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DOI: 10.4103/wjnm.WJNM_1_18
Sprengel's deformity as congenital scapular asymmetry on bone scintigraphy
Abstract
Bone scintigraphy is a valuable imaging modality for a diverse range of skeletal abnormalities. Incidental findings and congenital skeletal anomalies are not uncommonly encountered. Therefore, having a substantial knowledge of these findings, even rare ones, is essential for interpreting physicians. Here, we present a case of previously diagnosed malignancy referred for a bone scintigraphy for skeletal metastatic survey, in which Sprengel's deformity as congenital scapular asymmetry and cervicothoracic scoliosis was noted.
Introduction
Bone scintigraphy is a valuable and attractive imaging modality for a wide range of skeletal abnormalities. Incidental findings and congenital skeletal anomalies are not uncommonly encountered. Here, we present a case of previously diagnosed malignancy referred for a bone scintigraphy for skeletal metastatic survey, in which Sprengel's deformity (SD) as congenital scapular asymmetry and cervicothoracic scoliosis was noted.
Case Report
A 59-year-old male with a history of concomitant laryngeal squamous cell carcinoma and lung adenocarcinoma referred to our laboratory for a whole-body bone scintigraphy for the evaluation of bone metastasis. During history taking and physical examination, we realized that shoulders were asymmetric in size, left side seemed to be smaller than opposite side. The left upper limb was weak and to some extent abnormally positioned. The patient stated that these skeletal abnormalities were congenital and did not use much his left upper limb. The patient has also been limping since his previous motor accident. A planar whole-body bone scintigraphy and spot view [Figure 1] and [Figure 2] were performed at 2 h after intravenous injection of 740 MBq of 99mTc-methylene diphosphonate. As shown in [Figure 1], significant asymmetric size of scapulae but the normal-appearing shape and slightly higher position of the left scapula are detected. Other findings were mild cervicothoracic scoliosis as well as slightly asymmetric upper limbs. Focal uptake at midshaft of the right tibia was compatible with a recent trauma.




Discussion
There are a number of congenital or developmental musculoskeletal anomalies, among which some are related to scapula and shoulder.[1],[2],[3],[4],[5] Although the bone scintigraphy is not the primary modality for evaluating congenital skeletal abnormalities, these findings are occasionally present or incidentally discovered.
Although congenital anomalies of the scapula are rare, the most common of which is which can be associated with other skeletal deformities (e.g., vertebral and rib deformities, spinal scoliosis, and spina bifida).[2],[6],[7] To date, the embryogenic etiology of SD has not been convincingly elucidated.[2],[8] SD has been classified as low- to high-grade according to the severity of deformity.[2],[3],[8] Scapula is usually hypoplastic or dysplastic with abnormally elevated position.[2],[3],[6],[8] The scapula on opposite side is usually normal, therefore shoulders appear asymmetric.[2]
Since there is an increasing role for bone scintigraphy in daily practice for assessment of wide range of skeletal disorders, physicians should be familiar with many incidental and rare findings, including skeletal anomalies, on bone scintigraphy. Likewise, these abnormalities with abnormal uptake of radiotracer (either increased or decreased) may pose a significant challenge to interpretation and may mimic serious lesions particularly in patients suspected of metastatic skeletal diseases. To the best of our knowledge, no case of SD on bone scintigraphy has been reported.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Conflict of Interest
There are no conflicts of interest.
Financial support and sponsorship
Nil.
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References
- 1 McClure JG, Raney RB. Anomalies of the scapula. Clin Orthop Relat Res 1975;110:22-31.
- 2 Guillaume R, Nectoux E, Bigot J, Vandenbussche L, Fron D, Mézel A, et al. Congenital high scapula (Sprengel's deformity): Four cases. Diagn Interv Imaging 2012;93:878-83.
- 3 Cavendish ME. Congenital elevation of the scapula. J Bone Joint Surg Br 1972;54:395-408.
- 4 Currarino G, Sheffield E, Twickler D. Congenital glenoid dysplasia. Pediatr Radiol 1998;28:30-7.
- 5 Simanovsky N, Hiller N, Simanovsky N. Partial duplication of the scapula. Skeletal Radiol 2006;35:696-8.
- 6 Ozsahin M, Uslu M, Inanmaz E, Okur M. Bilateral congenital undescended scapula (sprengel deformity). Am J Phys Med Rehabil 2012;91:374.
- 7 Williams MS. Developmental anomalies of the scapula-the “omo”st forgotten bone. Am J Med Genet A 2003;120A: 583-7.
- 8 Kadavkolan AS, Bhatia DN, Dasgupta B, Bhosale PB. Sprengel's deformity of the shoulder: Current perspectives in management. Int J Shoulder Surg 2011;5:1-8.
Address for correspondence
Publikationsverlauf
Eingereicht: 00. 00 2019
Angenommen: 00. 00 2019
Artikel online veröffentlicht:
22. April 2022
© 2019. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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References
- 1 McClure JG, Raney RB. Anomalies of the scapula. Clin Orthop Relat Res 1975;110:22-31.
- 2 Guillaume R, Nectoux E, Bigot J, Vandenbussche L, Fron D, Mézel A, et al. Congenital high scapula (Sprengel's deformity): Four cases. Diagn Interv Imaging 2012;93:878-83.
- 3 Cavendish ME. Congenital elevation of the scapula. J Bone Joint Surg Br 1972;54:395-408.
- 4 Currarino G, Sheffield E, Twickler D. Congenital glenoid dysplasia. Pediatr Radiol 1998;28:30-7.
- 5 Simanovsky N, Hiller N, Simanovsky N. Partial duplication of the scapula. Skeletal Radiol 2006;35:696-8.
- 6 Ozsahin M, Uslu M, Inanmaz E, Okur M. Bilateral congenital undescended scapula (sprengel deformity). Am J Phys Med Rehabil 2012;91:374.
- 7 Williams MS. Developmental anomalies of the scapula-the “omo”st forgotten bone. Am J Med Genet A 2003;120A: 583-7.
- 8 Kadavkolan AS, Bhatia DN, Dasgupta B, Bhosale PB. Sprengel's deformity of the shoulder: Current perspectives in management. Int J Shoulder Surg 2011;5:1-8.



