TY - JOUR
T1 - Characterization of Hand Anomalies Associated With Möbius Syndrome
AU - Koehler, Daniel M.
AU - Goldfarb, Charles A.
AU - Snyder-Warwick, Alison
AU - Roberts, Summer
AU - Wall, Lindley B.
N1 - Publisher Copyright:
© 2019 American Society for Surgery of the Hand
PY - 2019/7
Y1 - 2019/7
N2 - Purpose: To investigate the distinguishing morphological characteristics of the upper extremities in children with Möbius syndrome. Methods: Twenty-seven involved extremities in 14 patients with a diagnosis of Möbius syndrome were identified at 2 institutions. Medical records, radiographs, and clinical photographs were evaluated. Congenital hand differences were classified according to the Oberg, Manske, and Tonkin classification, and hands with symbrachydactyly were classified by the Blauth and Gekeler classification. The presence of other congenital anomalies was catalogued. Results: There was bilateral involvement in 93% of patients with congenital hand anomalies. Twelve patients demonstrated congenital hand anomalies and 2 patients had been diagnosed with arthrogryposis. Among the 12 patients with congenital hand anomalies, 21 hands were classifiable as symbrachydactyly by the Oberg, Manske, and Tonkin classification and could be categorized by the Blauth and Gekeler classification. Short finger type was the most common subtype of symbrachydactyly, present in 13 hands. Eleven of these 13 patients (85%) were primarily affected on the radial side of the hand. Proximal arm involvement was identified in 2 patients with symbrachydactyly, both of whom had Poland syndrome and an absent pectoralis major. Conclusions: Symbrachydactyly in Möbius syndrome differs from the typical presentation of symbrachydactyly. Characteristically, there is a bilateral presentation with a strong predilection for radially based brachydactyly. These described characteristics may help the hand surgeon appropriately assess patients, especially those with radial-sided symbrachydactyly. Type of study/level of evidence: Diagnostic III.
AB - Purpose: To investigate the distinguishing morphological characteristics of the upper extremities in children with Möbius syndrome. Methods: Twenty-seven involved extremities in 14 patients with a diagnosis of Möbius syndrome were identified at 2 institutions. Medical records, radiographs, and clinical photographs were evaluated. Congenital hand differences were classified according to the Oberg, Manske, and Tonkin classification, and hands with symbrachydactyly were classified by the Blauth and Gekeler classification. The presence of other congenital anomalies was catalogued. Results: There was bilateral involvement in 93% of patients with congenital hand anomalies. Twelve patients demonstrated congenital hand anomalies and 2 patients had been diagnosed with arthrogryposis. Among the 12 patients with congenital hand anomalies, 21 hands were classifiable as symbrachydactyly by the Oberg, Manske, and Tonkin classification and could be categorized by the Blauth and Gekeler classification. Short finger type was the most common subtype of symbrachydactyly, present in 13 hands. Eleven of these 13 patients (85%) were primarily affected on the radial side of the hand. Proximal arm involvement was identified in 2 patients with symbrachydactyly, both of whom had Poland syndrome and an absent pectoralis major. Conclusions: Symbrachydactyly in Möbius syndrome differs from the typical presentation of symbrachydactyly. Characteristically, there is a bilateral presentation with a strong predilection for radially based brachydactyly. These described characteristics may help the hand surgeon appropriately assess patients, especially those with radial-sided symbrachydactyly. Type of study/level of evidence: Diagnostic III.
KW - Congenital hand differences
KW - Möbius syndrome
KW - facial paralysis
KW - symbrachydactyly
UR - http://www.scopus.com/inward/record.url?scp=85064590277&partnerID=8YFLogxK
U2 - 10.1016/j.jhsa.2019.02.020
DO - 10.1016/j.jhsa.2019.02.020
M3 - Article
C2 - 31031024
AN - SCOPUS:85064590277
SN - 0363-5023
VL - 44
SP - 548
EP - 555
JO - Journal of Hand Surgery
JF - Journal of Hand Surgery
IS - 7
ER -