US For Glenohumeral Dysplasia
US For Glenohumeral Dysplasia
US For Glenohumeral Dysplasia
https://doi.org/10.1007/s00247-021-05180-y
Received: 18 May 2021 / Revised: 10 July 2021 / Accepted: 2 August 2021 / Published online: 21 September 2021
# The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2021
Abstract
Brachial plexus birth injury can lead to irreversible neuromuscular dysfunction and skeletal deformity of the upper extremity and
shoulder girdle, ultimately resulting in glenohumeral dysplasia. Diagnosis and treatment of affected infants requires a multi-
disciplinary approach in which imaging plays a vital role. While MRI is excellent for assessing both the shoulder and spine of
these children, it is costly and requires sedation and is thus typically reserved for preoperative planning. US, however, is
inexpensive, dynamic and readily available and provides excellent visualization of the largely cartilaginous glenohumeral joint.
As such, it has become a highly useful modality during early diagnosis and follow-up of children with brachial plexus birth
injuries. In this review, we describe the relevant anatomy of the glenohumeral joint, outlining the normal sonographic appearance
as well as providing tips and tricks for identifying and characterizing pathology.
Keywords Brachial plexus . Brachial plexus birth injury . Glenohumeral dysplasia . Infants . Magnetic resonance imaging .
Musculoskeletal . Ultrasound
Introduction motor innervation to the shoulder, upper chest, arm and hand.
Brachial plexus birth injury most commonly involves the up-
Brachial plexus birth injury has been reported to occur in 1.5– per trunks of the C5–T1 nerve roots, specifically C5–C6,
4 per 1,000 live births, though some report the incidence has though isolated lower trunk injuries rarely occur [5, 6]. The
been decreasing, possibly a result of an increasing rate of most common form of nerve injury — occurring in 70–90%
cesarean sections [1–3]. Typically seen during vaginal deliv- of children with brachial plexus injury — is neurapraxia, a
ery, downward traction on the infant’s head and neck com- focal traumatic “stretching” injury without axonal disruption;
bined with partial obstruction of the shoulders by the maternal this typically resolves spontaneously, usually by 2–3 months
pubis leads to predictable patterns of nerve injury [4]. Many of age. However, the remaining 10–30% of these children
risk factors for brachial plexus birth injury have been de- have residual impairment [7]. In 1987, the Narakas classifica-
scribed and include shoulder dystocia, macrosomia, instru- tion divided obstetric birth palsies into four groups based on
mentation during delivery, and gestational diabetes; some the palsies observed clinically. Widely adopted in clinical
children, however, present in the absence of known predispos- practice, each Narakas group corresponds with the level of
ing conditions [2, 5]. nerve root injury, whereby the higher the classification, the
The brachial plexus is primarily composed of anterior rami more nerve roots are injured [8].
originating from C5–T1 nerve roots, and provides sensory and Unfortunately, brachial plexus birth injury can lead to irre-
versible neuromuscular dysfunction and skeletal deformity of
the shoulder girdle, which can include joint contractures, pos-
terior humeral head subluxation and glenohumeral dysplasia
* Sarah J. Menashe
[email protected] [9–11]. The true incidence of glenohumeral dysplasia, a well-
recognized complication of brachial plexus birth injury in
1
children with persistent neurologic defect at 3 months, varies
Department of Radiology, Seattle Children’s Hospital
and University of Washington School of Medicine,
widely in the literature [6, 12]. However, though a result of the
4800 Sandpoint Way NE, MA.7.220, Seattle, WA 98105, USA initial insult to the brachial plexus, glenohumeral dysplasia is
2
Seattle Children’s Hospital,
considered a separate and evolving pathology with respect to
Medicine and University of Washington School of Medicine, the developing shoulder and can lead to significant shoulder
Seattle, WA, USA dysfunction and morbidity [13–15]. The pathophysiology of
Pediatr Radiol (2022) 52:1648–1657 1649
glenohumeral dysplasia is not entirely understood but is (Sup) the forearm and externally rotating (ER) the shoulder.
