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A seven-year-old boy presented with right leg pain. Plain film of the hips demonstrated sclerosis, flattening, and fragmentation of the bilateral femoral capital epiphyses as well as shortening and widening of the femoral necks with metaphyseal irregularity including cystic changes. There was involvement of the lateral pillars bilaterally, worse on the right compared to the left (Fig. 84.1). Additionally, slight lateral subluxation of the right hip was demonstrated, consistent with non-containment of the right femoral head within the acetabulum (Fig. 84.1). Findings were considered diagnostic of Legg–Calve–Perthes disease (LCPD).
LCPD is a condition characterized by idiopathic osteonecrosis of the capital femoral epiphysis. It affects approximately 4 to 15.6 per 100 000 children. Almost all are between three and 12 years old, with the peak incidence occurring around 7 years of age. Boys are affected four to five times more often than girls. Those diagnosed at a younger age typically experience a more benign course than those diagnosed at an older age, who often require increased rates of intervention. Both hips are involved in 10–20% of cases, usually successively rather than simultaneously. It is hypothesized that rapid growth of the bone in relation to the developing blood supply of the secondary ossification centers results in an interruption of adequate blood flow, making these areas prone to avascular necrosis.
A 15-year-old boy presented with left-sided chest pain and a palpable chest wall mass. PA radiograph of the chest demonstrated osseous expansion and periosteal reaction in the left mid 8th rib and a large associated soft tissue mass (Fig. 91.1a). Axial contrast-enhanced CT of the chest demonstrated a heterogeneous soft tissue mass along the left chest wall. There was expansion, destruction, and periosteal reaction of the involved rib (Fig. 91.1b, c). An MRI of the abdomen, obtained to evaluate for metastases, was negative and also included the lower chest (Fig. 91.1d, e, f). This demonstrated a large heterogeneous T2 bright mass surrounding the left lower rib. The mass and marrow of the affected rib demonstrated restricted diffusion (bright signal on diffusion-weighted imaging [DWI] with dark signal on ADC map). Bone scan (not shown) did not demonstrate any metastases. Ewing’s sarcoma of the chest wall was suggested as the most likely diagnosis. Subsequent pathology defined the lesion as an undifferentiated sarcoma of the chest wall.
A one-month-old girl had a physical examination suggesting developmental dysplasia of the hip (DDH). A coronal sonographic image through the right hip in neutral position, performed at one month of age, demonstrated an abnormal alpha angle of 47 degrees and less than 50% coverage of the femoral head under the acetabular roof (Fig. 83.1a). Pelvic AP radiograph at three months of age demonstrated a steep right acetabular roof with asymmetrically delayed ossification of the right femoral head (Fig. 83.1b).
A three-year-old girl with a chromosomal deletion syndrome presented with bilateral hip pain and difficulty with abduction. Pelvic AP radiograph demonstrated dysplastic acetabulae with bilateral femoral head dislocation, pseudoacetabula formation, and valgus deformity (Fig. 83.2a). Frog leg view demonstrated that the hips did not relocate with abduction (Fig. 83.2b). This appearance is typical of secondary neurogenic hip dislocation related to muscle imbalance and overactive hip adductors.
AP and lateral radiographs of the right lower extremity in a young infant demonstrated diffuse exuberant periosteal reaction, diaphyseal sclerosis, and metaphyseal irregularity with horizontal metaphyseal lucent lines (Fig. 87.1a,b), suggestive of bony changes of congenital syphilis. AP radiograph of the bilateral lower extremities in a different infant with congenital syphilis demonstrated irregular, focal lucencies of the medial proximal metaphyses of the bilateral tibiae, the Wimberger sign (Fig. 87.2). AP radiographs of bilateral upper extremities in another infant demonstrated metaphyseal lucencies and diaphyseal sclerosis (Fig. 87.3).
