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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 3  |  Issue : 2  |  Page : 67-71

The Role of Imaging in Diagnosis and Management of Congenital High Scapula (Sprengel’s Deformity): Case Report and Review


1 Department of Radiology, Taher Sfar University Hospital, Mahdia, Tunisia
2 Department of Physical Medicine Rehabilitation, Taher Sfar University Hospital, Mahdia, Tunisia

Date of Web Publication16-Jul-2018

Correspondence Address:
Nada Garrouche
Rue Habib Zine el Abidine n°200 (7) Sahloul 2 Sousse 4054
Tunisia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/pors.pors_11_16

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  Abstract 

Sprengel’s deformity is the congenital failure of descent of the scapula. The diagnosis is based on a clinical examination and radiological procedures. Volume rendering three-dimensional computed tomography reconstructions analyze the precise topography and spatial proportions of examined bone structures. It enables an optional rotation of visualized bone structures to clarify the anatomical abnormalities. Ultrasound and magnetic resonance are useful in prenatal management and for the diagnosis of concomitant abnormalities. In this paper, we report our imaging experience from one child with Sprengel’s deformity and discuss the importance of imaging techniques with a particular focus on the role of three-dimensional reconstructions.

Keywords: Congenital high scapula, CT, MRI, Sprengel’s deformity, ultrasound, volume rendering 3D-CT


How to cite this article:
Garrouche N, Jerbi S, Chouchane N, Kessomtini W, Hamza H. The Role of Imaging in Diagnosis and Management of Congenital High Scapula (Sprengel’s Deformity): Case Report and Review. Paediatr Orthop Relat Sci 2017;3:67-71

How to cite this URL:
Garrouche N, Jerbi S, Chouchane N, Kessomtini W, Hamza H. The Role of Imaging in Diagnosis and Management of Congenital High Scapula (Sprengel’s Deformity): Case Report and Review. Paediatr Orthop Relat Sci [serial online] 2017 [cited 2018 Dec 11];3:67-71. Available from: http://www.pors.co.in/text.asp?2017/3/2/67/236711


  Introduction Top


Sprengel’s deformity, also called congenital elevated (or high) scapula or undescended scapula, is the most frequently seen congenital anomaly of the shoulder girdle.[1],[2],[3],[4] It is a result of the failure in the scapula’s caudal migration during early intrauterine life. This deformity causes functional and aesthetic problems and the treatment, when indicated is surgical, aiming to correct cosmetic deformity and improve the extremity function.[5] Computed Tomography (CT) scans and radiographs are conventional imaging techniques required for preoperative assessment. The aim of this paper is to present the imaging aspect of this rare deformity in the conventional and less conventional imaging techniques such as Magnetic Resonance Imaging (MRI).


  Case Report Top


A 4-year-old boy was referred for a children’s surgery consultation for shoulder asymmetry. Clinical examination confirmed a distinctly raised left scapula visible when the patient was dressed up. The shoulder joint were elevated by 3 cm comparatively to the opposite side which was normal, making it a Cavendish grade 3 deformity. Shoulder mobility was slightly altered with a limited abduction of the left shoulder to 95°. The omovertebral structure was palpable on the initial exam. The rest of the physical exam showed no other skeletal abnormalities. X-ray examination showed left Sprengel deformity and the presence of an omovertebral bone.

The CT scan and its two dimensional, and above all, Volume rendering three dimensional - CT reconstructions were done in order to evaluate the omovertebral association and demonstrate the spectrum of abnormalities that was required for thorough planning of surgery. 3D-CT were used preoperatively to evaluate the pathologic anatomy of the deformity, including malposition and dysplasia of the scapula. Volume rendering 3D- CT reconstructions showed, by comparison with the opposite side, the abnormally high position of the left scapula, and the presence of a fibro-osseous connection arising from the lamina of a cervical vertebra of C-7 reaching up to the medial border of the scapula, identifying the omovertebral bone [[Figure 1] and [Figure 2]]. We assessed the height to width ratio (1.42) on the scapular posterior view [[Figure 3] and [Figure 4]], the superior displacement ratio (1.22) [Figure 5] and rotational difference on the trunk posterior view [Figure 6], as well as the anterior curvature of the supra spinous portion on the scapular medial view. The affected scapula has been described as hypoplastic with a decreased height to width ratio and an anteriorly curved supraspinous portion.
Figure 1: 3D­CT reconstructions image in anterior trunk view image showing the difference between the positions of the scapulae: superior displacement of the left scapula in comparison to the controlateral shoulder