thought to be the result of an imbalance between the relatively Our institution adopted the use of the Sup-ER splint, and we
unaffected internal rotators and the paralytic external rotators, employ US monthly to evaluate the glenohumeral relationship
such that when left untreated, posterior subluxation and even- both in and out of the splint to ensure the splint is maintaining
tual dislocation of the humeral head ensues [16]. As with the proper reduction. However, if the Sup-ER splint is unable to
pediatric hip joint, evolving joint incongruency hinders nor- maintain proper glenohumeral alignment and subluxation oc-
mal development of the glenoid, eventually leading to curs, the infant is placed in a spica cast (Fig. 1).
glenohumeral dysplasia [17, 18]. Interestingly, more profound
nerve injury, and correspondingly higher Narakas classifica-
tion in the neonatal period, has been associated with less Ultrasound imaging
glenohumeral dysplasia in older children; this is thought to
be a function of earlier surgical intervention in more severely Imaging of infants with brachial plexus birth injury is done
affected children [19]. primarily with gray-scale static and dynamic US and non-
contrast-enhanced MRI. CT has been used historically,
though it is no longer a preferred modality, particularly in
Clinical presentation, treatment and imaging younger children and infants who are more susceptible to
algorithm the risks of ionizing radiation and in whom unossified carti-
lage is better visualized with other cross-sectional modalities
Our multidisciplinary brachial plexus injury clinic evaluates [5, 25]. MRI has the advantage of being able to evaluate mor-
infants starting at 1 month of age because the majority of phological changes of the glenohumeral joint and shoulder
neurapraxic injuries have resolved by this time. Assessments girdle, in addition to allowing for accurate measurement of
at each clinic visit include passive range of motion, active range many other established parameters of the bony and cartilagi-
of motion and Active Movement Scale scoring, which grades nous glenohumeral joint. As such, MRI is considered the gold
the infant’s ability to move the limb both with gravity eliminat- standard in the preoperative assessment of glenohumeral dys-
ed and against gravity [20]. Infants are followed up at least plasia, and it is often performed in older infants or in more
every 3 months to determine their recovery trajectory, and in- advanced cases of dysplasia to delineate complex anatomy
terventions are based on the algorithm established by the [25]. Nevertheless, MRI has the distinct disadvantage of re-
Hospital for Sick Children in Toronto, Canada [21]. quiring sedation outside of the neonatal period, the long-term
Glenohumeral US screening typically begins at 3 months of effects of which are poorly understood, in addition to being
age because glenohumeral subluxation has been shown to be more costly and, often, less available [26]. Thus, in neonates
present by this age. Infants are screened at 3 months and and infants without severe palsy requiring immediate surgical
6 months of age, regardless of clinical exam, and intervention intervention, US may be used in the screening evaluation of
is based on humeral head alpha angle (as described later) and glenohumeral dysplasia. A rapid, safe, cost-effective, dynamic
passive range of motion. Unfortunately, the clinical exam is not and readily available modality, US is valuable in the early
always predictive of shoulder subluxation, with many infants assessment and follow-up of these children [4, 25, 27]. As
exhibiting abnormal alpha angles despite having full passive noted, in our multidisciplinary brachial plexus injury clinic,
range of motion [19, 22]. If the alpha angle is greater than 40° US comprises the vast majority of imaging in infants with
and there is restricted passive external rotation, infants undergo suspected brachial plexus birth injury, and for routine
manual shoulder reduction, injection of botulinum toxin A into glenohumeral dysplasia monitoring to guide treatment
the pectoralis major, latissimus and subscapularis muscles, and efficacy.
