Variants of Exostosis of the Bone in Children

Randy Ray Richardson, MD

xostosis of the bone is a common finding in children

presenting with a mass in the extremity. The appearance
of a solitary exostosis of the bone can be characteristic and the
diagnosis of an osteochondroma is often made without further imaging. Multiple osteochondromas are seen in multiple
hereditary exostoses. Osteochondromas can be in specific
locations such as the epiphysis in Trevors disease. There are
also several osteochondroma-like lesions that can occur as
normal variants or as the result of trauma, congenital anomalies, systemic disorders, or various causes of periosteal reaction. In this article, we will review the imaging of the common osteochondroma and its associated diseases and then
focus on a variety of causes of bony exostosis that can have a
similar appearance.

Osteochondromas
Solitary osteochondromas are common lesions and may account for up to half of benign bone tumors.1,2 An osteochondroma is an outgrowth of benign cartilage from the growth
plate that undergoes endochondral bone formation. The etiology of osteochondroma is not clear. Many believe an osteochondroma to be a true bone neoplasm. This may be supported by the increased incidence of osteochondromas in
patients who have undergone radiation.3,4 Others have suggested that the outgrowth is a result of injury to the peripheral growth plate as suggested in animal models where the
growth plate has been traumatized and a typical solitary osteochondroma is produced.5-7
Whatever the etiology (neoplastic or traumatic), patients
with solitary osteochondromas typically present with a nontender slow-growing mass. Occasionally, fractures of the pedunculated osteochondroma can occur. Mass effect on adjacent structures such as bone (especially when they occur in
the forearm and leg), nerves, vessels, muscles, or even the
spinal cord can also be symptomatic1,8-10 (Fig. 1).
Osteochondromas occur in any long bones that undergo
endochondral ossification. The lesions usually begin
growing at the growth plate and are most frequently found
Pediatric Radiologist, Phoenix Childrens Hospital, Mesa, AZ.
Address reprint requests to: Randy Ray Richardson, MD, Pediatric Radiologist, Phoenix Childrens Hospital, 553 N. Orange Street, Mesa, Arizona
85201. E-mail: randyjenny@hotmail.com

380

0037-198X/05/$-see front matter 2005 Elsevier Inc. All rights reserved.

doi:10.1053/j.ro.2005.01.020

in the metaphysis or metadiaphysis and tend to grow away

from the physis. The most common location for these
lesions is in the lower extremity. The femur is the most
common location followed by tibia and then humerus1,2
(Figs. 2 and 3).
Radiographically these lesions appear either pedunculated
with a stalk or sessile with broad cortical attachment. There is
characteristic medullary and cortical continuity seen in both
types of lesions. The appearance of the cartilaginous cap is
variable but can have rings of calcifications seen with other
chondroid lesions. Radiographically, mineralization or ossification of the cap can be seen with skeletal maturation (Fig.
4). The thickness of the cap is typically measured by MRI and
can vary widely in thickness based on skeletal maturation.1,11-12
Excision of the osteochondroma is typically definitive, although recurrence has been reported in 2% of excised lesions.13

Hereditary Multiple
Exostoses (HME)
HME is one of the most common skeletal dysplasias in children and, as the name implies, is an inherited disorder transmitted as an autosomal dominant trait. Males are affected
more frequently than females due to incomplete penetrance
in females.14-15 The overall incidence is approximately nine
per million.
Patients with HME are typically identified during the
first decade of life.16 Patients most commonly present with
a palpable mass or deformity from angulation of an extremity. Short stature is seen in 40% of patients, although
most patients still fall within two standard deviations of
the mean.17 The short stature is thought to result from
bowing and angulation deformity as well as the development of exostoses during peak growth periods of childhood and puberty.18
The osteochondromas of HME differ from the patients
with solitary osteochondromas in that 90% of the lesions are
of the sessile type19 (Fig. 5). Lesions in patients with HME
have been described in almost every bone in the body excluding the calvarium. The most common location is about the
knee (distal femur and proximal tibia). In fact, it is so com-

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381

Figure 3 MR imaging of osteochondroma of the distal femur with

mass affect on muscle.
Figure 1 Osteochondroma of proximal tibia with mass affect on the
fibula.

