The Diagnosis and Management of Common Childhood Orthopedic Disorders

The Diagnosis and Management of Common Childhood Orthopedic Disorders Blaise Nemeth, MD, MS

Musculoskeletal illness represents a significant portion of office visits to primary care physicians. Despite this, little emphasis is placed on learning pediatric orthopedics during medical school or residency. Many articles have been written on selected disorders, or approaches to common conditions, such as the limping child. Sometimes determining where to start and how to move toward a diagnosis prevents prompt evaluation, referral, and treatment. Based on the author’s experience as a general pediatrician trained in, and exclusively practicing, nonoperative pediatric orthopedics, approaches to selected complaints related to

the musculoskeletal system are presented. Emphasis is placed on conditions resulting in frequent consultation with pediatric orthopedists from pediatricians and other primary care practitioners, either in the office or over the phone. Important features that may not be familiar to the primary care physician are highlighted, including the identification of aspects that require further evaluation and indications for consultation. Guidance regarding inoffice management is also provided.

ssues related to the musculoskeletal system account for a significant portion of primary care practice. Almost one fifth of all visits to emergency departments and primary care physicians relate to the musculoskeletal system,1 and musculoskeletal pain alone accounts for 6% of visits to general pediatricians’ offices.2 Unfortunately, knowledge and comfort regarding musculoskeletal care historically ranks the lowest of all specialties/systems by pediatricians.3 Excellent resources exist to learn more about specific pediatric orthopedic disorders on-line and through textbooks, but often the difficulty is in getting to the diagnosis. This article aims to provide the general pediatrician with a reference from the perspective of the presenting complaint, providing common differential diagnoses, key findings on history and physical examination, suggestions for initial evaluation and recommendations regarding referral to pediatric orthopedists. The reader is directed to seek additional details through this article’s references,

recommended readings, and/or textbooks in pediatrics or pediatric orthopedics.

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From the American Family Children’s Hospital, Department of Orthopedics and Rehabilitation, University of Wisconsin School of Medicine and Public Health, Madison, WI. Curr Probl Pediatr Adolesc Health Care 2011;41:2-28 1538-5442/$ - see front matter © 2011 Mosby, Inc. All rights reserved. doi:10.1016/j.cppeds.2010.10.004

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Historical Points As tempting as it is to order an x-ray when a patient presents with a musculoskeletal complaint, especially following an episode of trauma, patient evaluation must start with a good history. Following trauma, the mechanism of injury enables the clinician to determine consistency of the history with physical examination and radiographic findings. Close attention should be paid to inconsistencies that may require consideration of alternative diagnoses, such as malignancy, infection, or inflammatory disorders or nonaccidental trauma. For patients without a history of trauma, typical features of onset, progression, exacerbating/ relieving factors, and associated symptoms assist in creating a differential diagnosis. a. Onset—an injurious event immediately before development of pain is consistent with a true traumatic mechanism. Pain that develops a day or more following an apparent trauma, or the retrospective association of an “injury,” should raise concerns for another etiology. Arthritis, infection, or malignancy commonly present with pain and, in active children,

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coincidental injury frequently occurs resulting in errant association and delayed diagnosis. b. Progression and exacerbating features—Worsening pain, pain outside of activity or that interferes with activity, and pain that awakens a child at night raise concerns regarding more significant pathology than a simple sprain or bruise. c. Relieving factors—Pain at night relieved by nonsteroidal anti-inflammatory drugs (NSAIDs) is classic for osteoid osteoma, although other inflammatory disorders may respond in a similar manner (eg, fracture, arthritis, and malignancy); lack of pain relief is even more concerning. d. Associated symptoms—Discovering the presence of unexplained fevers, pain in other joints, swelling, weight loss, or rashes should generate concern regarding malignancy, infection, or systemic inflammatory disorders.

and outside of school may seem trivial but are important considerations in returning an injured athlete back to play or in understanding the impact of illness on a child’s life.

Physical Examination The instruction in musculoskeletal physical examination techniques is beyond the scope of this article. Readers are encouraged to refer to a textbook on orthopedic examinations or learn skills through hands-on experience at continuing medical education conferences or by spending time with an experienced clinician. However, specific features of the physical examination helpful in the evaluation of certain musculoskeletal complaints are presented as appropriate.

Radiographic Imaging

Parents often raise concerns about Radiographs gait and alignment issues in their The risk of radiation children. While many are normal deImaging of the musculoskeletal exposure and the benefit velopmental variants, pathologic dissystem should begin with plain raof the information gained orders may present in a similar fashdiographs. While best used to idenfrom radiographs should tify bony pathology, variations in ion. Prenatal and birth histories may suggest risk factors for the developbe considered in selecting soft tissue shadows may be seen. ment of cerebral palsy. DevelopWhile radiographs for traumatic the most useful imaging mental history assists in differeninjuries seem obvious, they are tiating isolated gross motor delays tests (see companion piece equally helpful for pain without on radiographic that may arise from peripheral neutrauma, identifying pathologic causes, rologic disorders, muscular dystroevaluation). such as benign and malignant bone phy, cerebral palsy, or hypotonia, tumors or osteochondritic lesions. from more global developmental At least 2 views obtained at 90 delays arising from central neurodegrees to one another should be logic or genetic disorders. Most normal variants graduobtained, although a third view is frequently indicated. ally improve, whereas progressive worsening of sympParents and practitioners alike harbor concerns retoms should raise concern for pathologic disease garding exposure to radiation from radiographs. The processes. primary care physician’s role in minimizing radiation Finally, past medical, family, and social histories exposure involves ordering radiographic evaluation remain equally important whether the presentation is only when clinically indicated, taking into considerdue to trauma or not. Frequent past injuries raise ation radiation exposure, the views that best image the concern regarding nonaccidental trauma or bone fraarea of concern, and whether another modality better gility syndromes; family history of frequent fractures addresses the clinical question. The risk of radiation suggests the latter. Autoimmune and inflammatory exposure and the benefit of the information gained disorders often run in families. Specific infectious from radiographs should be considered in selecting the etiologies, such as tuberculosis, Lyme disease, and most useful imaging tests (see companion piece on rickettsial diseases, should be suspected based on radiographic evaluation). residence in, or travel to, endemic locales. Many As a reference for comparison, total annual backorthopedic disorders have a genetic association, such ground radiation exposure is 3 mSv, while a transcontinental flight generates 0.03 mSv of additional radiaas hip dysplasia, clubfeet, and scoliosis. Activities in

