Skip to main content
Top
Published in: Journal of Orthopaedic Surgery and Research 1/2024

Open Access 01-12-2024 | Trisomy 21 | Research article

Outcomes of surgical treatment of patellar instability in children with Down syndrome

Authors: Assem Zein, Alaa Zenhom Mahmoud Hassan, Amr Mohamed Soliman, Mohamed Mohamed Azmy Mohamed

Published in: Journal of Orthopaedic Surgery and Research | Issue 1/2024

Login to get access

Abstract

Background

patellar instability is a relatively frequent musculoskeletal disorder in children with Down syndrome (DS). However, such a condition has seldom been studied in the literature, even less its surgical treatment. Different techniques have been offered for this condition; the evidence for surgical options is scarce and primarily based on case reports or case series with few patients and heterogeneous techniques. Given this background, we aimed to evaluate the outcomes of a uniform kind of surgical procedure for such a condition that combined lateral soft tissue release, medial patellofemoral ligament (MPFL) reconstruction (using a partial-thickness quadriceps tendon autograft), the Roux-Goldthwait procedure, and V-Y quadricepsplasty (if needed).

Materials and methods

This retrospective study involved 11 skeletally immature patients (12 knees; 9 males and 2 females), 5.5 to 14.1 years of age, with DS who had patellofemoral instability (PFI) and were managed by this technique between October 2018 and March 2020. Preoperative radiography, CT scan, and MRI were performed to evaluate the physis status, lower limb alignment, patellar height, trochlear morphology, and any associated knee pathology. A functional knee assessment was done by using the Kujala score and the modified Lysholm score.

Results

The mean time of follow-up (± SD) was 47.7 ± 5.8 months (range: 39–56). Pre-operatively, the Kujala score (± SD) was 52.6 ± 14.3 (range: (31–74), and at final follow-up, it was 92.2 ± 4.4 (range: (88–98), showing a significant improvement (P < 0.001). The preoperative modified Lysholm score (± SD) was 54.3 ± 8.1 (range: 39–62), and at final follow-up it was 92.4 ± 5.3 (range: 82–96), showing a significant improvement (P < 0.001). All patients had a stable patella without a recurrence of instability and regained full ROM. There was no incidence of a patellar fracture or femoral physis injury.

Conclusions

Our proposed technique of combined soft tissue procedures, including lateral soft tissue release, MPFL reconstruction (using a partial-thickness quadriceps tendon autograft), the Roux-Goldthwait procedure, and V-Y quadricepsplasty, was an effective method for treating patellar instability in children with DS while avoiding physeal injury and patellar fracture. Functional scores and radiological outcomes were improved.