application of a shoulder spica cast for 6 weeks to maintain joint
reduction [23]. Of note, casting for glenohumeral dysplasia General approach
involves abducting and externally rotating the shoulder, similar
to hip spica casting for developmental hip dysplasia. US is Ultrasound of the glenohumeral joint is performed from a
initially repeated when the cast is removed to ensure appropri- posterior approach with a high-frequency linear transducer
ate glenohumeral reduction, then again at 1–2 months post cast (e.g., 10–15 mHz). Infants are typically seated on the exami-
removal to ensure the reduction is maintained. nation table facing away from the sonographer, head and trunk
Prevention of shoulder subluxation can be a challenging supported by the caregiver, though the child might also be
endeavor for children with brachial plexus birth palsy. One placed in the lateral decubitus position. The arm is then
reasonable option to address this issue is a splinting protocol adducted against the body (i.e. elbow against the infant’s side
developed at British Columbia Children’s Hospital called the and wrist against the lower torso) with the shoulder in internal
Sup-ER splint [24]. The splint is designed to keep the shoulder rotation, to accentuate or unmask posterior glenohumeral sub-
passively positioned in glenohumeral reduction by supinating luxation, if it is present. The probe is placed in a transverse
1650 Pediatr Radiol (2022) 52:1648–1657
oblique plane along the axis of the scapular spine (Fig. 2). positioning and imaging approach to these children.
Both affected and unaffected sides are imaged to provide in- Furtherm ore, the radiologist’s understanding of
ternal controls for comparison. If the child has undergone glenohumeral anatomy and the expected evolution of
splinting or casting, this is usually removed prior to imaging, glenohumeral dysplasia is paramount for accurate
though both in- and out-of-splint imaging can be performed. assessment.
Two to three static images of the affected shoulder can be
helpful in ascertaining the humeral head alpha angle, and cine Normal anatomy
sweeps through the joint can be performed either routinely or
only for troubleshooting purposes. With the probe in place posteriorly, the posterior scapular
Sonographic evaluation of the humeral head with re- spine is readily identified as a linear, highly echogenic struc-
spect to the scapula has been likened to that of the hip, ture, deep to which resultant acoustic shadowing precludes
where evaluation of dysplasia is both reliable and repro- visualization of deeper intrathoracic structures. At the lateral
ducible in experienced hands [28]. Conversely, a lack of margin of the scapula lies the bony glenoid; it is typically
operator expertise can lead to false-negative or -positive echogenic with a sharp posterior margin or corner, similar
exams. It is therefore important that sonographers and to that seen along the superolateral acetabular roof in the
interpreting radiologists alike are trained in the optimal pediatric hip. The normal glenoid fossa angles steeply away
from the transducer such that its anterior margin is difficult to
visualize from this posterior approach. The overlying
glenoid cartilage is a thin, homogeneously hypoechoic,
cap-like structure, which, along with the subjacent bony
glenoid, can be likened to a “steep cliff” on sonographic
images. Immediately superficial to the posterior corner of
the bony glenoid and the overlying cartilaginous cap, one
can identify the posterior component of the glenoid labrum,
a circumscribed, triangular, fibrocartilaginous structure that
is typically more echogenic than the surrounding subcuta-
neous tissues. Finally, the humeral head is recognized
more laterally as a round, largely hypoechoic structure
with a speckled echotexture that is similar to other epiph-
yseal cartilaginous structures. A central rounded
echogenicity with posterior acoustic shadowing might be
present soon after birth and reflects the normal ossifica-
tion center, a structure that increases in size during infan-
cy. The humeral head should be spherical in configuration
with smooth circular margins throughout. The soft tissues
Fig. 2 Clinical image shows glenohumeral US acquisition from the superficial to the echogenic scapula at this level consist
posterior approach with the infant in the seated position, the arm
adducted, flexed at the elbow, and internally rotated with the elbow at
primarily of the deltoid and infraspinatus muscles, though
the infant’s side and the wrist along the lower torso. The US probe is held portions of the supraspinatus might also be evident
in a transverse oblique plane in the axis of the scapular spine (Fig. 3) [27].
