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Hand Clin 24 (2008) 139–152

Elbow Instability in Children


Lisa L. Lattanza, MDa,b,*, Greg Keese, MDc
a
Division of Hand and Upper Extremity Surgery, Department of Orthopaedic Surgery, University of California,
San Francisco Medical Center, 500 Parnassus Avenue, MU320W, San Francisco, CA 94143, USA
b
Shriner’s Hospital of Northern California, Sacramento, CA, USA
c
1360 West 6th Street, Suite 305, San Pedro, CA 90732, USA

Recurrent instability in the adult elbow post in childhood and the multiple ossification centers
trauma has been well documented [1–7]; however, appearing at different times during development
little has been written about instability in the (Fig. 1). When a pediatric patient presents to the
pediatric elbow. Instability in the pediatric elbow emergency department with an elbow injury, a frac-
can be secondary to trauma, developmental disor- ture or dislocation must be ruled out. It is
ders, congenital anomalies, inherited disorders, or frequently helpful to also radiograph the uninjured
acquired systemic processes. The purpose of this extremity for a normal comparison. It is much
article is to review current concepts of injuries more common in children for a dislocated elbow
leading to elbow instability, discuss how to recog- to spontaneously reduce; therefore, a high index
nize and treat the instability, and address other, of suspicion is necessary at times to make the diag-
nontraumatic causes of elbow instability. nosis of a dislocation in a child. It is possible that
Dislocations of the elbow are the most common radiographs may benormal in appearance or have
type of dislocation in the pediatric age group. only subtle signs such as an avulsion fracture of
Elbow fractures account for 7% to 9% of all the tip of the coronoid, a radial head fracture, or
pediatric fractures [8–11], whereas the incidence a medial epicondyle fracture. The significance is
of dislocations is quoted to be 3% to 6% [12,13], that late instability can be seen when these injuries
increasing at age 13 to 14 years as the growth plates are unrecognized or undertreated.
close. Fractures and dislocations can lead to insta- The examiner must also carefully assess the
bility of the pediatric elbow by direct injury to sta- neurovascular status of the extremity. Although
bilizing structures or indirectly by way of malunion uncommon, the median nerve can become entrap-
or nonunion. ped in the joint with reduction after dislocation. It
As in the adult patient, fractures and disloca- can have devastating consequences when it goes
tions of the elbow are the leading cause of unnoticed.
instability in the elbow. The mechanism of injury
is that of a fall on an outstretched hand with an
axial load to the elbow in forearm pronation, as Traumatic causes of instability
described by O’Driscoll and colleagues [14].
Medial epicondyle fracture/nonunion
Elbow radiographs in children can be difficult to
evaluate due to the cartilaginous nature of the joint During a dislocation of the elbow, the medial
epicondyle can be disrupted and become entrapped
in the joint. Incarceration usually occurs in
* Corresponding author. Division of Hand and Up-
conjunction with an elbow dislocation and sponta-
per Extremity Surgery, Department of Orthopaedic Sur-
gery, University of California, San Francisco Medical
neous ulnohumeral reduction. The garden-variety
Center, 500 Parnassus Avenue, MU320W, San Francisco, medial epicondylar separation due to indirect ten-
CA 94143. sioning of the flexor origin generally does not
E-mail address: lattanza@orthosurg.ucsf.edu produce enough energy to displace the epicondyle
(L.L. Lattanza). below the trochlea. The epicondylar incarceration
0749-0712/08/$ - see front matter Ó 2008 Elsevier Inc. All rights reserved.
doi:10.1016/j.hcl.2007.11.007 hand.theclinics.com
140 LATTANZA & KEESE

