Revisiting Radiographic Characteristic of the Hands in Apert Syndrome: A Literature Review with Cases
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Abstract
Introduction: Syndactyly of the hand in Apert syndrome involves all components of the hands. In 1991, Joseph Upton introduced a comprehensive classification of the Apert hands based on its severity. Plain x-ray is the most commonly used to evaluate bony abnormality.
Case Presentation: We present two cases with classic characteristics of Apert syndrome with demonstrated different types of Apert hands; type II and type III. Radiographs exhibited bony deformities with typical misalignment and multiple osseous formations in several parts of phalanges.
Discussion: Apert hands present complex syndactylies involving the index, middle, and ring fingers in different stages regarding Upton’s classification system. Almost all Apert hands also lack the active motion of the interphalangeal joints. The ossification allowed the synostosis and symphalangism to become evident on radiographs. The abnormality of the epiphysial growth causes short and deviated fingers.
Conclusion: Apert hands demonstrate complex deformity involving bony structures that developed prenatally and postnatally. Typical deformities including complex syndactyly, clinodactyly, and symphalangism.
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References
I. Upton J. Apert syndrome. Classification and pathologic anatomy of limb anomalies. Clin Plast Surg. 1991;18(2):321-355.
II. Pettitt DA, Arshad Z, Mishra A, McArthur P. Apert syndrome: A consensus on the management of Apert hands. Journal of Cranio-Maxillofacial Surgery. 2017;45(2):223-231. doi:10.1016/j.jcms.2016.11.018
III. Cohen MM, Kreiborg S. Hands and feet in the Apert syndrome. Am J Med Genet. 1995;57(1):82-96. doi:10.1002/ajmg.1320570119
IV. Green SM. Pathological anatomy of the hands in Apert’s syndrome. The Journal of Hand Surgery. 1982;7(5):450-453.
doi:10.1016/S0363-5023(82)80038-5
V. Hoover GH, Flatt AE, Weiss MW. The hand and Apert’s syndrome. J Bone Joint Surg Am. 1970;52(5):878-895.
VI. Kim JH, Rhee SH, Gong HS, Lee HJ, Kwon ST, Baek GH. Characteristic radiographic features of the central ray in Apert syndrome. J Hand Surg Eur Vol. 2013;38(3):257-264. doi:10.1177/1753193412462895
VII. Caouette-Laberge L, Laberge C, Egerszegi EP, Stanciu C. Physiolysis for correction of clinodactyly in children. The Journal of Hand Surgery. 2002;27(4):659-665. doi:10.1053/jhsu.2002.33702
VIII. Arkin AM, Katz JF. The Effects of Pressure on Epiphyseal Growth: The Mechanism of Plasticity of Growing Bone. The Journal of Bone & Joint Surgery. 1956;38(5):1056-1076.
doi:10.2106/00004623-195638050-00009
IX. Guero SJ. Algorithm for Treatment of Apert Hand: Techniques in Hand and Upper Extremity Surgery. 2005;9(3):126-133. doi:10.1097/01.bth.0000185378.87246.61
X. Theman TA, Upton J, Taghinia AH, Firriolo JM, Nuzzi LC, Labow BI. Central Coalition Osteotomy of Phalangeal Synostoses in the Management of the Type III Apert Hand. The Journal of Hand Surgery. 2018;43(11):1042.e1-1042.e8. doi:10.1016/j.jhsa.2018.03.050