Page 39 - Delaware Medical Journal - September/October 2018
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CASE REPORT
shoulder deformity. Figure 2D shows an anteroposterior view of the left shoulder of an age- and gender-matched non-OI patient.
VIGNETTE 3. Figure 3A demonstrates
an anteroposterior view of the right shoulder of a 12-year-old boy with severe OI, also a manual and power wheelchair user. Shoulder abduction is limited to
70 degrees, as is the ability to perform overhead activities. Intramedullary rodding and humeral realignment was performed using a retrograde approach due to this patient’s severe shoulder protrusio.
DISCUSSION
The presence of shoulder deformity in the osteogenesis imperfecta (OI) population is notable for an envelopment of a typically round humeral head in an acromial process reaching across and around the humeral head, often with an apex dorsal downward hook of the distal third of the
of the humeral head or glenoid. We seek to introduce the concept of shoulder protrusio in patients with severe OI. This report is descriptive in nature and seeks to establish the phenomenon to set the stage for further investigation.
The measurement of acetabular protrusio
and other radiographic measurements performed on an anteroposterior (AP) view of the pelvis, including the lateral- center-edge angle of Wiberg and other radiographic parameters.5,9 There are also a number of measurements of the shoulder to assess acromial coverage of the humeral head, described in the shoulder literature, mostly in relation to rotator cuff pathology such as the acromion index,6
the critical shoulder angle7 and the lateral acromion angle.10 The angles measured on the AP view of the shoulder require a true
AP view of the glenohumeral joint. In our population of OI patients, unfortunately, this true AP view is often limited by the deformity of the shoulder itself, deformity of the associated cervical spine and trunk,
Advanced imaging modalities such as MRI will provide further information regarding the three-dimensional nature of shoulder protrusio as well as information regarding the status of the soft tissues, such as the rotator cuff. Ultrasound of
the shoulder to evaluate rotator cuff pathology in manual wheelchair users with spinal cord injury has been found to be a reliable method.11 There is no such data on the utility of ultrasound of the shoulder in patients with OI. This has led us to undertake efforts to validate
the use of ultrasound in the OI shoulder and subsequently use ultrasound data to use, shoulder pain, and quality-of-life measures.
OI patients with severe, progressive upper-extremity deformity interfering
with functional abilities are often indicated for realignment and intramedullary rodding of the upper extremity long bone segments.12-14 Antegrade insertion of a humeral intramedullary rod requires access to the humeral head and may be affected by the shoulder protrusio in patients with severe OI. In their report of a single-center surgical experience treating humeral deformity and fractures in children with OI using the Fassier-Duval telescopic nail, Grossman et al.13 describe their antegrade surgical approach to the humerus through a minimal, deltoid-splitting incision exposing and splitting the supraspinatus insertion. They also report an alternative approach in patients with severe proximal humerus varus deformity to access the medial aspect of the greater tuberosity without impingement on the acromion by guided entrance point posterior to
1A. Anteroposterior (AP) view of the left shoulder demonstrating the envelopment to the humeral head in a down-sloping overreaching acromion process.
1B. Three-dimensional CT scan of the left shoulder further demonstrating the envelopment of the humeral head in the acromion process.
1C. AP view of the left shoulder following the acromioplasty procedure.
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FIGURE 1. 15-year-old boy with severe OI and left shoulder pain