Level of evidence

IV; retrospective case series.
Literature
1.
go back to reference -Kumar V, Abbas AK, Jon C, Aster. (2015) Genetic Disorders. Robbins and Cotran Pathologic Basis of Disease. Ninth edition. Philadelphia, PA: Elsevier/Saunders. -Kumar V, Abbas AK, Jon C, Aster. (2015) Genetic Disorders. Robbins and Cotran Pathologic Basis of Disease. Ninth edition. Philadelphia, PA: Elsevier/Saunders.
3.
go back to reference -Karousou E, Stachtea X, Moretto P, Viola M, Vigetti D, D’Angelo ML, Raio L, Ghezzi F, Pallotti F, De Luca G, Karamanos NK, Passi A. New insights into the pathobiology of Down syndrome–hyaluronan synthase-2 overexpression is regulated by collagen VI α2 chain. FEBS J. 2013;280(10):2418–30. https://doi.org/10.1111/febs.12220. Epub 2013 Mar 28. PMID: 23452080.CrossRefPubMed -Karousou E, Stachtea X, Moretto P, Viola M, Vigetti D, D’Angelo ML, Raio L, Ghezzi F, Pallotti F, De Luca G, Karamanos NK, Passi A. New insights into the pathobiology of Down syndrome–hyaluronan synthase-2 overexpression is regulated by collagen VI α2 chain. FEBS J. 2013;280(10):2418–30. https://​doi.​org/​10.​1111/​febs.​12220. Epub 2013 Mar 28. PMID: 23452080.CrossRefPubMed
4.
go back to reference -Foley C, Killeen OG. Musculoskeletal anomalies in children with Down syndrome: an observational study. Arch Dis Child. 2019;104(5):482–7. 10.1136. Epub 2018 Nov 24. PMID: 30472668; PMCID: PMC6557226. /archdischild-2018-315751.CrossRefPubMed -Foley C, Killeen OG. Musculoskeletal anomalies in children with Down syndrome: an observational study. Arch Dis Child. 2019;104(5):482–7. 10.1136. Epub 2018 Nov 24. PMID: 30472668; PMCID: PMC6557226. /archdischild-2018-315751.CrossRefPubMed
5.
go back to reference -Tsou AY, Bulova P, Capone G, Chicoine B, Gelaro B, Harville TO, Martin BA, McGuire DE, McKelvey KD, Peterson M, Tyler C, Wells M, Whitten MS. Global Down Syndrome Foundation Medical Care Guidelines for Adults with Down Syndrome Workgroup. Medical Care of Adults With Down Syndrome: A Clinical Guideline. JAMA. 2020;324(15):1543–1556. https://doi.org/10.1001/jama.2020.17024. PMID: 33079159. -Tsou AY, Bulova P, Capone G, Chicoine B, Gelaro B, Harville TO, Martin BA, McGuire DE, McKelvey KD, Peterson M, Tyler C, Wells M, Whitten MS. Global Down Syndrome Foundation Medical Care Guidelines for Adults with Down Syndrome Workgroup. Medical Care of Adults With Down Syndrome: A Clinical Guideline. JAMA. 2020;324(15):1543–1556. https://​doi.​org/​10.​1001/​jama.​2020.​17024. PMID: 33079159.
6.
go back to reference -Dugdale TW, Renshaw TS. Instability of the patellofemoral joint in Down syndrome. J Bone Joint Surg Am. 1986;68(3):405–13. PMID: 2936745.CrossRefPubMed -Dugdale TW, Renshaw TS. Instability of the patellofemoral joint in Down syndrome. J Bone Joint Surg Am. 1986;68(3):405–13. PMID: 2936745.CrossRefPubMed
7.
go back to reference -Mendez AA, Keret D, MacEwen GD. Treatment of patellofemoral instability in Down’s syndrome. Clin Orthop Relat Res. 1988;(234):148–58. PMID: 2970357. -Mendez AA, Keret D, MacEwen GD. Treatment of patellofemoral instability in Down’s syndrome. Clin Orthop Relat Res. 1988;(234):148–58. PMID: 2970357.
8.
go back to reference -Bettuzzi C, Lampasi M, Magnani M, Donzelli O. Surgical treatment of patellar dislocation in children with Down syndrome: a 3- to 11-year follow-up study. Knee Surg Sports Traumatol Arthrosc. 2009;17(4):334–40. Epub 2008 Oct 31. PMID: 18974972.CrossRefPubMed -Bettuzzi C, Lampasi M, Magnani M, Donzelli O. Surgical treatment of patellar dislocation in children with Down syndrome: a 3- to 11-year follow-up study. Knee Surg Sports Traumatol Arthrosc. 2009;17(4):334–40. Epub 2008 Oct 31. PMID: 18974972.CrossRefPubMed
