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Sprengel deformity
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Sprengel deformity

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Contributors: David Sullo MD
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Synopsis

A rare condition where one or both shoulder blades are underdeveloped and abnormally high, leading to decreased range of motion in the neck and shoulders. Also called congenital high scapula, or congenital elevation of scapula, it has been reported as an autosomal-dominant inherited condition, but most often occurs sporadically due to a disruption in normal fetal skeletal development. More often seen in girls than boys, common signs and symptoms include nuchal rigidity, neck and shoulder muscle hypoplasia, muscle atrophy, and limited range of motion.

Management may include physical therapy to develop and maintain a greater range of motion and functionality. Surgery, when chosen, is recommended in early childhood (under 8 years old). Multiple surgical procedures include Green's procedure, scapula osteotomy, and the Woodward procedure.

For more information, see OMIM.

Codes

ICD10CM:
Q74.0 – Other congenital malformations of upper limb(s), including shoulder girdle

SNOMEDCT:
79120002 – Congenital elevation of scapula

Differential Diagnosis & Pitfalls

Poland anomaly

Best Tests

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References

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Last Updated: 03/29/2017
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Sprengel deformity
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Sprengel deformity : Muscle atrophy, Nuchal rigidity, Limited range of motion
Copyright © 2019 VisualDx®. All rights reserved.