nonunion rate, but the amount of displacement


had no correlation with fracture union. In addi-
tion, the presence of pseudoarthrosis did not im-
pact function such as symptomatic medial
instability; however, pseudoarthrosis was associ-
ated with ulnar nerve symptoms.
The significance of the medial epicondyle is the
attachment of the ulnar collateral ligament. A rare
complication of fracture nonunion is symptomatic
valgus instability of the elbow [22]. It is believed
to result from motion at the fracture site, distal dis-
placement of the epicondyle, and medial collateral
ligament stretching at the time of injury. The inci-
dence and the severity of this problem relate to the
activity demands of the patient [23,24] and have
important treatment implications in the setting of
acute pediatric medial epicondylar fractures, be-
cause operative treatment yields a higher union rate.
Fig. 1. Secondary ossification centers of the elbow. Num-
Historical treatment recommendations for me-
bers represent the year of age that the ossification center dial epicondyle fractures were made during a time
appears. (From Mizuno K, Hirohata K, Kashiwagi D. when our awareness of subtle instability was non-
Fracture-separation of the distal humeral epiphysis in existentdbefore the description of several essential
young children J Bone Joint Surg Am 1979;61:570; with physical examination maneuvers. In addition, the
permission.) past several decades have seen a surge in high-level
athletic participation without a commensurate
typically presents with significant swelling and change in the treatment philosophy. A frank
limited elbow motion, with a variable degree of ulnar discussion with the patient’s parents should ensue
neuropathy. Lateral radiographs show epicondylar regarding the risks of surgery versus possible long-
displacement at the joint line. Anteroposterior term implication of chronic instability. Operative
radiographs are often difficult to interpret due to intervention should be recommended based on
a block of elbow extension. In children 5 years or clinical findings of instability and the activity
younger, the diagnosis can only be made on clinical requirements of the individual child.
examination, arthrogram, or MRI because the The treatment of symptomatic medial epicon-
ossification center is not seen radiographically until dyle nonunion is open reduction internal fixation
age 5 to 7 years. Generally, this displacement can be or fragment excision with repair/reconstruction
manipulated back into place by applying a valgus of the ulnar collateral ligament, particularly if
stress to open the medial joint line while flexing and the fragment is small [25,26]. In the largest series
extending the elbow (the forearm is supinated and reported in the literature, Gilchrist and McKee
digits are extended, tensioning the common flexor reported excellent results using fragment excision
origin to aid in the reduction) [15]. and suture anchor fixation of the medial collateral
In rare cases, an open reduction may be ligament [22].
necessary to remove the epicondyle from the joint.
Surgical indications in the absence of entrapment
Lateral ulnar collateral ligament injury
are widely variable [16–20]. No consensus has been
after dislocation
established with respect to the amount of displace-
ment and need for surgery. Multiple investigators Posterolateral rotatory instability (PLRI) in
report success using 3 mm as a cut-off [16–18]. the adult secondary to trauma was well defined in
Blount [19] recommended greater than 5 mm, the literature by O’Driscoll and others in 1991
whereas Wadsworth [20] used displacement greater [4,14]. O’Driscoll and colleagues [27] described
than 50% of the base of the fracture fragment. a spectrum of injury to the soft tissues, with dislo-
Adding to the confusion, Josefsson and Danielsson cation traveling from lateral to medial (Fig. 2). In
[21] reported 35-year follow-up on 51 fractures most cases, the elbow becomes stable with a simple
with displacement ranging from 1 to 15 mm. Con- short period of immobilization after reduction. In
servative management yielded a high (50%) some cases, however, injury of the lateral ulnar
ELBOW INSTABILITY IN CHILDREN 141