Figure 2 Sessile osteochondroma of the distal femur.

Figure 4 Ossification of cartilaginous cap of a pedunculated osteochondroma of the proximal tibia.

R.R. Richardson

382

mon around the knee that another diagnosis should be considered if the bones about the knee are completely normal.1
Other common locations include humerus, elbow, wrist, hip,
ankle, ribs, hands, feet, and scapula. Vertebral involvement is
rare but can occur and has been described arising from the
posterior elements and from the vertebral body itself. The
distribution of lesions can be bilateral and almost perfectly
symmetric or unilateral and very asymmetric (Fig. 6). The
difference in distribution patterns may be attributed to different genetic types of HME.1,19
Radiographically the evaluation of the patient with HME centers on deformity and possible complications. Deformation is
most commonly manifested as bowing of the bones. Coxa valga
is seen in the proximal femurs, which can lead to uncovering of
the femoral heads.20 The forearm is frequently bowed with ulnar
deviation and shortening of the ulna (Fig. 7). Growth disturbance of the distal radius and ulna can also result in a Madelung deformity of the wrist (Fig. 8). In addition to bowing of
the leg, synostosis of the bones may occur when large osteochondromas grow together. This is typically seen in the distal
tibia and fibula but can occur in other parts of the body20
(Fig. 9). Progressive erosion of an adjacent bone can be seen
especially in the forearm and leg where long bones are juxtaposed11 (Fig. 10).
Figure 5 Sessile osteochondroma of the proximal humerus.

Figure 6 Symmetric bilateral exostoses in a patient with HME.

Variants of exostosis of the bone in children

383
logically, the exostoses are identical to osteochondromas
with a cap of hyaline cartilage and endochondral ossification. The distal femur, distal tibia, and talus are most
frequently involved. The process is typically unilateral;
however, there are several case reports of bilateral disease.
Multiple bones of the same extremity are commonly involved and the lesions occur along the medial epiphysis
more often than the lateral.25-27
Radiographically, small ossifications are seen along the
medial or lateral aspect of a developing epiphysis or tarsal
bone. The small ossifications eventually become confluent
with the epiphysis, forming an asymmetric mass. Deformation and angulation do not commonly occur; however, when
present, they can be severe and debilitating (Figs. 12 and 13).
Because the lesions occur in the epiphysis, arthrography can
be used to define the extent of the lesion. Arthrography is also
useful to distinguish exostosis from loose bodies seen in synovial osteochondromatosis.28

Posttraumatic Exostosis

Figure 7 Shortening of the ulna with bowing of the forearm in a

patient with HME.

Complications seen with both solitary osteochondromas and HME include fracture, vascular injury, bursa formation, neurologic compromise, and malignant transformation. The most worrisome of these complications is
malignant transformation. For solitary osteochondromas,
malignant transformation occurs in less than 1% of cases.1,8 In HME, the frequency is higher. Studies have shown
a prevalence of malignancy ranging from 2 to 25%.1,8 The
most recent studies seem to favor a prevalence of around 3
to 5%.21-24 Radiographic manifestations include increasing
size of an exostosis after puberty, lucencies or indistinctness developing within the exostosis, destruction of cortical bone, and soft-tissue mass. Cross-sectional imaging is
useful in the evaluation of the cartilaginous cap. Generally,
a cartilaginous cap thicker than 1.5 to 2 cm in a skeletally
mature patient should be considered worrisome for possible malignant degeneration. In children, the cartilaginous
cap can be thicker, sometimes normally reaching a thickness of up to 3 to 5 cm21-24 (Fig. 11).