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tion exposure. Radiographs of the limbs result in exposure to an additional 0.015 mSv, while higher radiations doses result from x-rays of the spine (1.5 mSv). By comparison, radiographs of the abdomen result in 6-8 mSv, computed tomography (CT) scans 2-10 mSv, and chest x-rays 0.1 mSv.4 Findings of certain bone lesions, genetic syndromes, or suspicion of nonaccidental trauma all warrant complete skeletal evaluation with a skeletal survey. A full skeletal survey consists of 20 images: anteroposterior (AP) view of each humerus, AP view of each forearm, posteroanterior (PA) view of each hand, AP view of each femur, AP view of each lower leg, PA view of each foot, AP and lateral views of the trunk (including ribs, thoracic, and upper lumbar spine), AP pelvis (including lumbar spine), lateral view of the lumbosacral spine, AP and lateral views of the cervical spine, and frontal and lateral views of the skull.5 Images may reveal additional lesions, characteristic findings of specific bone or genetic disorders or fractures in various stages of healing indicative of nonaccidental trauma.

Advanced Imaging It is tempting to jump straight to magnetic resonance imaging (MRI) when radiographs do not reveal any abnormalities since both soft tissue and occult bony injuries may be identified. However, given a normal radiograph, further imaging may not be necessary if a trial of a therapy (casting, physical therapy) seems reasonable and imaging would not change the course of therapy. Based on the clinical concern or differential diagnoses that need to be differentiated, specific imaging modalities may be indicated. CT scans image bone in exceptional 3-dimensional detail, resulting in their use in determining whether surgery is necessary to restore alignment in fractures. Due to high radiation exposure, CTs should be used judiciously in children; many institutions have developed protocols using a limited number of cuts when a specific answer is sought to a specific question (such as placement of hardware following surgery). Bone scans are useful to identify bone lesions in patients where localization of a single source of pain is difficult or to identify other areas of injury or bone lesions in patients with suspected nonaccidental trauma or certain types of bone lesions. Since bone scans are not specific, further imaging may be necessary to differentiate potential etiologies. To evaluate for spondylolysis, use of single-photon emission computed tomography better images the vertebrae.

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Ultrasound plays an important role in pediatrics, allowing for imaging of structures before ossification, such as the hips. Additionally, the ability to image while moving the area of interest provides dynamic information regarding injury or instability. Ultrasound may also provide sufficient information when imaging soft tissue injuries to preclude the need for other more expensive or invasive advanced imaging. Answering a specific clinical question that will change management should guide the ordering of MRIs; “evaluate for injury” provides little information to guide the radiologist in developing an imaging protocol and/or reading the films. Based on the question of interest or injury of concern, MRI may require augmentation by intravenous or intra-articular contrast.

Newborn Disorders Developmental Dysplasia of the Hip Developmental dysplasia of the hip represents a spectrum of congenital disorders ranging from dislocated hips to hips that are stable but have abnormal anatomy radiographically. The newborn hip examination plays a crucial role in identification of a dislocated hip, serving as the primary “screening” test.6 The Ortolani and Barlow maneuvers should be familiar to all primary care physicians and should be performed at all well-child checks until the child demonstrates a normal gait. The child should be examined on a firm surface, such as on an examination table and not on the parent’s lap, to minimize rocking of the pelvis. Examine each hip individually, rather than “butterflying” the baby’s legs. Minimal force usually results in the characteristic “clunk” of dislocation/relocation (Fig 1). The astute and experienced examiner may be able to detect subluxation, instability without dislocation, above and beyond normal joint laxity at birth. The muscle tension in a crying or fussy child often stabilizes an unstable hip, rendering a falsely negative examination, so having the baby suck on a finger or drink from a bottle during the examination may help calm him; alternatively, examine the baby after she has fed or at a follow-up appointment. Often “clicks,” representing soft tissue elements, such as tendons or ligaments, are identified that have little, if any, association with hip dysplasia.7,8 Identification of an unstable hip, with either dislocation or subluxation, necessitates referral to an orthopedist for management. Radiographs are not necessary and should be left to the discretion of the consultant.

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Fig 2. Late presentation of bilateral hip dislocations in an 8 month old. Hilgenreiner’s line (drawn horizontally through the triradiate cartilage bilaterally) and Perkins’ lines (drawn perpendicularly to Hilgenreiner’s line at the lateral edge of the acetabulum) create 4 quadrants. In the absence of ossification of the femoral head, the medial edge of the proximal femoral metaphysis, should be positioned in the inferomedial quadrant—lateral and/or superior positioning, as in this patient, suggests dislocation.

Fig 1. (A) Barlow maneuver—with the hips and knees bent at 90 degrees, the baby’s left leg is adducted slightly while light posteriorly directed pressure is applied. (B) Ortolani maneuver—the left leg is abducted while the ring finger of the examiner’s hand lifts anteriorly over the greater trochanter of the femur. Note—for both maneuvers, the examiner’s left hand secures the right thigh and pelvis to prevent movement in the contralateral hip. (Color version of figure is available online.)

Dysplasia may exist without clinically evident instability of the hip. The American Academy of Pediatrics has published guidelines regarding radiographic screening for children based on 3 primary risk factors—female sex, breech presentation, and a family history of hip dysplasia.9 Dynamic ultrasound evaluation should be performed between 4 and 6 weeks of age to minimize false positives due to inherent laxity; alternatively, x-rays may be used at 4-6 months of life. There may be benefit to performing radiographs at 6 months of age even after normal ultrasound in those with the risk factor of breech presentation.10 The association of foot deformities (calcaneovalgus and metatarsus adductus) and torticollis remains unclear, but clubfoot is not associated with hip dysplasia.11-13 While the risk: benefit ratio of screening has been questioned,14 pedi-

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atric orthopedists favor the current recommendations.15 Abnormal results should prompt referral to a pediatric orthopedist to determine whether treatment, or continued observation, is most appropriate. Rarely, a stable, but dysplastic, hip may progress to dislocation.16 Children typically present with either a waddling gait or an apparent leg length discrepancy and short-legged gait. On physical examination, hip abduction of less than 60 degrees should raise concern regarding the presence of a late dislocation, demonstrated on radiographic evaluation (Fig 2). Since surgical treatment is required, referral to a pediatric orthopedist should occur promptly, but need not be emergent.