or pain’’ in the elbow and a remote history of


vague trauma. Often, the specific type of injury is
not known. A history of open reduction internal
fixation of a radial head fracture or a lateral scar
on the elbow should also alert one to the possibil-
ity of iatrogenic injury.
Unlike adults, young children do not tend to
complain of a sensation of ‘‘giving way’’ of the
elbow with PLRI, although adolescents more often
do. Children may simply complain of pain or more
commonly present with a forearm pronation
contracture. A possible explanation for this is
that the child senses a subluxation of the joint
with extension and supination and simply begins to
guard against these motions. A contracture then
develops overtime.
Thorough examination, although important,
can be less than satisfying. Positive findings may
Fig. 2. Drawing of the Horii circle of soft tissue injury include tenderness to palpation along the course
progressing from lateral to medial. LUCL, lateral ulnar of the LUCL. The pivot shift test is rarely positive
collateral ligament; MUCL, medial ulnar collateral liga- in the office, although an apprehension sign with
ment. (From O’Driscoll. The unstable elbow. J Bone this test should lead one to at least consider
Joint Surg Am 2000;82:725; with permission.) the diagnosis. Radiographs of the injured and
uninjured side for comparison can be diagnostic.
In some cases, the subluxation of the radial head
collateral ligament (LUCL) leads to ulnohumeral
posteriorly with a widening of the ulnohumeral
joint instability as evidenced on radiography by
joint can be seen [28].
a posterior subluxation of the radial head in rela-
One should be suspicious of an LUCL injury in
tion to the capitellum (Fig. 3).
a child who has loss of forearm rotation with or
As in the adult, to make the diagnosis of PLRI,
without evidence of an old radial head fracture with
one must have a high index of suspicion. PLRI
overgrowth. MRI has not been shown to be helpful
can present in children in many ways. A docu-
in confirming the diagnosis of PLRI in the adult
mented history of an elbow dislocation is helpful;
[29]. Obtaining an MRI in a child usually requires
however, in the authors’ practice, patients are
sedation and, although it may show a tear in the
frequently seen late as referrals from other
LUCL, it does not necessarily confirm the diagnosis
institutions with a chief complaint of ‘‘stiffness
of PLRI. Ultimately, examination under anesthesia
with a well-thought-out plan for reconstruction is
in order for the pediatric patient presenting with
these complaints. Arthroscopy can be an invalu-
able tool for visualizing loose bodies, addressing
radial head overgrowth or articular damage, and
assessing stability.
Clough and colleagues [30] reported a case of
an 11-year-old boy who had a history of recurrent
dislocations after injuring his elbow falling from
his bike at age 6 years. A diagnosis of PLRI was
made by a positive pivot shift test on examination
under anesthesia. These investigators reported
that the isometric point of the LUCL on the
lateral epicondyle is proximal to the capitellar
growth plate and therefore does not pose a prob-
Fig. 3. Radiograph of PLRI. The radiocapitellar joint is lem with tunnel placement for reconstruction.
not aligned. The radial head is subluxed posteriorly, and MRI and radiographic assessment, however,
there is incongruence of the ulnohumeral joint. do not support this statement. The lateral
142 LATTANZA & KEESE

epicondyle and distal humerus share a confluent growth arrest is a concern with accurate tunnel
physis near skeletal maturity. The LUCL isometric placement, and lateral epicondylar growth arrest
origin is distal to this common physis, rendering it should not have any clinical consequences in the
vulnerable during tunnel placement (Fig. 4). In re- context of LUCL reconstruction.
ality, only the lateral epicondylar physis and the An 11-year-old boy presented to the clinic
most lateral aspect of the capitellar physis should 1 year after an injury to his dominant elbow
be vulnerable during tunnel placement. It remains with inability to rotate the forearm fully (range of
to be determined whether a 4.5-mm tunnel travers- motion [ROM] 90 pronation and 20 supination
ing an apophyseal physis near skeletal maturity with 0/140 elbow ROM). Radiographs showed
would have any detrimental impact. Most girls an old radial head fracture (Fig. 5). The patient
fuse this common physis by age 13 years, whereas was taken to the operating room for an elbow
most boys are fused by age 15 years [31]. In some arthroscopy and examination under anesthesia.
cases, the distal humeral growth plates close early An osteochondral loose body that had attached
after severe elbow trauma, making this a nonissue; to the radial head and scar tissue around the
however, some patients who have PLRI have open head were excised. Full forearm ROM was
growth plates, especially when the diagnosis is restored arthroscopically; however, when the fore-
made shortly after the trauma has occurred. arm was supinated, the patient demonstrated
The authors recommend ligament repair in PLRI clinically and on flouroscan (Fig. 6). The
cases of avulsion or when suitable soft tissue exists. elbow was opened through a Kocher approach.
Repair may be performed using suture tunnels or The LUCL was found to have a midsubstance
suture anchor. In cases of tissue attenuation, tear with a redundant capsule (Fig. 7). The liga-
reconstruction with palmaris longus or suitable ment was of adequate quality for repair, and the
allograft is advocated. There are some technical capsule was imbricated. At 2-year follow-up, the
differences between pediatric and adult reconstruc- patient has full ROM of the elbow and forearm
tion. The diameter of the ulnar bone tunnels should without evidence of instability. He is participating
be reduced to 2 to 3 mm, and the spanning bone in sports, including baseball.
bridge may have to be slightly narrower depending
on the size of the ulna. Graft size should be
Lateral ulnar collateral ligament attenuation
adjusted accordingly. At the isometric point,
due to cubitus varus
bone tunnel placement in linear direction and
depth must be accurate to avoid multiple passes PLRI of the elbow after cubitus varus defor-
and the need for tunnel enlargement in the physis. mity was reported in the literature in 1995 by Abe
The authors do not believe that distal humerus and colleagues [32]. They described four patients

Fig. 4. AP radiograph and MRI of a skeletally immature elbow. Relationship of growth plate to LUCL origin. Arrows
depict insertion sites of LUCL and placement of bone tunnel for reconstruction.
ELBOW INSTABILITY IN CHILDREN 143

Fig. 7. Midsubstance tear in LUCL. The arrows are on


the two portions of the LUCL. The capitellum is to the
left and radial head to the right.