Congenital syphilis is transferred through the placenta in the second or third trimester in mothers with untreated or recently treated primary or secondary syphilis. The pathogenesis of this disease is transplacental migration of Treponema pallidum bacteria. Bony changes are thought to result mostly from trophic effects rather than direct osteomyelitis. There is inhibition of osteogenesis and disturbance of active endochondral ossification. Symmetric involvement of the sites of endochondral ossification leads to bony changes at the epiphyseal-metaphyseal junctions, costochondral junctions, and endochondral ossification sites in the sternum and spine. A baby born to a mother with untreated syphilis in the primary or secondary stage has a nearly 100% chance of acquiring the infection. Radiographic changes occur approximately six to eight weeks after initial infection, so that they may not be present at birth but only manifest subsequently. Direct clinical examination, treponemal tests, VDRL (venereal disease research laboratory [test for syphilis]), and rapid plasma reagin are used to confirm the diagnosis. Results are considered conclusive when the infant’s titer is at least four times higher than that of the mother.
A 17-year-old girl presented with progressive asymmetric left eye swelling. CT images through the left orbit demonstrated a mass centered in the left superior orbital rim, extending through the left cribriform plate and into the posterior ethmoidal air cells. The internal matrix of this mass had a ground glass appearance (Fig. 90.1a, b). The orbital apex appeared to be spared (Fig. 90.1). T2-weighted MR images through the orbit demonstrated a homogeneous hypointense signal of the mass (Fig. 90.1c, d). The mass displaced rather than invaded adjacent soft tissue structures. It displaced the frontal lobe superiorly and extended into the conus of the orbit, with mass effect on the superior and medial rectus muscles. The optic nerve did not appear to be encased. There was homogeneous enhancement of the mass on the T1-weighted MR images after intravenous administration of gadolinium (Gd)-DTPA (Fig. 90.1d). Additional CT (Figs. 90.2 and 90.3b) and plain film (Fig. 90.3a) images of two other children with fibrous dysplasia (FD), one of whom has McCune–Albright syndrome (MAS) (Fig. 90.3), demonstrate the classic ground glass bony matrix.
A 16-month-old girl with bilateral foot deformities since birth had radiographs that demonstrated marked hindfoot and forefoot varus abnormalities bilaterally consistent with bilateral clubfeet (Fig. 82.1a). The talocalcaneal angles measured 0 degrees bilaterally on AP radiographs. The angle between the talus and the first metatarsal measured more than 20 degrees. Lateral views of both feet demonstrated decreased talocalcaneal angles bilaterally. In addition, the right foot demonstrated a fixed hindfoot equinus deformity (plantar flexed calcaneus) whereas the left calcaneus was able to be forcibly dorsiflexed (Fig. 82.1b).
Clubfoot (talipes equinovarus) is a congenital deformity consisting of hindfoot equinus, hindfoot varus (inversion), and forefoot varus (adduction) deformities. Clubfoot deformities affect three joints of the foot to varying degrees, including inversion of the subtalar joint, adduction of the talonavicular joint, and plantarflexion of the calcaneus relative to the tibia, resulting in “toe walking.” Clubfoot deformities can involve one foot or both. Bilateral involvement is found in 30–50% of cases. Clubfoot deformities can be classified as either postural or structural. Postural or positional clubfoot is not defined as true clubfoot. Structural clubfoot can be subdivided into either a flexible type, which is correctable without surgery, or a resistant type, which requires surgical release.
A 10-year-old boy presented with pain and soft tissue swelling of the left cheek. A CT scan demonstrated an extensive soft tissue mass centered in the left maxilla with associated cortical erosion of the left alveolar ridge and left maxillary sinus walls. Contralateral involvement of the right alveolar ridge and anterior maxillary sinus wall was also noted. Several teeth appeared to be “floating” within the maxilla. There was absence of adjacent soft tissue fat stranding (Fig. 9.1a–c).
A five-month-old infant presented with bi-parietal scalp swelling. CT of the head showed bi-parietal calvarial lytic lesions with associated soft tissue masses and a characteristic “beveled edge” appearance with greater destruction of the outer table of the calvarium relative to the inner table (Fig. 9.2a, b). On MRI, mixed T1 and T2 intensities and heterogeneous enhancement of the soft tissue mass was seen (Fig. 9.2c–e).
Langerhans cell histiocytosis (LCH) was confirmed pathologically in both cases.