Foot Disorders “Turned In.” Children who present with a foot turned in at birth usually have 1 of 3 entities— metatarsus adductus, metatarsus varus (skewfoot), or clubfoot. Metatarsus adducts is 3 times more common than either clubfoot or skewfoot17; clubfoot and skewfoot occur at a rate of 1 per 1000 live births.18 Differentiating among the 3 involves examination findings of the hind foot. Metatarsus adducts involves a medial deviation of the forefoot, and the hind foot is in neutral to varus,

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Fig 3. (A) Weight-bearing may increase medial deviation of the great toe (arrow) as children learn how to walk [note curved lateral border of the foot characteristic of metatarsus adductus (arrowhead)]. (B) Inability to completely correct metatarsus adductus (lines along the medial border of the foot) when abducting the forefoot (line arrow) against counterpressure at the apex of the deformity [the cuboid (arrowhead)] differentiates a foot that may benefit from corrective casting from a flexible foot that will spontaneously resolve. (Color version of figure is available online.)

meaning the heel is aligned with the leg, or turns in slightly (Fig 3). In skewfoot, the hindfoot is in valgus, turned out relative to the leg and forefoot. Flexibility of the forefoot usually determines prognosis in metatarsus adductus—a foot that can be manipulated by the examiner and stretched to become straight will likely correct on its own, but more rigid feet benefit from corrective casting. All cases of skewfoot should be referred to an orthopedist for possible casting. Whether the skewfoot is rigid or flexible, casting may not result in full correction.19 Clubfoot, also called talipes equinovarus, contains not only metatarsus adductus, but also cavus (a high arch), hind foot varus (heel turned in), and equinus (foot pointing down) (Fig 4). All the components are usually stiff and incompletely correctable. Talipes equinovarus differs from metatarsus adductus and metatarsus varus in the equinus positioning. The calcaneus sits high in the heel, the heel feels “empty,” and the foot cannot be dorsiflexed past a 90-degree alignment relative to the leg. While most clubfeet are idiopathic, a thorough examination should occur to assess for neurologic abnormalities, other joint contractures, or other clinical findings to suggest a specific

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etiology, such as a syndromic or neuromuscular disorder. Referral for Ponseti casting should occur within the first 2 months after birth for best correction of all types of clubfeet.20 Casting successfully corrects most, if not all, of the deformity in children, even after 2 years old.21 Ponseti casting was developed by Dr Ignacio Ponseti over 60 years ago and is the standard of care for clubfoot. With recent modifications in the technique, nearly all feet are correctable, and surgical correction is a rarity. Many children still require minor “tune-up” procedures for persistent components or recurrences, but the ability to restrict procedures to those involving the tendons and avoiding the joint spaces helps minimize later development of arthritis.22 “Turned Up and Out.” Feet that turn up and out at birth usually represent either calcaneovalgus foot or congenital vertical talus (CVT). Calcaneovalgus foot is present in 1-2:1000 children at birth, while CVT is rare at a birth incidence of 1:10,000.12,18,23 Calcaneovalgus foot is mostly correctable into plantarflexion and will fully correct within a few months after birth on its own (Fig 5). CVT has a “rocker-bottom” deformity to the foot, and, like clubfoot, the heel feels empty (Fig 6). Calcaneovalgus feet require no treat-

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Fig 4. (A) Dorsolateral view of talipes equinovarus demonstrating equinus (toe-down position). (B) Plantar view demonstrates hind foot varus, cavus, and metatarsus adductus. (Color version of figure is available online.)

ment, whereas CVT may be corrected with casting and a minor surgical procedure through a recently developed technique, although complete reconstruction may occasionally be necessary.24 Calcaneovalgus foot may be associated with a higher risk of developmental hip dysplasia, while CVT is not.12

Newborn Fractures Infants often sustain fractures during birth, usually associated with shoulder dystocia, although forceful uterine contractions or extraction may also cause fractures. The humerus, clavicle, and femur are most commonly involved. Treatment of fractures around the shoulder involves either a sling and swath or pinning the sleeve of a long-sleeved shirt to the torso of the shirt. Femur fractures at birth are treated with a Pavlik harness set with the hips flexed 90 degrees without

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Fig 5. (A) Resting position of calcaneovalgus foot at birth. (B) Complete resolution, without any treatment, by 2 months [same patient as (A)]. (Color version of figure is available online.)

restriction on adduction.25 In both cases, the treatment is a reminder to caregivers to exercise caution and provide motion limitation until the fracture starts to heal and stabilize itself within 1-2 weeks. Treatment may be discontinued after 3-4 weeks, once a palpable callus exists. Angulation is seldom a concern because the fracture will remodel itself over the first year of life. Fractures off the end of the bones should be followed for potential growth arrest because they represent epiphyseal avulsions that may affect the growth plate.26 Fractures of other bones, or presenting after a child goes home from the hospital, require further evaluation for underlying bone dysplasia, metabolic bone disease (including renal disorders), and nonaccidental trauma.

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Fig 7. Typical appearance of congenital muscular torticollis due to contracture of the right sternocleidomastoid. (Color version of figure is available online.)

Fig 6. (A) Lateral radiograph of a foot with congenital vertical talus in forced plantarflexion. The axis of the talus (solid line) remains vertical relative to the axis of the first metatarsal (dashed line). (B) Complete correction of the foot shown in (A) using casting and a minimal surgical procedure. The axis of the talus now coincides almost perfectly with the axis of the first metatarsal (line) as would be seen in a normal foot.

