Congenital dislocation of the knee in combination with Meyer-Gorlin syndrome: A case report
- Authors: Kruglov I.Y.1,2, Rumyantsev N.Y.1, Omarov G.G.3,4, Rumyantseva N.N.1, Kagantsov I.M.5,4
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Affiliations:
- Almazov National Medical Research Centre
- H. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery
- H. Turner National Medical Research Center for Children’s Orthopedics and Trauma Surgery
- North-Western State Medical University named after I.I. Mechnikov
- Institute of Perinatology and Pediatrics, Almazov National Medical Research Centre
- Issue: Vol 9, No 4 (2021)
- Pages: 447-454
- Section: Clinical cases
- URL: https://bakhtiniada.ru/turner/article/view/62569
- DOI: https://doi.org/10.17816/PTORS62569
- ID: 62569
Cite item
Abstract
BACKGROUND: Meyer-Gorlin syndrome is a rare genetic and autosomal recessive disease that is characterized by the classical triad, including, microtia, very small size or complete patellar absence, and nanism.
CLINICAL CASE: Herein, presented the first clinical case description of a Russian patient with Meyer-Gorlin syndrome in combination with congenital anterior shin dislocation. The main clinical disease manifestations are characterized by a combination of microtia, patellar pathology, and dwarfism.
DISCUSSION: In the practice of pediatric orthopedic surgeons, cases of congenital knee dislocations are extremely rare, especially as part of any syndromes, which cause interest in the presented clinical case publication. Our patient analysis, as well as the patients described in the literature, showed the presence of typical clinical manifestations, which allowed us to suspect the presence of Meyer-Gorlin syndrome during a clinical examination.
CONCLUSIONS: This report is the first case of combined congenital knee dislocation and Meyer-Gorlin syndrome with a diagnostic triad (short stature, microtia, and patellar aplasia) in the Russian Federation. Conservative treatment with constant traction and flexion must be carefully performed to avoid complications. Without the effect of conservative therapy, surgical treatment is indicated.
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##article.viewOnOriginalSite##About the authors
Igor Y. Kruglov
Almazov National Medical Research Centre; H. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery
Email: dr.gkruglov@gmail.com
ORCID iD: 0000-0003-1234-1390
SPIN-code: 7777-1047
paediatric orthopaedic surgeon, junior researcher of Research Laboratory of congenital and hereditary pathology surgery
Russian Federation, 2 Akkuratova str., Saint Petersburg, 197341; Saint PetersburgNicolai Y. Rumyantsev
Almazov National Medical Research Centre
Email: dr.rumyantsev@gmail.com
ORCID iD: 0000-0002-4956-6211
MD, paediatric orthopaedic surgeon
Russian Federation, 2 Akkuratova str., Saint Petersburg, 197341Gamzat G. Omarov
H. Turner National Medical Research Center for Children’s Orthopedics and Trauma Surgery; North-Western State Medical University named after I.I. Mechnikov
Email: ortobaby@yandex.ru
ORCID iD: 0000-0002-9252-8130
MD, PhD, research associate
Russian Federation, 196603, St. Petersburg, Pushkin, Parkovaya str., 64-68; Saint PetersburgNatalia N. Rumyantseva
Almazov National Medical Research Centre
Email: natachazlaya@mail.ru
ORCID iD: 0000-0002-2052-451X
MD, paediatric orthopaedic surgeon, junior researcher of Research Laboratory of congenital and hereditary pathology surgery
Russian Federation, 2 Akkuratova str., Saint Petersburg, 197341Ilya M. Kagantsov
Institute of Perinatology and Pediatrics, Almazov National Medical Research Centre; North-Western State Medical University named after I.I. Mechnikov
Author for correspondence.
Email: ilkagan@rambler.ru
ORCID iD: 0000-0002-3957-1615
SPIN-code: 7936-8722
MD, PhD, D.Sc., Chief Researcher, Professor of the Chair
Russian Federation, 2 Akkuratova str., Saint Petersburg, 197341; Saint PetersburgReferences
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