9.
go back to reference -Camanho GL, Bitar AC, Hernandez AJ, Olivi R. Medial patellofemoral ligament reconstruction: a novel technique using the patellar ligament. Arthroscopy. 2007;23(1):108e. 1-4. Epub 2006 Oct 16. PMID: 17210439.CrossRef -Camanho GL, Bitar AC, Hernandez AJ, Olivi R. Medial patellofemoral ligament reconstruction: a novel technique using the patellar ligament. Arthroscopy. 2007;23(1):108e. 1-4. Epub 2006 Oct 16. PMID: 17210439.CrossRef
10.
go back to reference -Amis AA, Firer P, Mountney J, Senavongse W, Thomas NP. Anatomy and biomechanics of the medial patellofemoral ligament. Knee. 2003;10(3):215 – 20. doi: 10.1016/s0968-0160(03)00006-1. Erratum in: Knee. 2004;11(1):73. PMID: 12893142. -Amis AA, Firer P, Mountney J, Senavongse W, Thomas NP. Anatomy and biomechanics of the medial patellofemoral ligament. Knee. 2003;10(3):215 – 20. doi: 10.1016/s0968-0160(03)00006-1. Erratum in: Knee. 2004;11(1):73. PMID: 12893142.
11.
go back to reference -Vetrano M, Oliva F, Bisicchia S, Bossa M, De Carli A, Di Lorenzo L, Erroi D, Forte A, Foti C, Frizziero A, Gasparre G, Via AG, Innocenti B, Longo UG, Mahmoud A, Masiero S, Mazza D, Natali S, Notarangelo C, Osti L, Padulo J, Pellicciari L, Perroni F, Piccirilli E, Ramponi C, Salvatore G, Panni AS, Suarez T, Tarantino U, Vittadini F, Vulpiani MC, Ferretti A, Maffulli N. I.S.Mu.L.T. First-time patellar dislocation guidelines. Musc)les Ligaments Tendons J. 2017;7(1):1–10. https://doi.org/10.11138/mltj/2017.7.1.001. PMID: 28717605; PMCID: PMC5505576.CrossRef -Vetrano M, Oliva F, Bisicchia S, Bossa M, De Carli A, Di Lorenzo L, Erroi D, Forte A, Foti C, Frizziero A, Gasparre G, Via AG, Innocenti B, Longo UG, Mahmoud A, Masiero S, Mazza D, Natali S, Notarangelo C, Osti L, Padulo J, Pellicciari L, Perroni F, Piccirilli E, Ramponi C, Salvatore G, Panni AS, Suarez T, Tarantino U, Vittadini F, Vulpiani MC, Ferretti A, Maffulli N. I.S.Mu.L.T. First-time patellar dislocation guidelines. Musc)les Ligaments Tendons J. 2017;7(1):1–10. https://​doi.​org/​10.​11138/​mltj/​2017.​7.​1.​001. PMID: 28717605; PMCID: PMC5505576.CrossRef
12.
go back to reference -Panni AS, Cerciello S, Maffulli N, Di Cesare M, Servien E, Neyret P. Patellar shape can be a predisposing factor in patellar instability. Knee Surg Sports Traumatol Arthrosc. 2011;19(4):663 – 70. https://doi.org/10.1007/s00167-010-1329-4. Epub 2010 Dec 11. PMID: 21153544. -Panni AS, Cerciello S, Maffulli N, Di Cesare M, Servien E, Neyret P. Patellar shape can be a predisposing factor in patellar instability. Knee Surg Sports Traumatol Arthrosc. 2011;19(4):663 – 70. https://​doi.​org/​10.​1007/​s00167-010-1329-4. Epub 2010 Dec 11. PMID: 21153544.
13.
go back to reference -Migliorini F, Pilone M, Eschweiler J, Marsilio E, Hildebrand F, Maffulli N. High rates of damage to the Medial Patellofemoral ligament, lateral trochlea, and Patellar Crest after Acute Patellar dislocation: magnetic resonance imaging analysis. Arthroscopy. 2022;38(8):2472–9. Epub 2022 Feb 12. PMID: 35157964.CrossRefPubMed -Migliorini F, Pilone M, Eschweiler J, Marsilio E, Hildebrand F, Maffulli N. High rates of damage to the Medial Patellofemoral ligament, lateral trochlea, and Patellar Crest after Acute Patellar dislocation: magnetic resonance imaging analysis. Arthroscopy. 2022;38(8):2472–9. Epub 2022 Feb 12. PMID: 35157964.CrossRefPubMed