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Figure 2: 3D­CT reconstructions image in posterior trunk view demonstrating the elevated position of the left scapula (red arrow). An omovertebral bone (white arrow) is visible between the lamina of a cervical vertebra and the medial border of the scapula. The shoulder joints (asterisks) are asymmetrical

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Figure 3: 3D­CT reconstructions image in scapular medial view. The height of the scapula is the measured length of the medial border between the superior angle and the inferior angle parallel to the cavity of the glenoid (gray line)

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Figure 4: 3D­CT reconstructions image in posterior trunk view. The width of the scapula is measured from the lateral end of the acromion to the most medial part of the spine scapula (orange line). The width of the body of the scapula is measured as the distance from the glenoid to the most medial part of the scapula (yellow line)

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Figure 5: 3D­CT reconstructions image in posterior trunk view. The vertical displacement of the scapula in comparison to the opposite side is measured by drawing two lines (A and B) from the center of the glenoid cavity, perpendicular to the vertebral axis line (blue line). Vertical displacement is the percentage of the difference between the levels of the two glenoids (C) divided by the height of the opposite scapula

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Figure 6: Preoperative trunk posterior 3D-CT view. Lines are drawn from the center of the glenoid cavity perpendicular to the vertebral axis line. The rotational difference is measured between the angles of tilt of both sides. Rotational difference = B–A

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CT scan showed none of the frequently associated malformations including fused ribs, chest wall asymmetry, cervical vertebral fusion, cervical ribs, congenital Scoliosis or cervical spina bifida.

The outcome of surgery was assessed on the basis of changes in shoulder abduction and the imaging findings evaluated using Cavendish 4 grades classification.

No abdominal or thoracic associated malformations have been found on the Ultrasound. Surgery was planned at the age of 5 for aesthetic reasons and to improve functioning of the upper limb. The Procedure consisted of simple ablation of the omovertebral structure. Postoperative recovery was uneventful.


  Discussion Top


Congenital high scapula, commonly known as Sprengel’s deformity, is a rare congenital condition of unknown etiology. Occurring equally in both sexes,[3] it is usually detected at birth or later in childhood, in one or both shoulders.[6] The scapula differentiates, at about five weeks of the gestation, opposite the inferior cervical vertebrae. Normally, it lies between the 2nd and the 7th–8th thoracal vertebrae on the posterior thoracic wall.[2] The undescended scapula occurs at the term of 9 to 12 weeks of fetal development. It is situated 1 to 12 cm (2 to 4 vertebral bodies) higher than its fellow, with an average of 3 to 5 cm. The high scapula is also rotated upon its frontal or sagittal axis.[3] The high position of the scapula in the process of skeletal development leads to a series of other musculoskeletal defects including hypoplasia, medialization and adduction of the scapula, prominence of its upper angle, distal rotation and lateral angulation of the glenoid cavity, changes in the position of the clavicle, anomalies of the cervicothoracic vertebrae and ribs, and muscular hypoplasia or atrophy of the shoulder musculature.[5],[7] Children with Sprengel’s deformity have the restriction of shoulder and cervical spine movements resulting in difficulties with sliding down shoulder strap and abducting and raising the upper limb.[8] Therefore, the undescended scapula is often accompanied by significant cervicothoracic spine and thoracic rib cage abnormalities, and changes in the clavicle position.[5],[9],[10]

In 25–50% of the cases, a bony structure is interposed between the scapula and the cervical spine called omovertebral bone.[2] It is a rhomboid-shaped or trapezoid-shaped structure of cartilage or bone that usually lies in a strong fascial sheath, which extends from the superior-medial border of the scapula to the spinous processes, lamina, or transverse processes of the cervical spine, most commonly at C4 to C7.[11]