In children and adolescents partial or complete avulsion

fractures commonly occur due to the inherent weakness of
the apophyses compared with the tendons. Avulsion injuries at the musculotendinous junction continue to occur
until patients reach their mid-twenties (Fig. 14). The healing process of such injuries may leave a prominent exostosis of the bone, which should not be mistaken for an
osteochondroma or any other neoplastic or infectious process.28,29 Entities such as OsgoodSchlatter and Sinding
Larsen Johanssen, which have been mistakenly attributed

Trevors Disease
Trevors disease or dysplasia epiphysealis hemimelica is
another process where exostosis of the bone is seen in
children. No inheritance pattern has been established. In
Trevors disease, there is cartilaginous outgrowth typically
involving tarsal bones or epiphyses of long bones. Histo-

Figure 8 Madelung deformity in a patient with HME.

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384

Figure 9 Synostosis of exostoses of the distal fibula and tibia.

to osteonecrosis, now are considered to be part of this

spectrum of partial avulsive injury to the apophysis.30 The
resulting exostosis from a patient with chronic Osgood
Schlatter disease can be dramatic (Fig. 15).

Figure 10 A large osteochondroma of the proximal fibula causing

erosion of the lateral tibial metaphysis.

Myositis ossificans from trauma is a well-established

cause of soft-tissue calcifications. When the injury is deep,
the periosteum of the bone can be involved and ossification can attach to the bone. The result of this process can
be mistaken for an osteochondroma (Fig. 16). Synostosis
can even be seen when myositis ossificans occurs between
bones that are closely apposed. This can look similar to

Figure 11 MR imaging of a thick cartilaginous cap in an adolescent.

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385

Figure 13 Postsurgical appearance of the same patient after resection

of the osteochondroma.

sis, which may often be separated from the rest of the phalanx
by a lucent line32,33 (Fig. 17).
Bizarre parosteal osteochondromatous proliferation
(BPOP) is a rare, benign lesion that was first described by
Nora and coworkers in 1983 in the hands and feet of young

Figure 12 Exostosis of the lateral epiphysis of the proximal tibia with

associated valgus deformity in a patient with Trevors disease.

patients with HME where two large osteochondromas fuse

together.31
Subungual exostosis is an uncommon lesion that can appear very similar to an osteochondroma. The exostosis occurs
at a nail bed of a digit of the hand or foot. The great toe is most
commonly affected. The lesion is thought to be due to trauma
with a reaction similar to myositis ossificans. Histologically
they consist of trabecular bone with a cap of fibrocartilage.
Radiographically they appear as a lobulated bony protuberance emanating from the nail bed.1
Turret exostosis is another type of posttraumatic exostosis
specific to the proximal or middle phalanx of the hand. Radiographically the lesion appears to develop from disruption
of the periosteum with formation of a subperiosteal hematoma. Radiographs demonstrate a broad-based bony exosto-

Figure 14 MR imaging of the avulsive injury in a patient with

OsgoodSchlatter disease.

386

R.R. Richardson

Figure 17 Turrets exostosis of the 1st proximal phalanx.

Figure 15 Chronic OsgoodSchlatter disease.

adults. The lesion is characterized by heterotopic bone rising

from otherwise normal cortex, typically without evidence of
periosteal change or medullary contiguity (Fig. 18). BPOP is
believed to be the result of minor blunt trauma to the affected
area.34

Figure 16 Myositis ossificans attached to the periosteum of the distal

ulna in a patient with healing fractures of the distal radius and ulna.