Tilted Head Children are often born with a head turned and/or tilted to 1 side. Usually this is due to torticollis, contraction of the sternocleidomastoid (SCM) muscle. The head should be tilted toward the side of the involved muscle, with the chin turned to the opposite side (Fig 7). Often a fibrotic mass is palpable within the muscle body, the pseudotumor. Rather than trying to manipulate the head manually to test range of motion, rattling a set of keys to either side of the child often prompts turning of the head, allowing for identification of deficits. Since there may be an association

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Fig 8. Lateral cervical spine radiograph demonstrating congenital cervical fusions evidenced by 4 spinous processes (arrowheads), rather than the normal 7, in a patient with Klippel–Feil syndrome.

between torticollis and developmental dysplasia of the hip, the hips should be examined, and screening imaging may be warranted.11 Most cases resolve spontaneously or with physical therapy. Cases that have persistent limitations in motion of ⬎15 degrees Curr Probl Pediatr Adolesc Health Care, January 2011

Fig 9. Radiographic findings in patients presenting with acute onset of torticollis due to different primary etiologies. (A) Increased retropharyngeal soft tissue thickness (line) in a patient with Grisel syndrome due to retropharyngeal cellulitis. (B) Flattening of the vertebral body (arrowhead), also known as vertebra plana, compared with the normal adjacent vertebral bodies (arrows) in a patient with histiocytosis. (C) Atlanto-axial rotary subluxation identified on a CT scan axial image [note rotation (arrow) of C1 on the dens (arrowhead) of C2].

beyond 1 year old despite physical therapy should be referred for further evaluation and surgical treatment.27 If the head tilts and turns in the same direction, or physical therapy fails to correct the torticollis, cervical spine anomalies or visual problems should be investigated as a possible cause.28 Congenital torticollis due to sternocleidomastoid muscle (SCM) tightness has head tilt with rotation to the opposite side; structural abnormalities should be suspected in ipsilateral rotation. Klippel–Feil syndrome, which involves cervical spine fusions, also presents with a short neck and low hairline, and range of motion may be limited (Fig 8). Sprengel deformity, a small and incompletely descended scapula, may also be present. Plain radiographs of the cervical spine are often diagnostic but may be falsely negative at young ages, before the vertebral bodies have ossified enough to demonstrate the fusions. MRI may be helpful in identifying cartilaginous fusions as well as spinal cord abnormalities because many patients also have underlying stenosis or spinal cord syrinx. Since the heart and kidneys develop at the same time embryologically, both systems should be evaluated for abnormalities as well. Patients should be restricted from contact sports to prevent catastrophic spinal cord injury.29 Acute presentation of torticollis in the older child requires a thorough evaluation for an underlying

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cause. Physical examination should be conducted for possible retropharyngeal or cervical abscesses, lymphadenitis, or cellulitis as a cause of Grisel syndrome, along with a thorough neurologic and muscular evaluation to ensure there is no evidence of compromise from rotary subluxation or vertebral tumor. Radiographs may identify bony or soft tissue abnormalities suggestive of a specific etiology (Fig 9). If no other underlying cause is found, rotary subluxation should be suspected. CT scan may be necessary for diagnosis, and cases refractory to conservative treatment require surgical reduction and fixation.

Flatfoot Children almost universally are “flat-footed” when they start walking.30 Intrinsic laxity and a lack of neuromuscular control result in flattening of the foot when weightbearing. Arch development occurs through early childhood, and by age 8, the prevalence of flatfootedness equals that of the adult population.31 As a result, the diagnosis of “flat-foot” in children before school age is premature. Use of orthotics in children before development of true deformity is premature and does not hasten arch development.32 In fact, foot wear may deter normal development of an arch.33 As a result, shoes should be

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considered protection for the feet when outdoors and they should be flexible and well-fitting. Indoors, children should be encouraged to be barefoot, in stocking feet or minimal slippers.34 Flatfeet are normal in children. Shoes do not induce arch development and should be viewed only as protection for the feet in children under 5 years old. Most flatfeet are “flexible” and nonpainful. Flexible flatfeet appear flat during weight-bearing, but the hind foot assumes a varus position and an arch develops when the child rises onto her toes (Fig 10). When pain does develop, a tight Achilles tendon is almost always present. Dorsiflexion at the ankle, while holding the forefoot in slight supination and the knee extended, tests the tightness of the Achilles tendon. Improved dorsiflexion when testing with the knee flexed suggests tightness amenable to stretching with physical therapy (Fig 11). Children with hypermobility, including Marfan and Ehler-Danlos syndromes, inherently have flatfeet. Foot pain frequently develops secondary to progressive deformity during weight-bearing, often in the absence of a tight Achilles tendon.35 When the heel does not move into varus with rising onto the toes, and inversion and eversion of the foot is limited, the foot is termed a rigid flatfoot. Radiographs of the foot should be obtained to identify tarsal coalition, a connection between 2 bones of the hindfoot that occurs most commonly between the calcaneus and either the talus or the navicular (Fig 12). Tarsal coalitions frequently remain asymptomatic and go undetected but should be suspected in patients with recurrent ankle sprains.36 Once detected, the contralateral foot should also be imaged because about 50% of patients have bilateral involvement. Painful rigid flatfeet without an identifiable cause occur almost exclusively in overweight children.37 Orthotics for children with flatfeet should be reserved for those with pain or progressive deformity. When prescribing orthotics, emphasis should be on control of the heel, and not “supporting” the arch. Occasionally, off-the-shelf orthotics suffice, but often custom orthotics are necessary. Varying the construction of the orthotic imparts different characteristics (Fig 13). Semirigid orthotics, constructed from foam and/or cork, provide some control but afford flexibility. Rigid orthotics, made of plastic, work best for hypermobility disorders or progressive deformity; styles that rise higher around the ankle (such as a supramalleolar orthosis or ankle-foot orthosis) and

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Fig 10. (A) A patient with the appearance of flatfootedness, more so on the left than on the right. (B) When rising onto the toes, the right heel moves into the normal varus position, while the left remains in mild valgus. The patient was found to have left-sided tarsal coalition on radiographs. (Color version of figure is available online.)

extend down the lateral border of the foot provide optimal control. Foot pain and deformity refractory to conservative treatment with physical therapy and orthotics require surgery. Of course, surgery is never indicated for toddlers with normal “flatfeet.” By contrast, treatment of tarsal coalition with conservative measures usually fails, and surgical resection of the coalition is often necessary.