Pediatr Radiol (2022) 52:1648–1657 1651
Fig. 4 Glenohumeral joint in left arm palsy. a, b Transverse oblique US images of the glenohumeral joint in a 3-month-old boy with left arm palsy. Left-
side image (a) demonstrates a smaller humeral head epiphyseal ossification center when compared to the right (b)
1652 Pediatr Radiol (2022) 52:1648–1657
Fig. 5 Suboptimal positioning during glenohumeral head imaging. a, b humeral head, simulating posterior subluxation and apparent dysplasia.
Transverse oblique US images in a 5-month-old girl’s left glenohumeral In intermediate external rotation (b), the humeral head has a less
joint with the shoulder in maximal (a) and intermediate (b) external accentuated oblong configuration rather than the normal spherical
rotation. In maximal external rotation (a), the greater tuberosity shape, which could still be falsely interpreted as dysplasia
(arrows) comes into view and creates an oblong appearance to the
comparisons to the unaffected shoulder or even more remote head displacement. Some authors have used US to measure
images of the affected side. This might be evident as overall glenoid version, or the degree of glenoid sloping as noted on
decreased muscle bulk or increased muscle echogenicity, sug- an axial images, as well [25].
gestive of fatty infiltration (Fig. 7) [32]. Because muscle atro-
phy in this setting reflects sequela of nerve injury, this can take Alpha angle
time to develop and might not be identified in very young
infants or in children with milder dysplasia. Additionally, The humeral head alpha angle is obtained by drawing a line
asymmetrical arm positioning — whether iatrogenic or as a along the posterior margin of the scapula and extending it
result of dysplasia — can lead to perceived differences in through the humeral head, then drawing a second line origi-
muscle bulk that might be inaccurate. For example, if the nating from the posterior glenoid margin and extending it
unaffected side is less internally rotated, the deltoid might be tangential to the posterior margin of the humeral head. The
less stretched over the scapula and appear symmetrical or even angle that is formed along the posterolateral intersection of
smaller than the affected side (Fig. 7). these lines is the humeral head alpha angle. A normal angle
is considered approximately 30° or less, with angles greater
Quantitative measurements in glenohumeral than 30° indicating increasing posterior subluxation of the
dysplasia humeral head [12]. One must take care not to obtain this mea-
surement on an image captured in external rotation because
Measurements of the glenohumeral joint that can be made on this can underestimate the alpha angle and result in a normal
US include the alpha angle and, in neonates, posterior humeral angle measurement in children with mild dysplasia. Likewise,
Fig. 6 Glenoid and labrum. a Transverse oblique US image in a 1-year- (open arrow). b Transverse oblique US image of the normal contralateral
old girl with right-side glenohumeral dysplasia. Image of the right glenohumeral joint in the same girl is provided for comparison. Note both
shoulder shows a rounded configuration of the posterior glenoid margin the sharp margin of the posterior glenoid and the steep slope of the normal
(solid arrow), a shallow visualized glenoid fossa (arrowhead) and an glenoid fossa
irregularly contoured posterior labrum with heterogeneous echogenicity
Pediatr Radiol (2022) 52:1648–1657 1653
Fig. 7 Shoulder muscles. Serial transverse oblique US images in an right side at 7 months of age demonstrates an interval increase in
infant girl with right-side glenohumeral dysplasia. a, b US images at echogenicity and decrease in size of the right infraspinatus muscle,
2 months of age show that although there is right brachial plexus palsy, consistent with fatty atrophy. d Axial dual-echo steady-state (DESS)
the right infraspinatus muscle (a) appears fairly symmetrical or even MRI sequence of bilateral shoulders at 10 months of age confirms the
larger than the normal left side (b). c An additional US image of the atrophy of the right infraspinatus muscle
in external rotation, the greater tuberosity, which is humeral head by the glenoid, but posterior humeral head dis-
posterolaterally located along the proximal humerus, comes placement is recorded as a percentage rather than an angle. To
into view. This can mimic the posterior margin of the humeral make this measurement, the posterior scapular line is ex-
head and lead to overestimating the alpha angle (Fig. 8). tended laterally through the entirety of the humeral head.
