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.
Key words
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Article info
Publication history
Published online: April 25, 2019
Accepted:
February 26,
2019
Received:
July 31,
2018
Footnotes
No benefits in any form have been received or will be received related directly or indirectly to the subject of this article.
Identification
Copyright
© 2019 by the American Society for Surgery of the Hand. All rights reserved.