High Arches Cavus feet may be within the spectrum of normal variation of arch structure, but those on the more severe end—presentation at birth or in toddlerhood, failure to compress with weight-bearing, progressive deformity, or concomitant clawing of the toes—should be critically evaluated for the presence of neuromuscular disease. Spinal dysraphism, such as syrinx or tethered cord, represents 1 potential cause.38 Heredi-

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Fig 11. (A) Allowing the heel to fall into valgus is a compensatory maneuver to generate some dorsiflexion when the Achilles is tight. (B) True dorsiflexion of the ankle, in the same patient as (A), is tested with the forefoot slightly supinated, which helps keep the hind foot in a neutral position [note the ability to see the dorsum of the foot, which is not visible in (A)]. (C) Increased dorsiflexion with the knee bent. (Color version of figure is available online.)

tary motor sensory neuropathies, the most common of which is Charcot–Marie–Tooth, represent another and may also demonstrate flattening of the thenar and hypothenar muscles, evidence of wasting of the intrinsic muscles of the hands. Consultation with a neurologist, MRI of the spine, and possibly nerve conduction studies encompass the usual evaluation. Surgery to restore more normal biomechanics is best performed before the cavus foot becomes rigid.39 For patients who refuse surgical correction, orthotics may help ameliorate symptoms until the patient elects

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Fig 12. (A) Talocalcaneal coalition may be difficult to identify on plain radiographs (arrow). (B) CT scan confirms the diagnosis, as well as bilateral involvement (arrowheads).

surgical correction. Semirigid orthotics cushion the foot, but as further deformity occurs, and children develop stress fractures, rigid orthotics may be necessary to more evenly distribute pressures across the entire sole of the foot.

Foot Pain Without Trauma Common causes of foot pain include flatfeet with a tight tendo-Achilles, rigid flatfoot, hypermobility, Kohler disease, Freiberg infraction, accessory navicular, apophysitis, arthritis, malignancy, and infection. Radiographs are usually indicated to assess for bone lesions, and laboratory testing should occur if there is concern regarding malignancy, arthritis, or infection.

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Identification of flexible and rigid flatfeet is distal children. Decreasing traction of the Achilles tendon cussed under the section on Flatfeet. Other causes on the apophysis using a heel pad usually relieves should be considered before attributing pain to flatfeet symptoms, although occasionally stretching of the Achil(since most flatfeet are not painful). In general, treatles tendon is necessary, until spontaneous resolution ment includes stretching of the Achilles tendon and during puberty. Differentiating fifth metatarsal apophyuse of orthotics to control hindfoot motion. For chilsitis from a base of the fifth metatarsal fracture in the dren with hypermobilty, generalized physical therapy setting of trauma is a common dilemma (Fig 17), so 40 to improve conditioning proves helpful, and orthotimmobilization until pain resolves is reasonable until a ics to control foot motion may be necessary. fracture can be excluded. Otherwise, symptomatic treatKohler disease and Freiberg infraction represent 2 of ment with NSAIDs or activity modification is usually the juvenile osteochondroses. Kohler disease usually effective. presents in the preschool-aged child. Radiographs identify compression and/or fragmentation of the naCrooked Legs vicular (Fig 14). Treatment is symptomatic, using rest and/or NSAIDs as necessary, and reossification occurs Children predictably progress through changes in spontaneously. In older children, Freiberg infraction their alignment that often raise concerns, but must be may develop. It is characterized by flattening of the differentiated from pathologic conditions. At birth, metatarsal head, classically the second (Fig 15). Treatmost children appear bowlegged ing pain with NSAIDs and meta(genu varum), straightening out by tarsal pads provides pain control 18-24 months of age. They then while awaiting spontaneous resofall into genu valgum (knocklution. Rarely, surgical interven- Congenital torticollis due to kneed), which is maximal around tion is necessary for those with SCM tightness has head tilt age 3, ultimately straightening out either condition who experience by age 5-6 (although around 5 with rotation to the refractory pain.41 degrees of valgus remain radioopposite side; structural Accessory navicular represents a graphically)42 (Fig 18). Crooked abnormalities should be prominence of the medial portion legs after age 6 are abnormal and of the navicular, and often an ossuspected in ipsilateral an orthopedic referral is warsicle is identified radiographically ranted. Most children are born rotation. (Fig 16). Since an accessory navicwith internal tibial torsion43 (Fig ular may occur without pain and 19). Children are also born with present due to the prominence excessive femoral anteversion, alalone, other causes of pain should be considered though this is masked by external rotation contractures before attributing causality to the accessory navicular. about the hips due to in utero positioning44 (Fig 20). While acute avulsion fracture causes pain, it is more Both internal tibial torsion and excessive femoral common for pain from an accessory navicular to occur anteversion typically improve during the first decade as the result of direct irritation of the prominence or of life.43 The combination of sagittal plane deformities traction from the posterior tibialis tendon on the (varus and valgus), rotational deformities (torsion and synchondrosis with the navicular, often in the setting anteversion), and neuromuscular development creates of flatfoot with tight tendo-Achilles. Physical therapy a complicated evaluation. helps stretch the Achilles and decreases tension on the Observation of a child’s gait helps identify the posterior tibialis, but orthotics, with a padded area of source of alignment issues, localization of pain or relief at the prominence, are often necessary to control abnormal biomechanics, and even uncover other hindfoot motion and/or decrease direct irritation. Perunsuspected findings. Watching a child walk in the sistent pain may respond best to excision of the examination room is insufficient. Children should accessory fragment. be observed walking in a hallway long enough to Apophysitis often occurs at the insertion of the allow them to obtain a natural rhythm. Talking to Achilles tendon on the calcaneus or the peroneus the child helps him adopt his natural gait pattern and brevis on the fifth metatarsal. Calcaneal apophysitis, confirm with the parents that what is seen in clinic also called Sever disease, typically occurs in prepuber-

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Fig 13. (A) Noncustom foot orthosis, available over the counter. (B) Custom semirigid foot orthosis, made by creating a mold of the patient’s foot and using layers of different density materials. (C) Custom-made, rigid University Of California, Berkeley Labs orthosis. (D) Supramalleolar orthosis custom-fit based on either a mold or measurements of the patient’s foot. (Color version of figure is available online.)