Normal Variants and

Congenital Anomalies
Mistaken for Osteochondromas
The supracondylar process of the humerus may be easily
mistaken for an osteochondroma. It is simply an outgrowth
of bone that occurs along the anteromedial surface of the
distal humerus and is typically oriented toward the joint,
whereas pedunculated osteochondromas are oriented away
from the joint (Fig. 19). A persistent portion of the coracobrachialis muscle may insert on the process. It can also serve
as an anomalous origin of the pronator teres muscle. Patients
with this anomaly may develop symptoms from compression
of the median nerve by the ligament of Struthers, which
originates from the supracondylar process and inserts to the
medial epicondyle.35 Other similar bony processes occur
throughout the body such as the os intermetatarsale, which
occurs between the first and second metatarsals, and the
trochlear process of the calcaneus, which can be very prominent.
Congenital anomalies can produce osteochondromalike lesions. In patients with diastematomyelia a bone spur
can form within the central canal (Figs. 20 to 22). This can
be differentiated from an osteochondroma by the presence
of a tethered cord with the cord split into two halves at the
level of the bony spur. This is best depicted on MR imaging.
Innumerable normal variations and normal appearances
of bone exist in children that can have an appearance
similar to osteochondromas. The oblique view of the first
metatarsal in young children has a prominent exostosislike process distally, which is normal (Fig. 23). Another
common finding in children is spurring around the growth
plate that occurs with partial or incomplete closure along

Variants of exostosis of the bone in children

387

Figure 18 Bizarre parosteal osteochondromatous proliferation (BPOP). (Reprinted with permission34). (A) Radiograph of humerus after shows no fracture. Soft-tissue
swelling is seen in lateral aspect of upper arm. (B) Six
months later, radiograph of humerus shows development
of myositis ossificans. (C) Twelve months after injury,
axial CT shows peripherally calcified soft-tissue mass (not
shown) consistent with myositis ossificans. Cortically
based lesion is seen without medullary contiguity, consistent with BPOP.

the periphery of the physis. These epiphyseal and metaphyseal spurs should not be mistaken for exostosis of the
bone or fractures36 (Fig. 24). Anomalies of the digits have
a wide variety of appearances with duplications, supernumerary, and bifid digits as well as other various types of
polydactyly36 (Fig. 25).

of the diffuse ossification of ligamentous insertions resulting in metaphyseal exostoses. Fortunately, these systemic
disorders with soft-tissue ossification and osteochondroma-like lesions often have characteristic findings of the
hands and other laboratory data to differentiate them from
HME.37

Systemic Disorders

Periosteal Reaction

Systemic disorders such as pseudohypoparathyroidism,

pseudopseudohypoparathyroidsim, tumoral calcinosis,
and myositis ossificans progressiva can have osteochondroma-like lesions and can be difficult to differentiate
from hereditary multiple exostoses. In particular, myositis
ossificans progressiva, a rare progressive familial disease,
can have an appearance strikingly similar to HME because

There are many causes of periosteal reaction that can cause

bone formation and exostosis that can appear similar to osteochondromas. Potential etiologies include a parosteal osteosarcoma, osteoid osteoma, and osteomyelitis. Parosteal
osteosarcoma with its typical sessile attachment to the external cortex can radiographically mimic an osteochondroma.
Patients with a parosteal osteosarcoma (Fig. 26) are usually

R.R. Richardson

388

Figure 21 Axial CT of a central bony spur in a patient with diastematomyelia.

older and present with a painful mass. Also, in contrast to

osteochondromas, parosteal osteosarcomas arise from the
cortex of the bone and do not commonly involve the medullary cavity.38 Even so, definitive differentiation is usually a

combination of imaging and clinical and histological evaluation.39

In summary, exostosis of the bone is a common finding in
children. Despite the many normal variants, posttraumatic
lesions, systemic disorders, and various causes of periosteal
reaction that can mimic osteochondromas, the diagnosis of a
solitary osteochondroma and HME is often established without advanced imaging because of their characteristic conventional radiographic appearance.

Figure 20 Saggital CT of a central bony spur in a patient with diastematomyelia.

Figure 22 MR imaging of a diastematomyelia.

Figure 19 Supracondylar process of the humerus.

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389

Figure 25 Bifid distal phalanx.

Figure 23 Oblique view of the foot shows a prominent exostosis-like

process distally in the first metatarsal.

Figure 24 Normal variant epiphyseal spurs of the distal radius in an

adolescent.

Figure 26 Parosteal osteosarcoma of the distal femur.

R.R. Richardson

390

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