddy, Roshan Kumar Mohanty

DOI- https://doi.org/10.13107/ijpo.2024.v10.i03.208

Open Access License: CC BY-NC 4.0

Copyright Statement: Copyright © 2024; The Author(s).

Submitted: 15/11/2024; Reviewed: 22/11/2024; Accepted: 01/12/2024; Published: 10/12/2024

Authors: Vivek Madankar MS Ortho [1], K. Ramkumar Reddy MS Ortho [1], Roshan Kumar Mohanty MS Ortho [1]

[1] Department of Orthopaedics, Kakatiya Medical College, Warangal, Telangana, India.

Address of Correspondence

Dr. Vivek Madankar,
Department of Orthopaedics, Kakatiya Medical College, Warangal, Telangana, India.
E-mail: vivekmadankar@gmail.com

Abstract

Introduction: Osteofibrous dysplasia (OFD) is a rare, benign, and self-limiting fibro-osseous lesion primarily affecting the cortical bone of the tibia and fibula in children and adolescents. OFD presents diagnostic challenges due to its clinical and radiological resemblance to adamantinoma, a malignant bone tumor. Accurate diagnosis and appropriate treatment are essential to prevent complications and recurrence.
Case Report: We report the case of an 11-year-old boy with a four-month history of pain and a longstanding swelling in his right tibia. Clinical examination revealed a firm, well-circumscribed lesion in the tibial cortex. Imaging demonstrated a mixed sclerotic and lytic lesion with characteristic features, including a multilocular “soap bubble” appearance. Histopathological evaluation confirmed the diagnosis of OFD. The patient underwent a hybrid treatment approach involving surgical excision sparing the posterior cortex, followed by functional cast bracing. This strategy facilitated early mobilization and complete recovery. Radiological evaluation at six weeks demonstrated neo-osteogenesis, with full bone healing achieved by 12 weeks. At the two-year follow-up, the patient exhibited full functional recovery with no signs of recurrence.
Conclusion: This case highlights the successful management of OFD using a hybrid surgical and non-surgical approach, emphasizing the importance of accurate diagnosis and tailored treatment strategies. Combining surgical resection with functional bracing allowed for effective management, early weight-bearing, and prevention of complications. This approach adds to the orthopedic literature by illustrating an alternative, cost-effective treatment protocol for this rare bone disorder.
Keywords: Osteofibrous dysplasia, Tibial lesion, Hybrid treatment, Surgical excision, Functional cast bracing, Pediatric orthopedics

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13. Treurniet S, Eekhoff EM, Schmidt FN, Micha D, Busse B, Bravenboer N. A clinical perspective on advanced developments in bone biopsy assessment in rare bone disorders. Front Endocrinol.2020;11:399-410.
14. Westacott D, Kannu P, Stimec J, Hopyan S, Howard A. Osteofibrous dysplasia of the tibia in children: outcome without resection. J Pediatr Orthop. 2019;39(8): 614-621.
15. Kamal AF, Anshori F, Kodrat E. Osteofibrous dysplasia-like adamantinoma versus osteofibrous dysplasia in children: a case report of challenging diagnosis. Int J Surg Case Rep. 2021;80:e105599.
16. Scholfield DW, Sadozai Z, Ghali C, Sumathi V, Douis H, Gaston L, Grimer RJ, Jeys L. Does osteofibrous dysplasia progress to adamantinoma and how should they be treated?. Bone Joint J. 2017;99(3):409-416.
17. Parra O, Linos K. Molecular pathogenesis of soft tissue and bone tumors. Diagnostic Molecular Pathology. Academic Press.2024:485-551.
18. Agaram NP. Molecular genetics of bone tumors. Diagn Histopathol. 2022;28(12):541-551.

How to Cite this Article:  Madankar V, Reddy KR, Kumar R | A Case of Osteofibrous Dysplasia Treated with a Hybrid Approach: Clinical and Radiological Outcomes | International Journal of Paediatric Orthopaedics | September-December 2024; 10(3): 33-38.

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A New Technique in the Treatment of Congenital Vertical Talus: A Case Series of Four Patients

Volume 10 | Issue 3 | September-December 2024 | Page: 25-32 | Harsharan Singh Oberoi, Baldish Singh Oberoi

DOI- https://doi.org/10.13107/ijpo.2024.v10.i03.206

Open Access License: CC BY-NC 4.0

Copyright Statement: Copyright © 2024; The Author(s).