Sprengel’s deformity has been associated with a variety of other congenital anomalies including Floating–Harbor syndrome, velocardial facial syndrome, association with the VATER complex (vertebral defects, imperforate anus, trachea-esophageal fistula, and radial and renal dysplasia), Klippel–Feil syndrome, Greig’s syndrome, Poland’s syndrome, Goldenhar syndrome, and X-linked dominant hydroecephalus.[12]

Cavendish[13] extensively studied the appearance and dysfunction shoulders in 100 patients and proposed a grading system based on the clinical examination and the cosmetic aspects of the Sprengel’s deformity.[10],[14] It differentiates four clinical grades of increasing severity, depending on the size of the swelling and how far the scapula is raised: grade I (very mild deformity) is when the deformity is minimally invisible when patient is dressed. Grade II (mild) is when the deformity is appears as a bump due to a convexity of the superomedial portion of the high scapula. Grade III (moderate) is when a visible elevation of the affected shoulder by 2–5 cm. Grade IV (severe) is when the elevation of the affected shoulder is more than 5 cm, accompanied by neck webbing.[2],[13]

Imaging plays a prominent role in preoperative evaluation. Prenatal diagnosis, on the basis of ultrasonography, is possible.[12] Chinn[12] reported that the diagnosis relies on the observation of only one scapula on a true axial view which indicates that the imaged scapula is elevated. An ipsilateral asymmetric nuchal soft tissue thickening should also be found in the coronal and axial planes in comparison with the unaffected side. The diagnosis is easily confirmed whenever an omovertebral bone is present.[12] Cho et al.[1] outlined the interest of three-dimensional ultrasound imaging in preoperative planning for the description of any associated malformation. The radiologist evaluates skeletal anomalies (bilateral clavicular anomalies including pseudoarthroses or hypoplasia are seen in Floating–Harbor syndrome), genitourinary anomalies (cryptorchidia or renal dysplasia seen in Floating Harbor complex, VATER complex, or Greig’s syndrome), vertebral anomalies (vertebral fusion or spina bifida related to Klippel–Feil syndrome), or thoracic anomalies (absence of the chest muscle, dextrocardia and diaphragmatic hernia seen in Poland’s syndrome).

Ultrasound also allows the assessment of shape modifications, height-to-width ratio, the areas of both scapulae, the anterior curvature of the supraspinous portion and glenoid version on scapular posterior, medial, and inferior views.[1]

Postnatal diagnosis is based on CT scan which can delineate fine skeletal structures.[15] Volume rendering is the preferred technique for musculoskeletal system examinations.[15] Improvements in software have made it quicker and easier to produce high quality images.[16] The utilization of the spiral technique has allowed the rapid procurement of overlapping slices for the 3D reconstruction of the data without any additional radiation exposure to the patients.[16] Volume rendering 3D-CT reconstructions are very useful for detecting an omovertebral bone, giving its site and size, and evaluating the degree of dysmorphia of the scapula (lateral tilt in the frontal plane, anterior tilt in the sagittal plane and medial tilt in the transverse plane).[17] It is also useful for detecting associated bone abnormalities, particularly of the vertebrae.[1],[16],[17]

Technically, six images are required: the trunk anterior or posterior view corresponded to the image of the whole shoulder girdle as seen from the front [Figure 1] or behind [Figure 2], that of the superior trunk outlet to the image of the upper trunk seen from above, and the scapular posterior view to the en-face image of the scapula as seen from behind. The scapular medial view is obtained by rotating the single scapula around the vertical axis, and the scapular inferior view by rotating it around the transverse axis. The height of the scapula is the length of the medial border measured on the scapular medial view between the superior angle and the inferior angle parallel to the cavity of the glenoid [Figure 3]. The scapular posterior view is used to measure the width of the scapula. It is measured from the lateral end of the acromion to the most medial part of the spine scapula. The width of the body of the scapula is measured as the distance from the glenoid to the most medial part of the scapula [Figure 4]. The trunk posterior view evaluates the vertical displacement in comparison to the opposite side.