14.
go back to reference -Abouelsoud MM, Abdelhady A, Elshazly O. Anatomic physeal-sparing technique for medial patellofemoral ligament reconstruction in skeletally immature patients with ligamentous laxity. Eur J Orthop Surg Traumatol. 2015;25(5):921-6. https://doi.org/10.1007/s00590-015-1618-1. Epub 2015 Mar 11. PMID: 25757696. -Abouelsoud MM, Abdelhady A, Elshazly O. Anatomic physeal-sparing technique for medial patellofemoral ligament reconstruction in skeletally immature patients with ligamentous laxity. Eur J Orthop Surg Traumatol. 2015;25(5):921-6. https://​doi.​org/​10.​1007/​s00590-015-1618-1. Epub 2015 Mar 11. PMID: 25757696.
16.
go back to reference -Nelitz M, Dreyhaupt J, Williams SRM. Anatomic reconstruction of the medial patellofemoral ligament in children and adolescents using a pedicled quadriceps tendon graft shows favourable results at a minimum of 2-year follow-up. Knee Surg Sports Traumatol Arthrosc. 2018;26(4):1210–5. https://doi.org/10.1007/s00167-017-4597-4. Epub 2017 Jun 10. PMID: 28601942.CrossRefPubMed -Nelitz M, Dreyhaupt J, Williams SRM. Anatomic reconstruction of the medial patellofemoral ligament in children and adolescents using a pedicled quadriceps tendon graft shows favourable results at a minimum of 2-year follow-up. Knee Surg Sports Traumatol Arthrosc. 2018;26(4):1210–5. https://​doi.​org/​10.​1007/​s00167-017-4597-4. Epub 2017 Jun 10. PMID: 28601942.CrossRefPubMed
25.
go back to reference -Rebouças Moreira TA, Demange MK, Gobbi RG, Mustacchi Z, Pécora JR, Passarelli Tírico LE, Camanho GL. Trochlear dysplasia and patellar instability in patients with Down syndrome. Rev Bras Ortop. 2015;50(2):159–63. PMID: 26229910; PMCID: PMC4519639.PubMedPubMedCentral -Rebouças Moreira TA, Demange MK, Gobbi RG, Mustacchi Z, Pécora JR, Passarelli Tírico LE, Camanho GL. Trochlear dysplasia and patellar instability in patients with Down syndrome. Rev Bras Ortop. 2015;50(2):159–63. PMID: 26229910; PMCID: PMC4519639.PubMedPubMedCentral
28.
go back to reference -Joo SY, Park KB, Kim BR, Park HW, Kim HW. The ‘four-in-one’ procedure for habitual dislocation of the patella in children: early results in patients with severe generalised ligamentous laxity and aplasis of the trochlear groove. J Bone Joint Surg Br. 2007;89(12):1645-9. https://doi.org/10.1302/0301-620X.89B12.19398. PMID: 18057367. -Joo SY, Park KB, Kim BR, Park HW, Kim HW. The ‘four-in-one’ procedure for habitual dislocation of the patella in children: early results in patients with severe generalised ligamentous laxity and aplasis of the trochlear groove. J Bone Joint Surg Br. 2007;89(12):1645-9. https://​doi.​org/​10.​1302/​0301-620X.​89B12.​19398. PMID: 18057367.
31.
go back to reference -Bitar AC, D’Elia CO, Schor B, Bertoni J, Mustacchi Z, Grangeiro PM. Clinical and radiologic outcomes of the Surgical treatment of Patellofemoral instability in children with Down Syndrome and Dislocated Patella: Case Series. J Orthop Case Rep. 2018 Jul-Aug;8(4):61–5. https://doi.org/10.13107/jocr.2250-0685.1162. PMID: 30687666; PMCID: PMC6343554. -Bitar AC, D’Elia CO, Schor B, Bertoni J, Mustacchi Z, Grangeiro PM. Clinical and radiologic outcomes of the Surgical treatment of Patellofemoral instability in children with Down Syndrome and Dislocated Patella: Case Series. J Orthop Case Rep. 2018 Jul-Aug;8(4):61–5. https://​doi.​org/​10.​13107/​jocr.​2250-0685.​1162. PMID: 30687666; PMCID: PMC6343554.