Submitted: 21/11/2024; Reviewed: 23/11/2024; Accepted: 01/12/2024; Published: 10/12/2024

Authors: Harsharan Singh Oberoi MS, DNB Ortho [1], Baldish Singh Oberoi MS Ortho, MPH [2]

[1] Department of Orthopaedics, Oberoi Hospital, Jalandhar City, Punjab, India.

Address of Correspondence

Dr. Baldish Singh Oberoi
Consultant Orthopaedic Surgeon, Oberoi Hospital, Jalandhar City, Punjab, India.
Email: baldishoberoi@gmail.com

Abstract

Congenital Vertical talus is a rare condition present at birth that occurs in 1 in 10000 live births. We present a case series of four patients that we treated by the minimal invasive procedure followed by serial casting. Satisfactory correction, both cosmetic and functional, was achieved in all the cases. These results indicate that minimal invasive surgery followed by serial casting is a good way to correct the deformity in patients presenting early. Often, Vertical Talus patients are corrected by extensive surgical procedures and wire fixations leading to complications and resulting in a stiff and painful foot. We have devised a minimally invasive procedure without any wire fixation for patients presenting early with satisfactory results.
Keywords: Congenital Vertical talus, Foot abduction Orthosis, Talo-calcaneal angle

References

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11. Seimon LP. Surgical correction of congenital vertical talus under the age of 2 years. J Pediatr Orthop. 1987 Jul-Aug;7(4):405-11.
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13. Alaee F, Boehm S, Dobbs MB. A new approach to the treatment of congenital vertical talus. J Child Orthop. 2007 Sep;1(3):165-74.
14. Zorer G, Bagatur AE, Dogan A. Single stage surgical correction of congenital vertical talus by complete subtalar release and peritalar reduction by using the Cincinnati incision. J Pediatr Orthop B. 2002 Jan;11(1):60-7.
15. Miller M, Dobbs MB. Congenital Vertical Talus: Etiology and Management. J Am Acad Orthop Surg. 2015 Oct;23(10):604-11.
16. Sehmi S. Etiology and Treatment of Congenital Vertical Talus: A Clinical Review. AMEI’s Curr Trends Diagn Treat 2020;4(2):91–94.
17. Giannestras NJ. Recognition and treatment of flatfeet in infancy. Clin Orthop Relat Res. 1970 May-Jun;70:10-29.
18. Silk FF, Wainwright D. The recognition and treatment of congenital flat foot in infancy. J Bone Joint Surg Br. 1967 Nov;49(4):628-33.
19. Hafez M, Davis N. Outcomes of a Minimally Invasive Approach for Congenital Vertical Talus With a Comparison Between the Idiopathic and Syndromic Feet. J Pediatr Orthop. 2021 Apr 1;41(4):249-254.
20. Doski J. Current lines and angles used in paediatric foot radiograph: a scoping review of literature. Ann Ib Postgrad Med. 2024 Apr 30;22(1):51-61.

How to Cite this Article:  Oberoi HS, Oberoi BS | A New Technique in the Treatment of Congenital Vertical Talus: A Case Series of Four Patients | International Journal of Paediatric Orthopaedics | September-December 2024; 10(3): 25-32.

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Tibial Tuberosity Fractures in Adolescents: Anatomo – Clinical Assessment and Treatment Results

Volume 10 | Issue 3 | September-December 2024 | Page: 12-18 | Ake YL, Ouattara SJJ, Bonny R, Kouassi-Dria AKS

DOI- https://doi.org/10.13107/ijpo.2024.v10.i03.202

Open Access License: CC BY-NC 4.0

Copyright Statement: Copyright © 2024; The Author(s).

 Submitted: 28/10/2024; Reviewed: 10/11/2024; Accepted: 03/12/2024; Published: 10/12/2024

Authors: Ake YL [1], Ouattara SJJ [1], Bonny R [1], Kouassi-Dria AKS [1]

[1] Department of Paediatric Surgery, Cocody Teaching Hospital, Abidjan, Ivory Coast

Address of Correspondence

Dr. Ake Yapi Landry
Associate Lecturer, Department of Paediatric Surgery, Cocody Teaching Hospital, Abidjan, Ivory Coast
Email: akeyapilandry@yahoo.fr