Lines are drawn from the center of the glenoid cavity perpendicular to the vertebral axis line. Vertical displacement is the percentage of the difference between the heights levels of the two glenoids divided by the height of the opposite scapula. Positive sign denotes superior displacement and negative sign inferior displacement [Figure 5]. The elevation is difficult to estimate, because the height of the scapula is reduced in comparison to the unaffected shoulder. If the measurement is performed to compare the height of the glenoids, the amount of the elevation will be underestimated because of the associated rotatory deformity.[14] This technique seems to be the most accurate and reproducible method of evaluation.[1],[14]

Recognition of the glenoid version is important for preoperative evaluation, because surgical strategies can change significantly in the presence of major retroversion.[18],[19],[20] The reference method for glenoid version angle measurement is the Friedman’s method[19] based on volume rendering 3D-CT superior view or on CT axial reconstruction.[18]

The rotational component of the deformity is partly responsible for the decreased shoulder abduction.[4] The anomalous connections of the scapula to the chest wall and to the spine, and the anterior curve of the supraspinous portion may also contribute to the limitation of scapulothoracic movement and poor mobility gain after surgery.[4]

The degree of humeral head subluxation should also be evaluated as inferior glenohumeral subluxation may account for near-normal abduction in the child with congenital elevation of the scapula.[21]

Plain radiography is largely used to evaluate the surgical effects on scapular lowering, the positional differences in the inferior scapular angle and the lower glenoid margin between the healthy and the affected sides. The anteroposterior X-ray of the chest is taken with the patients in the same aforementioned position preoperatively and at the final visit which gives an estimation of the height difference between the healthiest and the highest scapula. Rigault radiological classification is the objective for surgical indication.

There may be a role for MRI to assess an omovertebral connection that may not be seen on CT scans or radiographs.[2] It can be useful both for analyzing these fibrous and/or cartilaginous components and for detection-associated medullary abnormalities.[22] The omovertebral connection is screened best in coronal and axial cross­sections.[2] In the sagittal images, Dilli et al. described the appearance of omovertebral band with peripheral fat tissue as “Ra’s eye” sign seen when the patient is placed with the scapula of the healthy shoulder raised by a suitable wedge and cervical flexion to the normal side.[2],[22]

Surgical treatment for patients with the Sprengel’s deformity is indicated when patient and family expectations are either improved cosmetic or improved function.[23] Woodward’s procedure[24],[25] has been considered the gold standard and the reference procedure, with over 80% satisfactory functional and cosmetic results.

The muscles involved with scapular movement, especially the trapezius, levator scapulae and rhomboids, are often hypoplastic or infiltrated with fibrofatty tissue and the serratus anterior muscle may be weak, adding risk of postoperative scapular winging and worsening the clinical deformity.[4] After releasing the muscular and tethering structures, relocation should focus on the dominant deformity be it superior displacement or rotational deformity. MRI and CT scan help in assessing the surgical outcome by evaluating the muscles trophicity. The scapular posterior view of 3D-CT are useful in determining whether or by how much repositioning is possible[1] and how much of the scapular descent and subsequent abnormal development of bone and soft tissues affecting the entire shoulder girdle can be corrected (the scapula, its attached muscles, the shoulder joint and the clavicle).[4]

The surgical treatment is quite safe[4] with rare unpredictable complications described in literature review : poor cosmetic results, temporary Brachial plexus palsy (due to soft tissue compression), scapular winging (which is considered as a contraindication for surgical procedure if present preoperatively) and the reappearance of excised bone if the excision is incomplete.


  Conclusion Top


Sprengel’s deformity is a rare condition where imaging techniques has a major role in assessing the diagnosis and evaluating the indication and the results of the surgical treatment. CT-scan with its three-dimensional reconstruction delineates the scapular shape and the associated skeletal abnormalities such as the omovertebral bone. MRI is helpful when omovertebral bone is absent on CT scans and ultrasound plays a role in prenatal management. Although the use of these imaging techniques are still under evaluation, they are considered as effective new tools for early investigation and planning surgical management. The Sprengel’s deformity prenatal management consists of the diagnosis of the deformity and the associated congenital abnormalities of other organ systems which may require assistance through labor, delivery and postnatal care.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Cho TJ, Choi IH, Chung CY, Hwang JK. The Sprengel deformity. Morphometric analysis using 3D-CT and its clinical relevance. J Bone Joint Surg Br 2000;82:711-8.  Back to cited text no. 1
    