Fig 14. (A) AP foot radiograph of a normal foot. Note the navicular (arrow) which is smooth and the same density as the other bones of the foot. (B) Contralateral foot of the same patient demonstrating flattening and sclerosis (brighter white appearance of increased density) of the navicular, as seen in Kohler disease. (C) Radiograph of the same patient 12 months later demonstrating healing fragmentation of the navicular (arrow).

is what they are seeing at home. For the reluctant toddler, having the parent walk to a point down the hallway may prompt the child to walk, or run, to the parent.

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As the child walks away, observe whether the feet point in or out. Note whether the heel remains on the ground throughout weight-bearing (stance), comes off the ground early, or is always off the ground. As the

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child slows down and turns around, notice if they rise onto their toes. Assess whether the arch flattens in full weight-bearing, compresses some/mostly, or not at all. As she walks back, look at the patellae and note whether they point outward, straight ahead, or inward. Also, look at how much knee flexion and hip flexion occur as the child lifts her leg and moves it forward (swing). Toddlers flex little at both joints, but a more normal gait is observed by age 3, with full maturation by age 5-8 years old. Watch closely for any asymmetry, including the posture and swing of the upper extremities. Finally, having the child run may accentuate abnormalities and/or make underlying spasticity more obvious.

may be confirmed radiographically, although early infantile Blount syndrome must be considered. Unlike physiologic varus, infantile Blount syndrome (tibia vara) involves isolated tibial deformity and progressively worsens. Tibial metadiaphyseal angles that are greater than 16 degrees, especially if they are greater than those of the femur suggest infantile Blount syndrome (Fig 23). Increasing deformity of the medial proximal tibial metaphysis is seen radiographically as Blount syndrome progresses. Clinically, a lateral thrust of the knee may be seen during weight-bearing and may contribute to progression of the disease. Bracing using a valgus-producing knee-ankle-foot orthosis may lessen or reverse progression but must be instituted Bowed Legs early in the disease and before 3 years old.47 If progression to more advanced disease occurs, surMost cases of genu varum in infants and young gical correction is necessary before age 5. children are due to physiologic bowing, which is often Bone dysplasias and rickets will display characterseen in combination with internal istic bony findings on plain radiotibial torsion. Both deformities are graphs and children often present thought to be due to in utero posiwith short stature. Flared metaphtioning. The primary pathologic Shoes do not induce arch yses and irregularity of the physes cause is Blount syndrome, aloccur in rickets, while bone dysthough rickets and bone dyspladevelopment and should plasias involve varying changes sias should be considered in the be viewed only as depending on the underlying disdifferential. Parental concern or protection for the feet in order. Bowing from rickets may identification usually occurs shortly children under 5 years old. improve once correction of the after onset of walking. vitamin-D deficiency occurs, but In physiologic varus, children residual deformity requires surwalk with their feet pointing ingery. Likewise, bone dysplasias ward or forward. Close observausually develop progressive deformity that requires tion will demonstrate that the knees point outward. surgical correction to improve mechanics at the knee The combination of these findings suggests external and development of pain. rotation contractures at the hips with concomitant Bowed legs in older children usually represents internal tibial torsion (Fig 21). On the examination late-onset Blount syndrome. Children are often overtable, if the knees are positioned pointing forward/up, the weight. Unlike infantile Blount syndrome, late-onset feet will be found to point inward. Covering the feet (the Blount syndrome does not demonstrate radiographic “cover-up test”) with the examiner’s hand allows for changes to the medial tibial metaphysis and deformity unbiased evaluation of knee alignment, which is usually often arises from both the tibia and the femur (Fig 24). found to be straight.45 If the child is under 18 months old Excessive varus may cause knee pain, and progression and there is no concern for underlying bony abnormaliof knee osteoarthritis48; therefore, correction to neutral ties, observation is appropriate. If x-rays are obtained, a alignment is desirable for more extreme cases. If single hip-to-ankle AP radiograph demonstrates genu detected before onset of puberty, correction may be varum with equivalent contributions to the deformity obtained using minimally invasive surgery that uses from both the proximal tibia and the distal femur46 the principles of guided growth— hemiepiphysiode(Fig 22). sis, surgical tethering of the growth plate at the apex As the child approaches 2 years old, persistent of the deformity, allows for growth on the opposite, varus requires radiographic evaluation. Some chilconcave, side of the deformity, using the remaining dren take longer to resolve physiologic varus, which growth of the growth plate to straighten the leg (Fig

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Curr Probl Pediatr Adolesc Health Care, January 2011

Fig 15. (A) Flattening and the bright white sclerosis indicative of avascular necrosis of the fourth metatarsal head consistent with Freiberg infraction. (B) Complete healing, with residual flattening (arrowhead), in the same patient 6 months later.

Fig 16. (A) Accessory navicular with ossification center (circular density, arrow) and prominence of the overlying soft tissues. (B) MRI of the same patient demonstrates edema of the navicular (upper arrow) and accessory ossicle (lower arrow) due to traction on the intervening synchondrosis (arrowhead). (Color version of figure is available online.)

25). Once skeletal maturity is obtained, surgical correction is much more extensive (Fig 26). Therefore, children should be evaluated during prepubertal physical examinations for evidence of excessive genu varum or valgum.

Curr Probl Pediatr Adolesc Health Care, January 2011

Knock-Kneed After physiologic varus resolves, children progress into valgus at the knees and attain adult alignment by age 6. After this, children may appear knock-kneed

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Fig 17. (A) The fifth metatarsal apophysis is obliquely oriented (arrowhead). (B) A transverse fracture (arrowhead) through the base of the fifth metatarsal.

due to true genu valgum or apparent genu valgum from medial collapse of the knee during weightbearing. When watching the child walk, the patella will point medially if there is medial collapse from immature neuromuscular control. This may be demonstrated in a more isolated fashion by asking the child to perform a single-legged squat—the knee will collapse and medially rotate as the child squats, and in many cases the child will be extremely unsteady. In addition, the legs will appear straight when the child lies on the table with the patellae pointing forward. In true genu valgum, the patellae usually point forward when walking, and genu valgum persists when examining the child on the table. As with genu varum, radiographs are only necessary if true genu valgum is suspected clinically. Again, a single AP radiograph that extends from hips to ankles allows for evaluation of alignment and identification of the source of deformity (Fig 18). Early detection allows for guided-growth therapy before skeletal maturity, although more severe deformity requires more extensive surgery (Fig 25). Immature neuromuscular control with medial knee collapse almost always resolves spontaneously by skeletal maturity. Rarely, physical therapy may be indicated for the child with

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anterior knee or hip pain to improve core strength and control of hip internal rotation during weight-bearing.