Fig. 5. Lateral radiograph showing old radial head


relationship between cubitus varus deformity of
fracture with overgrowth.
the elbow and tardy PLRI in 24 patients. Six of
the 24 patients presented in the second decade
who had childhood elbow injuries and developed between 13 and 19 years of age (average age,
PLRI in adulthood. In 1997, these researchers 14 years). In some cases, the development of insta-
reported a case of an adolescent who sustained bility was related to a second injury or surgery. The
a supracondylar humerus fracture at age 5 years investigators reported improvement in all patients
and then developed PLRI at age 14 years. The after LUCL reconstruction with or without osteo-
patient was treated with lateral closing wedge tomy, although 3 continued to have some instabil-
osteotomy and LUCL repair that resolved the ity. No patient lost motion, and motion improved
instability [33]. O’Driscoll and colleagues [34], in in some.
a multicenter study in 2001, reported a ‘‘causal’’ The investigators proposed a viable biome-
chanical explanation for the development of the
tardy PLRI. Varus deformity creates a repetitive
varus torque at the elbow during axial loading
and resisted extension such as when rising from
a chair. This varus torque can lead to chronic at-
tenuation of the LUCL complex over time (Fig. 8)
[34–36]. Confirmation of this theory was accom-
plished intraoperatively in three patients by stim-
ulating the medial triceps while resisting
extension, which caused the elbow to sublux post-
erolaterally. They concluded that the combination
of varus malalignment and medial elongation of
the olecranon permitted the triceps to roll the
ulna into external rotation and to subluxate the el-
bow posterolaterally. After correction of the varus
deformity and transposition of part of the medial
triceps to the lateral olecranon, this same maneu-
ver did not cause subluxation [34].
Adolescents presenting with a childhood frac-
ture resulting in a cubitus varus deformity with new
Fig. 6. Intraoperative fluoroscan showing PLRI with complaints of lateral elbow pain, catching, or
ulnohumeral joint incongruence and posterior subluxa- giving way especially with a superimposed new
tion of the radial head in relation to the capitellum. injury, should be carefully evaluated and
144 LATTANZA & KEESE

Fig. 8. Biomechanics of the triceps mechanism and its effect on ulnohumeral stability. (A) Normal alignment with a slightly
valgus carrying angle. The triceps force vector (FT), which is almost perpendicular to the joint line, can be resolved into two
perpendicular vectors. A slight valgus force (F2val) exists. (B) Cubitus varus. The triceps force vector (FT1) can be resolved
into two force vectors: F1, which is perpendicular to the joint surface, and F2var, which is directed medially. This medial force
vector causes external rotation of the ulna about its long axis (Mroll). The offset between F1 and the axis of rotation (due to
the deformity at the supracondylar level) causes a moment arm (MA) through which external rotation and varus deforming
torques occur with triceps contraction. (From O’Driscoll SW. Tardy posterolateral rotatory instability of the elbow due to
cubitus varus. J Bone Joint Surg Am 2001;83:1364; with permission.)

considered for the diagnosis of tardy PLRI. Again, be seen in other circumstances. Biomechanical
careful lateral palpation and pivot shift, Lach- theories of valgus overload to the radiocapitellar
man’s, chair, and push-up tests should be per- joint during the acceleration phase of throwing
formed [16,17]. Radiographs to measure varus and [38–41] or during weight bearing in gymnastics are
assess ulnohumeral and radiocapitellar alignment commonly proposed mechanisms for the develop-
should be obtained. Examination under anesthesia ment of OCD [42,43].
may be necessary to confirm the diagnosis. In some patients, OCD can lead to late
Current recommendations are for corrective instability of the radial head as the problem
osteotomy to restore the normal carrying angle progresses [44]. Klekamp and colleagues [44]
and LUCL reconstruction. In some isolated cases have also seen progressive subluxation of the
with a varus deformity less than 15 in a low- radial head in OCD of the elbow. The reasons
demand patient, LUCL reconstruction alone may for this are not entirely clear.
be adequate [34]. In a study by Morrey and An [45], it was dem-
onstrated that the osseous articular cartilage
between the radius and the capitellum contributed
Osteochondritis dissecans
33% of the resistance to valgus stress at 90 of
Osteochondritis dissecans (OCD) involves elbow flexion. Schenck and Goodnight [46], in
avascular necrosis of a localized area of subchon- a cadaveric model, showed a mismatch in stiffness
dral bone, with loss of support for the adjacent between the capitellum and radial head. They pro-
cartilage. Only 6% of patients who have OCD posed that the less-stiff capitlellum is at risk from
have elbow involvement [37]. It occurs in early compressive forces at the radiocapitellar joint,
adolescents after the capitellum has ossified. which could account for the fragmentation of
The exact mechanism for development of OCD the capitellum but still does not fully explain the
is not known. It is more commonly seen in instability sometimes seen with the radial head
throwing athletes and gymnasts, although it can subluxing or dislocating in some patients.
ELBOW INSTABILITY IN CHILDREN 145

