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Dysosteosclerosis – A Rare Sclerosing Bone Dysplasia

Volume 7 | Issue 3 | September-December 2021 | Page: 26-28 | Akanksha Parikh, Vikas Basa DOI-10.13107/ijpo.2021.v07i03.119 Authors: Akanksha Parikh MD DNB Paediatrics [1], Vikas Basa DNB Ortho. [2] [1] Department of Paediatrics, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India. [2] Department of Orthopaedics, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India. Unlock the Glamour of Gaming: Aviator Pin-Up […]

Outcomes of Closed Reduction Percutaneous Pinning in Paediatric Supracondylar Humerus Fractures Using “Arm Board” Technique “

Volume 7 | Issue 3 | September-December 2021 | Page: 02-07 | Sandeep Patwardhan, Bismaya Sahoo, Vivek Sodhai, Parag Sancheti, Ashok Shyam, Mrunal Kodape DOI-10.13107/ijpo.2021.v07i03.114 Authors: Sandeep Patwardhan MS Ortho. [1], Bismaya Sahoo MS Ortho. [1], Vivek Sodhai MS Ortho. [1], Parag Sancheti MS Ortho. [1], Ashok Shyam MS Ortho. [1, 2], Mrunal Kodape D. Ortho. [1] [1] Department of Orthopaedic, Sancheti […]

Dysosteosclerosis – A Rare Sclerosing Bone Dysplasia

Outcomes of Closed Reduction Percutaneous Pinning in Paediatric Supracondylar Humerus Fractures Using “Arm Board” Technique “

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Dysosteosclerosis – A Rare Sclerosing Bone Dysplasia

Volume 7 | Issue 3 | September-December 2021 | Page: 26-28 | Akanksha Parikh, Vikas Basa

DOI-10.13107/ijpo.2021.v07i03.119

Authors: Akanksha Parikh MD DNB Paediatrics [1], Vikas Basa DNB Ortho. [2]

[1] Department of Paediatrics, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India.
[2] Department of Orthopaedics, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India.

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Address of Correspondence
Dr Akanksha Parikh
Consultant Paediatric & Adolescent Endocrinology, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India.
E-mail: gandhi.akanksha@gmail.com

Abstract

Dysosteosclerosis (DOS) is a rare inherited sclerosing bone disorder caused by lack of osteoclast differentiation. A nine-month-old infant presented with a past history of pathological fracture, developmental delay and facial dysmorphisms. The sclerotic radiographic changes along with histologically observed increased bone deposition on clavicular bone biopsy led to the initial suspicion of osteopetrosis. However, a genetic analysis revealed a mutation in the SLC29A3 gene confirming the diagnosis of DOS. Due to the close clinical and radiological resemblance most infants with DOS are misdiagnosed as osteopetrosis, a related skeletal dysplasia. The presence of purplish skin rash, platyspondyly on radiographs and absence of bone marrow involvement differentiates DOS from the latter. Treatment is supportive and overall prognosis is poor with the eventual neurological deterioration and recurrent fractures.
Keywords: Skeletal dysplasia, Osteopetrosis, Platyspondyly, SLC29A3

References

1. Kobayashi K, Goto Y, Kise H, Kanai H, Kodera K, Nishimura G et al. A case report of dysosteosclerosis observed from the prenatal period. Clin Pediatr Endocrinol 2010;19:57-62.
2. Campeau PM, Lu JT, Sule G, Jiang MM, Bae Y, Madan S et al. Whole-exome sequencing identifies mutations in the nucleoside transporter gene SLC29A3 in dysosteosclerosis, a form of osteopetrosis. Hum Mol Genet 2012 Nov 15;2:4904-9.
3. Noavar S, Behroozi S, Tatarcheh T, Parvini F, Foroutan M, Fahimi H. A novel homozygous frame-shift mutation in the SLC29A3 gene: A new case report and review of literature. BMC Med Gen 2019;20:147.
4. Boudin E, Van Hul W. Sclerosing bone dysplasias. Best Pract Res Clin Endocrinol Metab 2018;32:707-23.
5. Howaldt A, Nampoothiri S, Quell LM, Ozden A, Fischer-Zirnsak B, Collet C et al. Sclerosing bone dysplasias with hallmarks of dysosteosclerosis in four patients carrying mutations in SLC29A3 and TCIRG1. Bone 2019;120:495-503.
6. Elcioglu NH, Vellodi A, Hall CM. Dysosteosclerosis: A report of three new cases and evolution of the radiological findings. J Med Genet 2002;39:603-7.

