Tag Archive for: Skeletal dysplasia

Combined Hemiepiphysiodesis Using Tension Band Plate and Osteotomy for Severe Coronal Plane Deformities Around Knee Joint in Children with Skeletal Dysplasia – An Innovative Technique

Volume 8 | Issue 2 | May-August 2022 | Page: 20-23 | Anil Agarwal, Ankit Jain, Ravi Jethwa, Jatin Raj Sareen

DOI- https://doi.org/10.13107/ijpo.2022.v08i02.139

Authors: Anil Agarwal MS Ortho [1], Ankit Jain D Ortho [1], Ravi Jethwa MS Ortho [1], Jatin Raj Sareen MS Ortho [1]

[1] Department of Paediatric Orthopaedics, Chacha Nehru Bal Chikitsalaya, Delhi, India.

Address of Correspondence

Dr. Anil Agarwal
Department of Paediatric Orthopaedics, Chacha Nehru Bal Chikitsalaya, Delhi, India.
E-mail: rachna_anila@yahoo.co.in

Abstract

Skeletal dysplasia in children is sometimes associated with severe coronal plane angulations around the knee. The associated ligament laxity adds to the complexity of surgical correction. Osteotomies require precise surgical planning and execution. Hemiepiphyseodesis is usually employed only in mild and moderate deformity. Distraction osteogenesis method is labour intensive, costly and requires a prolonged treatment course. We describe an innovative surgical technique which combines hemiepiphysiodesis using tension-band plates and a metaphyseal osteotomy. The technique utilises acute bony correction by osteotomy followed by residual correction, if any and soft tissue fine tuning through growth modulation. Growth modulation also addresses recurrence to some extent. The surgical technique is described along with illustrative case examples.
Keywords: Skeletal dysplasia, Osteotomy, Hemiepiphyseodesis

References

1. Bassett GS. Orthopaedic aspects of skeletal dysplasias. Instr Course Lect. 1990;39:381-387.
2. Rosskopf AB, Buck FM, Pfirrmann CW, Ramseier LE. Femoral and tibial torsion measurements in children and adolescents: comparison of MRI and 3D models based on low-dose biplanar radiographs. Skeletal Radiol. 2017;46:469-476.
3. Thacker MM, Davis ED, Ditro CP, Mackenzie W. Limb lengthening and deformity correction in patients with skeletal dysplasias. In: Sabharwal S (eds.). Pediatric Lower Limb Deformities. Springer, Cham; 2016. doi: 10.1007/978-3-319-17097-8_19
4. Bell DF, Boyer MI, Armstrong PF. The use of the Ilizarov technique in the correction of limb deformities associated with skeletal dysplasia. J Pediatr Orthop. 1992;12:283-290. doi: 10.1097/01241398-199205000-00003
5. Pinkowski JL, Weiner DS. Complications in proximal tibial osteotomies in children with presentation of technique. J Pediatr Orthop. 1995;15:307-312.
6. Yilmaz G, Oto M, Thabet AM, Rogers KJ, Anticevic D, Thacker MM, Mackenzie WG. Correction of lower extremity angular deformities in skeletal dysplasia with hemiepiphysiodesis: a preliminary report. J Pediatr Orthop. 2014;34:336-345. doi: 10.1097/BPO.0000000000000089
7. Cho TJ, Choi IH, Chung CY, Yoo WJ, Park MS, Lee DY. Hemiepiphyseal stapling for angular deformity correction around the knee joint in children with multiple epiphyseal dysplasia. J Pediatr Orthop. 2009;29:52-56.
8. Shabtai L, Herzenberg JE. Limits of growth modulation using tension band plates in the lower extremities. J Am Acad Orthop Surg. 2016;24):691-701. doi: 10.5435/JAAOS-D-14-00234
9. Masquijo JJ, Artigas C, de Pablos J. Growth modulation with tension-band plates for the correction of paediatric lower limb angular deformity: current concepts and indications for a rational use. EFORT Open Rev. 2021;6:658-668. doi: 10.1302/2058-5241.6.200098
10. Bell DF, Boyer MI, Armstrong PF. The use of the Ilizarov technique in the correction of limb deformities associated with skeletal dysplasia. J Pediatr Orthop. 1992;12:283-290.
11. Myers GJ, Bache CE, Bradish CF. Use of distraction osteogenesis techniques in skeletal dysplasias. J Pediatr Orthop. 2003;23:41-45.

How to Cite this Article: K Agarwal A, Jain A, Jethwa R, Sareen JR |  Combined Hemiepiphysiodesis Using Tension Band Plate and Osteotomy for Severe Coronal Plane Deformities Around Knee Joint in Children with Skeletal Dysplasia – An Innovative Technique | International Journal of Paediatric Orthopaedics | May- August 2022; 8(2): 20-23.
https://doi.org/10.13107/ijpo.2022.v08i02.139

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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.

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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