Although not discussed in the literature pre- excellent clinical and radiographic results in seven
viously, one other possible contributing factor of eight patients and return to sport in six of eight
could be subtle ligamentous instability on the patients, including three pitchers.
lateral side of the elbow. Adult patients who have
PLRI usually have a lesion on the capitellum,
Monteggia fractures
similar in location to OCD, caused by the
impaction of the radial head as it subluxes poster- The Monteggia lesion (ulna fracture with
olaterally. It may be possible that athletes placing radial head dislocation) is uncommon but not
high demands on the elbow develop a very subtle rare. The results of this injury when recognized
instability similar to that seen in the shoulder in and treated acutely are generally good; however,
pitchers. This subtle instability could lead to this the results of treatment in the unrecognized or
lesion in a minority of cases; however, it has not chronic lesion are much less predictable. Sixteen
been proved biomechanically and is only a clinical percent to 33% of Monteggia lesions are missed
observation seen by the authors in a few cases. according to Dorman and Rang [50]. The reasons
At this point, there is no way to determine which for this are varied. Often, trauma radiographs are
patients who have OCD will develop a radial head inadequate or read by someone who has little
dislocation. In the authors’ experience, however, experience interpreting pediatric elbow radio-
the loss of motion is greater in patients who go on graphs. Even when recognized, the lesion may be
to develop a dislocation of the radial head. undertreated because some of the fractures/dislo-
Treatment of OCD lesions varies depending on cations can redisplace in a cast with closed treat-
the severity of the lesion. The Internal Cartilage ment alone. When this lesion is not recognized
Repair Society (ICRS) has suggested the following during the course of treatment, the patient will
arthroscopic classification system for OCD lesions go on to have a chronic radial head dislocation.
[47]: ICRS OCD grade I indicates a stable lesion There are also some Monteggia fractures that
with a continuous but softened area covered by in- are irreducible or ‘‘unstable.’’
tact cartilage; ICRS OCD grade II denotes a lesion There is no controversy regarding the treat-
with partial discontinuity that is stable when ment of acute Monteggia fractures. The ulnar
probed; ICRS OCD grade III denotes a lesion angulation is corrected and held in position,
with a complete discontinuity that is not yet dislo- which usually corrects the radial head dislocation.
cated; and ICRS OCD grade IV indicates an empty In some cases, however, a reduction of the radial
defect or a defect with a dislocated fragment or head cannot be achieved by closed reduction
a loose fragment lying within the bed. secondary to soft tissue interposition. Fig. 9A
Several treatment recommendations have been shows the radiograph of a 9-year-old who had
made by Takahara and colleagues [48] based on an acute Monteggia injury and radial nerve palsy.
a retrospective study of 106 patients who had The radial head was irreducible with closed treat-
OCD. Patients who had an open physis, normal ment. An open reduction was performed. Fig. 9B
elbow ROM, and a flattening of the capitellum shows the radial nerve that was interposed in the
radiographically were considered to have stable joint, blocking the reduction. The radial nerve
lesions. These patients responded well to rest was carefully removed from the joint, and the
without operative intervention. patient regained full function 3 months post oper-
Takahara and colleagues [48] further classified an ation. It is important to be aware of the possibility
unstable lesion as presenting with one of the follow- of nerve entrapment and to not attempt repeated
ing: a mature capitellum with closed growth plates, reductions under those circumstances without
radiographic fragmentation, and loss of motion of opening the joint.
at least 20 . These patients had a better outcome There is no consensus for absolute operative
with operative intervention. For grade II lesions, indications for the chronic Monteggia lesion. There
bone peg fixation was recommended. For grade III is certainly debate with regard to surgical interven-
lesions, fragment fixation and bone grafting was rec- tion in an asymptomatic child with good ROM.
ommended, and for grade IV lesions covering There are implications for decreased function,
greater than 50% of the capitellar surface, osteo- pain, progressive valgus instability, tardy ulnar
chondral mosaicplasty provided the best results. nerve palsy, and dislocation of the distal radioulnar
The use of the mosaicplasty in the elbow is joint when left untreated [51]. Some investigators
a relatively new technique, with a report by [52] state that as long as the radial head has a nor-
Iwasaki and colleagues [49] showing good to mal concave shape, the joint should be reduced.
146 LATTANZA & KEESE