2.
Dilli A, Ayaz UY, Damar C, Ersan O, Hekimoglu B. Sprengel deformity: Magnetic resonance imaging findings in two pediatric cases. J Clin Imaging Sci 2011;1:13.  Back to cited text no. 2
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3.
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4.
Grogan DP, Stanley EA, Bobechko WP. The congenital undescended scapula. Surgical correction by the Woodward procedure. Bone Joint J 1983;65:598-605.  Back to cited text no. 4
    
5.
Gonen E, Simsek U, Solak S, Bektaser B, Ates Y, Aydin E. Long-term results of modified Green method in Sprengel’s deformity. J Child Orthop 2010;4:309-14.  Back to cited text no. 5
    
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7.
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8.
Nakamura N, Inaba Y, Machida J, Saito T. Use of glenoid inclination angle for the assessment of unilateral congenital high scapula. J Pediatr Orthop B 2016;25:54-61.  Back to cited text no. 8
    
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Greitemann B, Rondhuis JJ, Karbowski A. Treatment of congenital elevation of the scapula: 10 (2–18) year follow-up of 37 cases of Sprengel’s deformity. Acta Orthop Scand 1993;64:365-8.  Back to cited text no. 10
    
11.
Füllbier L, Tanner P, Henkes H, Hopf NJ. Omovertebral bone associated with Sprengel deformity and Klippel-Feil syndrome leading to cervical myelopathy. J Neurosurg Spine 2010;13:224-8.  Back to cited text no. 11
    
12.
Chinn DH. Prenatal ultrasonographic diagnosis of Sprengel’s deformity. J Ultrasound Med 2001;20:693-7.  Back to cited text no. 12
    
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Cavendish ME. Congenital elevation of the scapula. J Bone Joint Surg Br 1972;54:395-408.  Back to cited text no. 13
    
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Rockwood CA. Rockwood and Matsen’s the shoulder. Elsevier; 2017.  Back to cited text no. 14
    
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van der Molen AJ, Prokop M, Galanski M, Schaefer-Prokop CM. Spiral and Multislice Computed Tomography of the Body. Stuttgart, New York: Georg Thieme Verlag; 2003.  Back to cited text no. 15
    
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Yuksel M, Karabiber H, Yuksel KZ, Parmaksiz G. Diagnostic importance of 3D CT images in Klippel-Feil syndrome with multiple skeletal anomalies: A case report. Korean J Radiol 2005;6:278-81.  Back to cited text no. 16
    
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Rasul ME, Reddy AV. The sprengel deformity. Int J Res Med Sci 2015;3:3869-71.  Back to cited text no. 17
    
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19.
Andrin J, Macaron C, Pottecher P, Martz P, Baulot E, Trouilloud P et al. Determination of a new computed tomography method for measuring the glenoid version and comparing with a reference method. Radio-anatomical and retrospective study. Int Orthop 2016;40:525-9.  Back to cited text no. 19
    
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Friedman RJ, Hawthorne KB, Genez BM. The use of computerized tomography in the measurement of glenoid version. J Bone Joint Surg Am 1992;74:1032-7.  Back to cited text no. 20
    
21.
Nyffeler RW, Jost B, Pfirrmann CW, Gerber C. Measurement of glenoid version: conventional radiographs versus computed tomography scans. J Shoulder Elbow Surg 2003;12:493-6.  Back to cited text no. 21
    
22.
Hamner DL, Hall JE. Sprengel’s deformity associated with multidirectional shoulder instability. J Pediatr Orthop 1995;15:641-3.  Back to cited text no. 22
    
23.
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.  Back to cited text no. 23
    
24.
Wada A, Nakamura T, Fujii T, Takamura K, Yanagida H, Yamaguchi T et al. Sprengel deformity: Morphometric assessment and surgical treatment by the modified green procedure. J Pediatr Orthop 2014;34:55-62.  Back to cited text no. 24
    
25.
Woodward JW. Congenital elevation of the scapula. J Bone Joint Surg Am 1961;43:219-28.  Back to cited text no. 25
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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