A perpendicular line is then drawn from the scapular line
Posterior humeral head displacement to the posterior margin of the humeral head. That value is
divided by the greatest diameter of the humeral head, then
Posterior humeral head displacement can also be assessed by multiplied by 100, resulting in the percentage of the hu-
sonography [28]. This calculation is similar to humeral head meral head that lies posterior to the scapular line. A nor-
alpha angle in that it is a metric for posterior uncovering of the mal value is considered less than 50% [28]. Posterior
Fig. 8 Alpha angle. a, b Transverse oblique US images of bilateral posterior margin of the humeral head is appropriately rounded and
glenohumeral joints in a 3-month-old boy with left glenohumeral smooth, suggesting that the infant’s shoulder is internally rotated rather
dysplasia. The normal alpha angle of 25° in the right shoulder (a) is than externally rotated, which might bring the irregular greater tuberosity
obtained from the intersection of the posterior scapular line and the line into view. The alpha angle on the affected left side (b) is 50°, indicating
tangential to the posterior margin of the humeral head. Note that the posterior subluxation
1654 Pediatr Radiol (2022) 52:1648–1657
Fig. 9 Posterior humeral head displacement. a, b Transverse oblique US humeral head diameter (dotted line) and multiplied by 100 to obtain a
images in a 4-month-old girl with shoulder contractures. The posterior percentage. The normal right side (a) has a posterior humeral head
humeral head displacement is obtained by drawing a line parallel to the displacement percentage less than 50%, a normal value, while the
scapular spine and measuring perpendicularly to the posterior margin of affected left-side (b) posterior humeral head displacement percentage is
the humeral head (solid line). This value is divided by the maximal greater than 50%, considered abnormal
Pediatr Radiol (2022) 52:1648–1657 1655
Fig. 10 MRI in advanced dysplasia. Axial dual-echo steady-state (DESS) posterior humeral head subluxation. The right glenoid (solid arrow) is
image in a 3-year-old boy with left glenohumeral dysplasia demonstrates normal; note that the normal glenoid fossa is perpendicular to the long
left glenoid retroversion (posteromedial sloping; dotted arrow) with axis of the scapula
Surgical treatment injured, considerations are made for nerve transfers rather than
reconstruction. With this technique, the site of the nerve injury
When surgery is required, the persistent weakness pattern, is bypassed, the existing nerve connection to the muscle is not
Narakas classification and MRI findings are considered for disrupted, and the nerve supply to the muscle is augmented by
treatment planning. Infants with more than upper trunk in- coaptation of a healthy adjacent nerve to the target peripheral
volvement, or in whom there is suspicion of nerve root avul- nerve [35]. This technique has been shown to have similar
sions or ruptures, should undergo nerve reconstruction. Often results to nerve reconstruction with the benefit of less opera-
with the surgical dissection, a neuroma involving one or more tive time and no lower extremity scar from sural nerve har-
nerve roots at the trunk level of the brachial plexus is found. vesting [35]. Outcomes literature for rebalancing the shoulder
This is removed and replaced by sural nerve grafts that allow muscles after nerve surgery reported that about 60% of pa-
for axon regeneration and reorganization to achieve sufficient tients have functional shoulder motion and do not require
nerve supply to the affected muscles [34]. If C5–6 are solely secondary tendon transfer surgery [36].
35. O’Grady KM, Power HA, Olson JL et al (2017) Comparing the Publisher’s note Springer Nature remains neutral with regard to jurisdic-
efficacy of triple nerve transfers with nerve graft reconstruction in tional claims in published maps and institutional affiliations.
upper trunk obstetric brachial plexus injury. Plast Reconstr Surg
140:747–756
36. Segal D, Cornwall R, Little KJ (2019) Outcomes of spinal
accessory-to-suprascapular nerve transfers for brachial plexus birth
injury. J Hand Surg 44:578–587