Fig. 9. Radiograph (A) and intraoperative photo (B) of an acute Monteggia injury with soft tissue, including the radial
nerve, blocking the reduction.

One of the main considerations in entertaining stability of the joint after osteotomy. In some cases,
operative intervention is the high complication rate an annular ligament reconstruction is necessary to
associated with surgical correction [53]. The list of augment the reduction but should not be relied on
potential complications includes nerve palsy, to hold the reduction. Overcorrection of the ulnar
nonunion of the ulnar osteotomy, loss of reduc- deformity is necessary to hold the radial head in
tion, compartment syndrome, infection, radioul- a reduced position throughout the arc of motion.
nar synostosis, loss of motion, and malunion of Inoue and Shionoya [54] found that, ultimately,
the ulnar shaft. A thorough discussion with the annular ligament reconstruction and pinning of
parents regarding these potential complications is the joint had no effect on stability. The only signi-
imperative. ficant difference in the result was whether the ulna
The authors’ preferred method for reduction of deformity was overcorrected.
the chronic Monteggia lesion is an ulnar osteotomy
with iliac crest bone graft or allograft. The ulnar
Radial head dislocations
length and the proper angulation must be restored
and held with a plate (Fig. 10). The radiocapitellar The most common cause of pediatric instability
joint is opened to remove any interposed material, at the elbow is radial head subluxation or disloca-
and an assessment is made with regard to the tion. Pediatric radial head instability can be

Fig. 10. Chronic Monteggia fracture/dislocation (A) and correction by ulnar osteotomy (B).
ELBOW INSTABILITY IN CHILDREN 147

classified by the direction, chronicity, and etiology.


The direction of the radial head subluxation/ Box 1. Abnormalities and syndromes
dislocation can be anterior, posterior, or lateral. associated with congenital
Acute injuries are usually directed anteriorly, developmental dislocation
whereas chronic instability is usually posterior. of the radial head
The etiology is classified as congenital, develop-
mental, or post-traumatic; however, the termi- Skeletal abnormalities
nology of congenital versus developmental Radio-ulnar synostosis
is confusing because it is often used interchange- Absence of ulna
ably. Box 1 is a list of most anomalies associates Antecubital pterygium
with dislocation of the radial head. Below-elbow deficiencies
Absence of thumb ray
Absence of ulnar ray
Congenital Ulnar oligodactyly
Radial head dislocation is the most common Metacarpal fusion
congenital anomaly of the elbow joint. It can Side-to-side phalangeal fusion
occur as an isolated anomaly or in association Cleft hand
with multiple syndromes. There has been debate Thumb duplication
in the past as to whether unilateral congenital Sprengel’s shoulder
dislocations exist or are just a missed Monteggia Multiple exostosis
injury. Agnew and Davis [55] reported on 6 cases Multiple enchondromatosis
of congenital radial head dislocations in which an Multiple epiphyseal dysplasia
elbow abnormality was noted by the parents Syndromes
or the physician at birth. Other investigators Acrocephalosyndactyly (Apert
[56–59] have reported similar cases of unilateral syndrome)
radial head dislocations present at birth. Acrocephalopolysyndactyly (Carpenter
The radiographic appearance of a congenital syndrome)
radial head dislocation is one of a convex radial Acro-osteolysis congenital
head (Fig. 11), sometimes associated with a hypo- Auriculo-ostedysplasia
plastic capitellum. Other possible radiographic Bird-headed dwarfism
findings are a more slender or longer radius than Cornelia de Lange syndrome
normal and a lack of anterior angulation of the Chondroectodermal dysplasia
distal humerus. In the case of a posterior disloca- Craniocarpotarsal dystrophy
tion, the posterior bow to the ulna is frequently Craniostenosis
exaggerated. It may be that the only certain crite- Cleidocranial dysostosis
rion for radiographic diagnosis of congenial radial Detenbach-Abrams syndrome
head dislocation is severe hypoplasia of all the Diastrophic dwarfism
bony elements of the elbow in conjunction with Ehlers-Danlos syndrome
a radial head dislocation. Konig disease (Hereditary
Morrey and Bell [60,61] devised a radiographic osteochondritis dessicans)
classification based on the amount of subluxation Larsen syndrome
of the radial head. Radiographs of type I lesions Mid-facial cleft syndrome
show a subluxation of the radiocapitellar joint, Nail-patella syndrome
but a portion of the radial head is still in contact Nievergelt syndrome
with the capitellum. In type II lesions, there is Oculomelic complexes
complete dislocation inferior to the capitellum, Robinow mesomelic dysplasia
but the radial head has not migrated proximal Rubinstein-Taybi syndrome
to a line drawn through the center of the humerus. Silver-Russell syndrome
In type III lesions, the radial head has migrated
proximal to the midline of the humerus. In this se-
ries, the type I lesions had the worst prognosis for dislocated radial head. Similarly, there is agree-
arthritis and pain. ment that radial head excision at skeletal maturity
There is general consensus that there is no is the operation of choice for pain but that
role for operative reduction of the congenitally restoration of motion is unpredictable at best.
148 LATTANZA & KEESE