In-Toeing and W-Sitting At birth, newborns’ feet may be noted to point inward, usually due to metatarsus adductus combined with preferential hind foot varus due to in utero positioning. Once children begin to walk, the feet may point in. This should really be considered part of the normal spectrum and not pathologic despite being a less common foot position during walking.43 Internal tibial torsion causes the inward positioning of the foot and is most easily measured by the thigh-foot angle, the alignment of the foot with the thigh when the patient is laying prone with his knees bent (Fig 19). Spontaneous improvement usually occurs, and bracing has no effect on changing the natural history.49 Surgical intervention is reserved only for children with persistent functional difficulties after age 6, such as tripping and falling. Such difficulties are common in children with cerebral palsy, but most neurologically normal children will adapt. Neurological consultation should be considered solely in children with persistent

Curr Probl Pediatr Adolesc Health Care, January 2011

Fig 18. The progression of the tibiofemoral angle in children during growth from varus in infancy (left), to valgus in toddlerhood (center), and finally to an adult-like appearance of mild valgus by age 5 (right).

difficulties or other neurologic Children with mild cerebral findings on examination. Parents palsy may also present with inCrooked legs after age 6 toeing.52 Findings suggestive of should be told that internal tibial cerebral palsy include ipsilateral torsion does not impart an athletic are abnormal and an upper extremity posturing and toedisadvantage, even if it does not orthopedic referral is walking during gait evaluation, fully resolve.50 warranted. Excessive femoral anteversion, and signs of spasticity on examiwhich presents as the external ronation, such as limited range of tation contractures of hip due to in motion at the ankle, hyperreflexia, utero positioning, resolves within and clonus. Examination of the a year of onset of walking. In children with internal hips may reveal excessive internal rotation; asymmettibial torsion, the torsion usually improves as the ric internal rotation of the hip is always abnormal and external rotation contractures at the hip resolve and may suggest spasticity on the side with increased excessive femoral anteversion becomes more obvious. range of motion, especially if there is also limitation in Therefore, little change in foot position occurs when hip abduction on the same side. walking. Examining the patient in the prone position Excessive femoral anteversion in neurologically norand noting the degrees of tibial torsion and internal mal children resolves by 8 years old, and bracing does and external rotation of the hip with each evaluation not change the natural course.53 Children with persistent excessive femoral anteversion may develop external demonstrate the resolution and evolution of the respectibial torsion as they approach adolescence.54 This “mistive processes. Identifying excessive femoral antevererable malalignment syndrome” commonly causes antesion clinically uses the relationship between internal rior knee pain and early referral is warranted. Physical and external rotation of the hips in a prone position therapy may help with symptomatic pain relief, but (Fig 27). Internal rotation at least 45 degrees greater persistent pain requires surgical correction of either 1 or than external rotation predicts true excessive femoral both rotational deformities.55 anteversion.51 Increased, but equivalent, passive range W-sitting raises many concerns from parents, grandof motion for both internal and external rotation parents, teachers, and therapists, especially regarding suggests generalized increased range of motion at the contributions to persistent intoe-ing, compensatory exterhips and is reassuring, even if there is underlying nal tibial torsion, and neuromuscular control. Children excessive femoral anteversion.

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Fig 19. With the patient prone, and the heel aligned with the lower leg, the thigh-foot angle (lines) defines the degree of tibial torsion. (Color version of figure is available online.)

Fig 21. (A) External rotation contractures (note the outward pointing patellae) combined with internal tibial torsion generates an appearance of genu varum. (B) Positioning the same child’s leg with the patella pointing upward while sitting results in near-complete resolution of the apparent varus deformity to a normal position. (Color version of figure is available online.)

Out-Toeing

Fig 20. External rotation of the hip results in an outward pointing of the patella (arrow), masking femoral anteversion, but internal tibial torsion results in a forward-pointing foot. (Color version of figure is available online.)

with and without femoral anteversion preferentially wsit56 (Fig 28). Telling children not to w-sit has no effect on the natural history of in-toeing or femoral anteversion,53 and most outgrow it, presumably because intrinsic laxity and femoral anteversion resolve. The risk for long-term hip problems remains unclear, but may be lower than one might think.57

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As children begin to walk, out-toeing gait is most common. This is due to external rotation contractures of the hips that are greater than any concomitant internal tibial torsion or in combination with true external tibial torsion. Improvement in foot position is usually spontaneous as the external rotation contractures about the hip resolve. External tibial torsion is considered pathologic when greater than 30 degrees as the biomechanics of gait during weight-bearing are abnormal.58 Unlike internal tibial torsion, external tibial torsion does not seem to go through the same regression to neutral alignment and may progress with time, resulting in worsening flatfoot deformity and development of pain.54 Surgical correction before onset of worsening deformity, usually during toddlerhood, is preferred.

Curr Probl Pediatr Adolesc Health Care, January 2011

Fig 22. Physiological varus, as evidenced by the mechanical axes (lines), drawn from the center of the femoral head to the center of the ankle joint, which passes medially to the knee, with grossly equal bowing from both the femur and tibia.

Fig 23. Isolated tibia vara with changes to the medial tibial metaphysis in a patient with infantile Blount syndrome. (Color version of figure is available online.)