A 13-year-old boy presented with left groin pain. A radiograph of the pelvis was obtained and demonstrated widening and irregularity of the physis of the left proximal femur associated with demineralization of the femoral head. Klein’s line, a line drawn along the tangent of the lateral margin of the femoral neck, did not intersect the left femoral head (Fig. 85.1a). Findings were consistent with slipped capital femoral epiphysis (SCFE). In addition, a “metaphyseal blush,” an area of increased density in the proximal metaphysis was seen, representing bony healing (Fig. 85.1c). The affected left femoral epiphysis appeared smaller compared to the right side due to the posterior slippage (Fig. 85.1c). The Southwick method for evaluating the head-shaft angle may be helpful for preoperative planning (Fig. 85.1b). Figure 85.2 shows a follow-up radiograph in a different 12-year-old boy, demonstrating surgical pinning of a left-sided SCFE.
An MRI of a 10-year-old girl with SCFE shows high T2 signal within the physis and posteromedial slippage of the right femoral capital epiphysis. There is also an associated joint effusion and bone marrow edema (Fig. 85.3).
A 13-year-old male patient had a twisting injury of the right ankle. Radiographs of the right ankle (in temporary cast) demonstrated a fracture of the right medial malleolus with a medially displaced fracture fragment (Fig. 88.1a). The fracture was noted to have metaphyseal, physeal, and epiphyseal components consistent with a Salter–Harris type IV injury. In addition, there was mild separation of the distal tibia and fibula, suggesting an injury of the tibiofibular syndesmosis. The patient was referred to orthopedic surgery for surgical fixation. A postsurgical radiograph of the right ankle demonstrated anatomic alignment of tibia and fibula with stabilizing screws in the medial malleolus and through the distal tibiofibular syndesmosis (Fig. 88.1b).
In 1931, McFarland described a pediatric fracture of the medial malleolus of the distal tibia that extended across the physis and sometimes into the metaphysis. These fractures were therefore previously described as McFarland fractures. The Salter–Harris classification has since become more frequently used to characterize pediatric fractures (Fig. 88.2). Salter–Harris I fractures extend through the physis. Type II fractures pass through the physis and metaphysis. Type III fractures extend through the physis and epiphysis. Type IV fractures pass through the epiphysis, physis, and metaphysis. Salter–Harris type V injury is a compression or crush injury of the physeal plate, associated with growth disturbance at the physis (Fig. 88.2). An avulsion fracture of the medial malleolus of the distal tibia that extends through the physis and epiphysis is therefore characterized as a Salter–Harris III or IV fracture depending upon whether there is extension of the fracture line into the metaphysis. The medial collateral ligament (MCL) of the ankle, also called the deltoid ligament, can be involved. The MCL is a strong ligamentous complex that is an important stabilizer of the ankle. The MCL components include a deep layer which courses from the medial malleolus to the talus and a deltoid-shaped superficial layer that extends from the medial malleolus to the navicular, the spring ligament, and the calcaneus. The importance of this fracture is that it often occurs in children and any disruption or damage to the developing growth plate can result in growth arrest.
A 12-year-old female presented with a history of injury to the left ankle. An AP radiograph of the left ankle demonstrated a fracture in the distal lateral metaphysis of the left tibia with associated widening at the lateral aspect of the physis and probable epiphyseal fracture also, suggesting a Salter–Harris type IV fracture (Fig. 89.1a). The lateral radiograph more clearly demonstrated an extension of the fracture into the epiphysis (Fig. 89.1b), suggesting a complex triplane Salter–Harris type IV fracture with the epiphysis fractured in the sagittal plane, the physis separated in the axial plane, and the metaphysis fractured in the coronal plane. A CT scan better demonstrated the extent of the fracture with an anterolateral bony fragment of the tibial epiphysis and intra-articular extension into the tibiofibular syndesmosis and tibiotalar joint (Fig. 89.1c). Coronal and sagittal CT reformats clearly demonstrated the fractures of the posterolateral metaphysis, anterolateral physis, and central epiphysis (Fig. 89.1d–f) with involvement of the tibial plafond. The intra-articular extension into the tibiotalar joint was characterized by wide separation of the epiphyseal fragments at the joint surface with a displaced anterolateral epiphyseal fragment. The medial malleolus was intact.
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