present versus a unilateral isolated congenital


dislocation so that treatment can be planned
accordingly. Of course, a history of elbow trauma
can be helpful but is often vague. When there is no
other history of skeletal abnormalities or trauma,
careful scrutiny of the radiographs may be help-
ful. If the radial head is convex or the capitellum is
hypoplastic, then it is likely a congenital disloca-
tion. A longstanding, chronic missed Monteggia
injury, however, can lead to a similar radiographic
appearance, causing confusion in making the
diagnosis.
Mizuno [33] recommended arthrography to
help differentiate congenital from traumatic radial
head dislocations. In congenital dislocations,
Fig. 11. Posterior congenital radial head dislocation arthrography shows the radial head to be located
with a convex radial head and exaggerated posterior within the joint capsule, whereas in the traumatic
bowing of the ulna. situation, the radial head is outside the joint
capsule. If one is contemplating surgical interven-
tion for a presumed chronic traumatic radial head
Developmental dislocation, then arthrography may be helpful to
This category refers to any condition, inherited confirm the diagnosis.
or acquired, resulting in differential growth
between the forearm bones, leading to a radial
head dislocation. One of the most common Nontraumatic causes of instability
examples is multiple hereditary exostoses. In the
Collagen disorders
case of developmental radial head dislocation, the
dislocation is not present at birth. The radial head Elbow instability is seen in multiple types of
gradually subluxes with growth. As with congen- collagen disorders. Joint laxity (or hypermobil-
ital dislocations, open reduction is generally not ity) can be classified into one of seven categories
advised. according to Beighton and colleagues [64]. These
A possible subgroup of the developmental categories are as follows: physiologic joint laxity,
dislocation is the ‘‘paralytic’’ dislocation seen in generalized joint laxity, inherited joint laxity
some patients who have brachial plexus birth syndromes, joint laxity in inherited connective
palsy and cerebral palsy. The incidence in brachial tissue disease, joint laxity in skeletal dysplasias
plexus birth palsy has been reported as high as with dwarfism, syndromes with joint laxity as
25% [62]. a minor component, and acquired joint laxity
(Box 2).
Post-traumatic The Wynne-Davies criteria [65] for diagnosing
Isolated radial head dislocations as pure liga- hypermobility requires that at least three of the
mentous injury are rare at best. Lincoln and following five conditions be present: elbow exten-
Mubarak [63] disputed the existence of such an sion beyond straight, ability to touch the thumbs
entity and contested that all radial head disloca- to the forearm passively on wrist flexion, fingers
tions are a variant of a Monteggia lesion. They that lie parallel to the forearm on passive exten-
described a method for scrutiny of the bow of sion of the wrist and metacarpophalangeal joints,
the ulna radiographically and recommended com- passive dorsiflexion of the ankle to 45 or more,
paring these values to the uninvolved side. The and knee extension beyond straight.
authors agree with this assessment. This theory
would also support the fact that in most series Generalized laxity
on missed Monteggia lesions, patients undergoing Multiple investigators [66–68] have quoted
ligament reconstruction alone, without addressing the incidence of generalized joint laxity to be
the ulnar bow, generally do not do well. anywhere from 5% to 12% among school-aged
It is important to determine whether an acute children. Joint laxity decreases throughout child-
or chronic traumatic radial head dislocation is hood. It is thought that joint laxity predisposes
ELBOW INSTABILITY IN CHILDREN 149