How to Cite this Article: Parikh A, Basa V| Dysosteosclerosis – A Rare Sclerosing Bone Dysplasia | International Journal of Paediatric Orthopaedics | September- December 2021; 7(3): 26-28.

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Outcomes of Closed Reduction Percutaneous Pinning in Paediatric Supracondylar Humerus Fractures Using “Arm Board” Technique “

Volume 7 | Issue 3 | September-December 2021 | Page: 02-07 | Sandeep Patwardhan, Bismaya Sahoo, Vivek Sodhai, Parag Sancheti, Ashok Shyam, Mrunal Kodape
DOI-10.13107/ijpo.2021.v07i03.114

Authors: Sandeep Patwardhan MS Ortho. [1], Bismaya Sahoo MS Ortho. [1], Vivek Sodhai MS Ortho. [1], Parag Sancheti MS Ortho. [1], Ashok Shyam MS Ortho. [1, 2], Mrunal Kodape D. Ortho. [1]

[1] Department of Orthopaedic, Sancheti Institute Of Orthopaedics and Rehabilitation, Pune, Maharashtra, India.
[2] Research department, Indian Orthopaedic Research Group, Thane [W], Maharashtra, India.

Address of Correspondence
Dr Bismaya Sahoo
Department of Orthopaedic, Sancheti Institute Of Orthopaedics and Rehabilitation, Pune, Maharashtra, India.
E-mail: bismay.ltmc.bs@gmail.com

Abstract

Introduction: Closed reduction percutaneous pinning (CRPP) is the standard treatment for displaced supracondylar fractures of humerus (SCH) in children. The purpose of our study is to analyze the functional outcomes of percutaneous Kirschner wire fixation of SCH fractures in paediatric population using a novel “arm board” technique.
Materials and Methods: A prospective study was carried out between October 2015 to October 2017 which included 71 patients of SCH fracture. Outcome evaluation was performed using Flynn’s score and Skagg’s grading. Age, gender, type of fracture, position of k-wires and time to surgery were also recorded. All patients were followed up for a minimum period of one year
Results: 90% were graded as excellent, 8.5% good, 1.5% fair according to Flynn’s score. By Skagg’s grading there was no loss of reduction in any patient. There were no significant differences in the outcomes based on age, gender, type of fracture, position of k-wires and time to surgery.
Conclusion: Good functional outcomes can be achieved with the “arm board” technique irrespective of age, gender, type of fracture, duration between injury and procedure if satisfactory reduction of the fracture can be achieved. Our technique uses a simple, locally fabricated radiolucent arm board that can be adapted to any standard operating table. It is a novel and innovative method that is safe, effective and can be easily replicated by orthopedic surgeons managing paediatric fractures.
Level of evidence: Therapeutic, Level III
Keywords: “arm board” technique, closed reduction percutaneous pinning, Paediatric; Supracondylar humerus fracture

References

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10. Younus S, Peracha A, Kamran S, et al (2018) Percutaneous Pinning for Displaced Supracondylar Humerus Fractures in Pediatric Age Group : Comparison between Lateral Pinning Versus Crossed Pinning Techniques. 1–4. https://doi.org/10.19080/OROAJ.2018.11.555817
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How to Cite this Article: Patwardhan S, Sahoo B, Sodhai V, Sancheti P, Shyam A, Kodape M | Outcomes of Closed Reduction Percutaneous Pinning in Paediatric Supracondylar Humerus Fractures using “Arm Board” Technique ” | International Journal of Paediatric Orthopaedics | September- December 2021; 7(3): 02-07.

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Dysosteosclerosis – A Rare Sclerosing Bone Dysplasia

Outcomes of Closed Reduction Percutaneous Pinning in Paediatric Supracondylar Humerus Fractures Using “Arm Board” Technique “

Dysosteosclerosis – A Rare Sclerosing Bone Dysplasia

Outcomes of Closed Reduction Percutaneous Pinning in Paediatric Supracondylar Humerus Fractures Using “Arm Board” Technique “

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Dysosteosclerosis – A Rare Sclerosing Bone Dysplasia

Authors: Akanksha Parikh MD DNB Paediatrics [1], Vikas Basa DNB Ortho. [2]

Abstract

(Article Text HTML) (Full Text PDF)

Outcomes of Closed Reduction Percutaneous Pinning in Paediatric Supracondylar Humerus Fractures Using “Arm Board” Technique “

Authors: Sandeep Patwardhan MS Ortho. [1], Bismaya Sahoo MS Ortho. [1], Vivek Sodhai MS Ortho. [1], Parag Sancheti MS Ortho. [1], Ashok Shyam MS Ortho. [1, 2], Mrunal Kodape D. Ortho. [1]

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