Abstract

Background: The objective of this study was to describe the anatomo-clinical, therapeutic aspect of TT fractures and the results.
Methods: This retrospective study was conducted over a period of 5 years (01 January 2018 to 01 January 2023) in the Paediatric Surgery Department of the Teaching Hospital and at theInternational clinic. The epidemiological- clinical, anatomopathological, therapeutic, and the results variables of TT fractures were studied.
Results: Data from 10 cases were included in this study. All patients were male, and their mean age was 14.3 years [range: 11-15 years]. All fractures occurred during sports activities through indirect mechanisms. Fractures occurred on both the left and right sides of the knee. We have compiled fractures of type IB (2 cases), type IIB (03 cases), IIIA (01 case), type IIIB (03 cases), and type IV (01 case). All patients underwent open reduction and osteosynthesis. No complications occurred in any of the patients, and their long-term results were satisfactory.
Conclusion: TT fractures are uncommon in adolescents; however, they are common in sports that involve jumping. The indications for treatment are based on the modified Ogden classification. These fractures have a good prognosis, with full recovery of function and activity after consolidation.
Keywords: Fracture, Tibial tuberosity, Adolescence

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How to Cite this Article:  Ake YL, Ouattara SJJ, Bonny R, Kouassi-Dria AKS | Tibial Tuberosity Fractures in Adolescents: Anatomo – Clinical Assessment and Treatment Results | International Journal of Paediatric Orthopaedics | September-December 2024; 10(3): 12-18.

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Hip Displacement in Children with Cerebral Palsy- A Clinico- Radiological Evaluation

Volume 10 | Issue 3 | September-December 2024 | Page: 6-11 | Deepa Metgud, Shruti Desai, Shreya Bavi, Vinuta Deshpande, Naveenkumar Patil, Santosh Patil

DOI- https://doi.org/10.13107/ijpo.2024.v10.i03.200

Open Access License: CC BY-NC 4.0

Copyright Statement: Copyright © 2024; The Author(s).

Submitted: 20/11/2024; Reviewed: 26/11/2024; Accepted: 08/12/2024; Published: 10/12/2024

Authors: Deepa Metgud PhD, MPT [1], Shruti Desai MPT [1], Shreya Bavi MPT [1], Vinuta Deshpande MPT [1], Naveenkumar Patil MS Ortho [2], Santosh Patil MD Rad [3]

[1] Department of Paediatric Physiotherapy, KAHER Institute of Physiotherapy, Belagavi, Karnataka, India.
[2] Department of Orthopaedics, KAHER’S JGMM Medical College Gabbur, Kotagondhunshi, Hubbali, Karnataka, India.
[3] Department of Radiology, JN Medical College, Belagavi, Karnataka, India.

Address of Correspondence

Dr. Deepa Metgud
Department of Paediatric Physiotherapy, KAHER Institute of Physiotherapy, Belagavi, Karnataka, India.
Email Id: deepametgud@klekipt.edu.in

Abstract

Background: Children with cerebral palsy (CP) are at risk for hip subluxation due to the spasticity and contractures of the hip adductors, medial hamstrings, and hip flexors. Hip displacement is often asymptomatic in these children until the hip is particularly or fully dislocated resulting in pain, gait disturbances and impaired sitting balance. Hip surveillance is a process of actively monitoring a child for early identification of hip displacement. In India, the National Hip Surveillance Program was established to support surveillance in preventing dislocations and reducing the need for surgery. In light of this, the present study aims to determine the prevalence of hip displacement in children with cerebral palsy in Belagavi.
Method: This descriptive, cross-sectional observational study was conducted at a tertiary care hospital and inclusive education schools. Children aged 2–18 years with cerebral palsy, underwent clinical examinations followed by radiographic evaluation and the Migration Percentage (MP) was calculated to categorize hip displacement, the primary outcome measure of the study. Prevalence of subluxation and its association with gender, age, GMFCS E&R levels and CP subtypes were assessed.
Results: Out of 128 children with CP assessed, 104 had subluxation, with the majority (73.44%) showing bilateral involvement, while 7.81% had right-sided subluxation. The prevalence of subluxation varied by CP subtypes, with spastic type accounting for the higher prevalence. A statistically significant association between CP subtype and subluxation was found on the right side (p = 0.003).
Conclusion: The study identifies an 81.3% occurrence of hip subluxation in children with CP, with bilateral involvement being the most prevalent (73.44%). The likelihood of subluxation was notably impacted by CP subtype, especially in spastic CP. Timely detection through clinical assessment and radiographic monitoring is vital to prevent advancement to dislocation. Future investigations should prioritize extended follow-ups and therapeutic approaches to optimize outcomes.
Keywords: Hip subluxation, Cerebral palsy, Radiography, Migration percentage

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How to Cite this Article:  Metgud D, Desai S, Bavi S, Deshpande V, Patil N, Patil S | Hip Displacement in Children with Cerebral Palsy – A Clinico- Radiological Evaluation | International Journal of Paediatric Orthopaedics | September-December 2024; 10(3): 6-11.

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