Inherited joint laxity


Box 2. Inherited and acquired Recurrent elbow dislocations and laxity can
syndromes associated with joint laxity occur with many of the syndromes (Fig. 12).
Splinting and therapy are the mainstays of treat-
Inherited joint laxity syndromes ment. In most cases, surgical intervention is not
Ehlers-Danlos syndrome warranted and can be fraught with complications.
Familial undifferentiated joint laxity Soft tissue procedures are often unsatisfying
syndromes owing to the underlying abnormal collagen and
Joint laxity in inherited connective propensity for recurrent instability. In general,
tissue disease bony stabilization procedures are usually more
Marfan syndrome successful but may sacrifice mobility for stability.
Marfanoid hypermobility syndrome Larsen’s syndrome, in particular, is commonly
Achard syndrome associated with elbow and radial head disloca-
Osteogenesis imperfecta tions. There is generally total disruption of the
Larsen syndrome radiocapitellar and ulnohumeral joints, at least in
part due to distal humeral and condylar hypopla-
Joint laxity in skeletal dysplasias sia [69]; however, the function is often good.
with dwarfism
Spondyloepimetaphyseal dysplasia Acquired joint laxity
with joint laxity Systemic disease processes such as juvenile
Pseudoachondroplasia rheumatoid arthritis and systemic lupus erythe-
Morquio syndrome matosis can also lead to instability of the elbow
(mucopolysaccharidosis IV) joint over time. This instability is usually a varus/
Desbuquois syndrome valgus instability from a stretching of the soft
Spondyloepipyseal dysplasia congenital tissue envelope from the disease process itself and
from the treatment with steroids. Bracing can be
Syndromes with joint laxity as a minor
an effective treatment when the instability and
component
Hajdu-Cheney syndrome pain are not severe. When pain and instability are
severe, however, total elbow arthroplasty may be
Hyperlysinemia
considered. Connor and Morrey [70], in a series of
Opitz trionocephaly syndrome
(C syndrome) 24 patients who had juvenile rheumatoid arthritis
and who underwent total elbow arthroplasty,
Trichorhinophalangeal syndrome
found that 9% were grossly unstable and 35%
X-linked cutis laxa
Aarskog syndrome were moderately unstable. The youngest patient
to require total elbow arthroplasty was 25 years
Cohen syndrome
old. Total elbow replacement in this group of
Multiple endocrine neoplasia type III
patients should not be entered into lightly. The
syndrome
complication rate is high and the surgery is techni-
Coffin and Siris syndrome
cally demanding, but in the well-chosen patient,
Acquired joint laxity total elbow replacement can lead to pain relief
Chronic renal failure and increased function.
Systemic lupus erythematosis
Cerebral palsy
Juvenile rheumatoid arthritis Summary
Elbow instability in children can be secondary
to trauma, developmental disorders, congenital
anomalies, inherited disorders, or acquired sys-
to injuries such as joint dislocations, including temic processes. Our knowledge and understand-
dislocations of the elbow. Treatment is symptom- ing of these problems are continuing to evolve.
atic and may include splinting, therapy, or surgi- Although the determination of what constitutes
cal reconstruction for ligament tears secondary appropriate treatment of some post-traumatic
to traumatic dislocations. Surgery simply to conditions may still be up for debate, it is the
address the underlying laxity is usually not authors’ opinion and experience that it may be
indicated. better to treat some nonunions, malunions, and
150 LATTANZA & KEESE

Fig. 12. (A) Lateral radiograph of a patient who has familial undifferentiated joint laxity syndrome. The radiograph
demonstrates subluxation of the elbow. (B) MRI scan of the same patient demonstrating subluxation of the elbow,
with a hypoplastic fossa. (C) Additional MRI image of the patient demonstrating radiocapitellar joint incongruency.

chronic instability of the post-traumatic nature and to devise more effective surgical interventions
more aggressively than has been done in the past. for treating the sequelae of injury and instability.
Only in the past 15 years have some instability
patterns of the elbow been recognized in the adult,
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