32.
go back to reference -Herbort M, Hoser C, Domnick C, Raschke MJ, Lenschow S, Weimann A, Kösters C, Fink C. MPFL reconstruction using a quadriceps tendon graft: part 1: biomechanical properties of quadriceps tendon MPFL reconstruction in comparison to the Intact MPFL. A human cadaveric study. Knee. 2014;21(6):1169–74. https://doi.org/10.1016/j.knee.2014.07.026. Epub 2014 Aug 7. PMID: 25178693.CrossRefPubMed -Herbort M, Hoser C, Domnick C, Raschke MJ, Lenschow S, Weimann A, Kösters C, Fink C. MPFL reconstruction using a quadriceps tendon graft: part 1: biomechanical properties of quadriceps tendon MPFL reconstruction in comparison to the Intact MPFL. A human cadaveric study. Knee. 2014;21(6):1169–74. https://​doi.​org/​10.​1016/​j.​knee.​2014.​07.​026. Epub 2014 Aug 7. PMID: 25178693.CrossRefPubMed
34.
go back to reference -Shea KG, Styhl AC, Jacobs JC Jr, Ganley TJ, Milewski MD, Cannamela PC, Anderson AF, Polousky JD. The relationship of the femoral physis and the Medial Patellofemoral ligament in children: a cadaveric study. Am J Sports Med. 2016;44(11):2833–7. Epub 2016 Jul 29. PMID: 27474384.CrossRefPubMed -Shea KG, Styhl AC, Jacobs JC Jr, Ganley TJ, Milewski MD, Cannamela PC, Anderson AF, Polousky JD. The relationship of the femoral physis and the Medial Patellofemoral ligament in children: a cadaveric study. Am J Sports Med. 2016;44(11):2833–7. Epub 2016 Jul 29. PMID: 27474384.CrossRefPubMed
36.
go back to reference -Ziegler CG, Fulkerson JP, Edgar C. Radiographic Reference Points Are Inaccurate With and Without a True Lateral Radiograph: The Importance of Anatomy in Medial Patellofemoral Ligament Reconstruction. Am J Sports Med. 2016;44(1):133 – 42. doi: 10.1177/0363546515611652. Epub 2015 Nov 11. PMID: 26561652. -Ziegler CG, Fulkerson JP, Edgar C. Radiographic Reference Points Are Inaccurate With and Without a True Lateral Radiograph: The Importance of Anatomy in Medial Patellofemoral Ligament Reconstruction. Am J Sports Med. 2016;44(1):133 – 42. doi: 10.1177/0363546515611652. Epub 2015 Nov 11. PMID: 26561652.
39.
go back to reference -Lind M, Nielsen T, Miller L, Sørensen OG, Mygind-Klavsen B, Faunø P. No Difference in Outcome Between Femoral Soft-Tissue and Screw Graft Fixation for Reconstruction of the Medial Patellofemoral Ligament: A Randomized Controlled Trial. Arthroscopy. 2019;35(4):1130–1137. doi: 10.1016/j.arthro.2018.11.051. Epub 2019 Mar 11. PMID: 30871907. -Lind M, Nielsen T, Miller L, Sørensen OG, Mygind-Klavsen B, Faunø P. No Difference in Outcome Between Femoral Soft-Tissue and Screw Graft Fixation for Reconstruction of the Medial Patellofemoral Ligament: A Randomized Controlled Trial. Arthroscopy. 2019;35(4):1130–1137. doi: 10.1016/j.arthro.2018.11.051. Epub 2019 Mar 11. PMID: 30871907.
Metadata
Title
Outcomes of surgical treatment of patellar instability in children with Down syndrome
Authors
Assem Zein
Alaa Zenhom Mahmoud Hassan
Amr Mohamed Soliman
Mohamed Mohamed Azmy Mohamed
Publication date
01-12-2024
Publisher
BioMed Central
Keyword
Trisomy 21
Published in
Journal of Orthopaedic Surgery and Research / Issue 1/2024
Electronic ISSN: 1749-799X
DOI
https://doi.org/10.1186/s13018-024-04730-y

Other articles of this Issue 1/2024

Journal of Orthopaedic Surgery and Research 1/2024 Go to the issue