Developmental Delay Delayed onset of walking should raise concern regarding an underlying neuromuscular problem, such

Curr Probl Pediatr Adolesc Health Care, January 2011

Fig 24. Adolescent Blount syndrome with unilateral bowing of both the left femur and tibia (arrowheads) without metaphyseal changes.

as hypotonia or spasticity. Bony alignment does not prevent gross motor development; therefore, evaluation should start with consultation by neurology, genetics, and/or developmental pediatrics. Developmental delay occurs secondary to neuromuscular, not orthopedic, disease. Since children with neuromuscular disorders are at risk for development of hip dysplasia and dislocation, scoliosis, and gait abnormalities, orthopedic monitoring is recommended, with radiographic screening of the hips for those at risk.59 Children with neuromuscular disorders often benefit from orthotic use, ranging from foot orthoses to ones that extend from the hip to the foot. Supramalleolar orthoses extend from the midfoot to above the ankle, but allow plantarflexion and dorsiflexion. Supramalleolar orthoses are particularly useful in children with hypotonia, improving their functional movements60; the use in children who are not yet walking is controversial. Children with spasticity (eg, cerebral palsy) or more profound lower extremity weakness (eg, spina bifida) may require even greater support from an ankle-foot orthosis, knee-ankle-foot orthosis or hip-knee-ankle-foot orthoses. Consultation with a pediatric orthopedist or rehabilitation physician is appropriate and is often necessary for optimal management of children with neuromuscular disorders.

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Fig 25. (A) Hemiepiphysiodesis, using lateral tethering of the growth plate, for genu varum (same patient as in Fig 24). (B) Correction of adolescent Blount syndrome to neutral alignment in the same patient by skeletal maturity. (C) Hemiepiphysiodesis may also be used in genu valgum.

Fig 26. Corrective osteotomy for genu valgum in a skeletally mature patient.

Leg Length Difference Children who have significant leg length differences usually present when they begin walking, unless there

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is an obvious limb deformity. The shorter leg will often be compensated for by the child walking on the tiptoes on that side and/or the child will be noted to “fall” to the side of the shorter leg. Clinically, limb length differences are assessed by measuring the difference between the height of the iliac crests when standing with knees straight, measuring from anterior superior iliac spine to the tip of the medial malleolus and measuring relative differences in the lengths of the femurs (by having the patient lay on their back with hips and knees both bent 90 degrees) and lower legs (by having the patient lay prone with their knees bent 90 degrees). Since all of these methods are subject to false measurement from pelvic tilt or rotation, radiographic measurement is indicated when significant differences are detected clinically (Fig 29A). Presentation of a dislocated hip in a child of walking age may appear as a “leg length difference,” but limitations in hip range of motion in the “shorter” leg coexist, and the dislocation is identifiable on radiographs (Fig 30). Otherwise, leg length differences may be due to processes that affect blood flow to the physes, trauma, or congenital disorders, although many cases are idiopathic. Inflammatory disorders, such as arthritis, infection, and vascular malformations, may all stimulate bone growth at the physes. By contrast, inflam-

Curr Probl Pediatr Adolesc Health Care, January 2011

Fig 27. (A) Hip internal rotation and (B) external rotation in the prone position. Both movements in this child are within the normal range. Often, these movements seem counterintuitive, as internal rotation of the hip and thigh result in the foot moving laterally, while external rotation of the hip and thigh results in the foot moving medially. (Color version of figure is available online.)

Fig 28. W-sitting is a common, and comfortable, position for many children. (Color version of figure is available online.)

matory disorders, infection, and bone tumors may injure the physes and hinder bone growth. Trauma is typically considered to induce growth arrest when the physis is involved (Salter-Harris fractures), but frac-

Curr Probl Pediatr Adolesc Health Care, January 2011

tures of the shaft of the tibia and femur may “overgrow” during the subacute phase of bone healing. Congenital hemiatrophy may be identified by underlying abnormal appearance to the bones on radiographs, although mild cases may have extremely subtle radiographic differences, such as blunting of the tibial spines or smaller femoral condyles, or a positive Lachman maneuver may be found on physical examination due to associated congenital absence of the anterior cruciate ligament. Care should be taken to differentiate hemiatrophy from contralateral hemihypertrophy. The difference in limb lengths at skeletal maturity determines long-term outcomes and the need for intervention. For etiologies with relatively constant differences in growth rates, ie, congenital causes, difference at skeletal maturity may be predicted based on remaining growth.61 Roughly speaking, differences in leg length at skeletal maturity will be 3 times greater in a 1 year old, 2 times greater in a 4 year old, and 1.5 times greater in a 6 year old; by 10 years old, less than a 20% increase in the difference would be expected. In cases of growth arrest, differences are determined based on the remaining growth of the comparable physis on the uninvolved side. At skeletal maturity, less than 1 cm difference in leg lengths is functionally insignificant in most people. Adults with 1- to 2-cm difference may develop pain and/or arthritis,62 so partial or complete correction using a heel lift placed inside the shoe and/or an in-sole lift are recommended— heel lifts of up to 1 cm may be tolerated within the shoe, but greater correction should use the insole of the shoe, modified by a skilled cobbler/orthotist. Once differences reach 2 cm, surgical correction is indicated.63 Counterintuitively, if the discrepancy is predicted to be less than 5 cm at skeletal maturity, the contralateral, longer leg, should undergo epiphysiodesis, a surgically induced growth arrest, during puberty to allow the shorter leg to “catch-up” by skeletal maturity. Differences greater than 5 cm would result in excessive loss of height if contralateral epiphysiodesis is used, so lengthening of the shorter limb is indicated (Fig 29B). An external fixation device is attached to the bone, which has been surgically cut, and the fixator is gradually lengthened; bone formation occurs in the intervening defect of bone. Multiple lengthenings may be staged, starting in early childhood, to correct more extreme differences by skeletal maturity.

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Hemihypertrophy

ings on examination. Children with any variations from this presentation require further evaluation with When a child is noted to have a difference in the radiographs, to evaluate possible hip disorders or girth of a limb, with or without a difference in length, tumors (Fig 31), and laboratory analysis should inhemihypertrophy should be suspected unless findings clude at a minimum a complete blood count with of a hemiatrophy syndrome exist. Since hemihypertrodifferential and erythrocyte sedimentation rate. C-rephy (also referred to hemihyperplasia) is considered active protein (inflammatory, not high-sensitivity), along the spectrum with Beckwith-Wiedeman synlactate dehydrogenase, alkaline phosphatase, 25-OH drome, children are at risk for development of intravitamin D, and/or Lyme titers (in endemic areas) abdominal tumors. Current recommendations include should also be considered.69,70 Both radiographs and screening for intra-abdominal tumors through serum laboratory tests should be interpreted with caution, but alpha-fetoprotein levels every 3 months until 4 years may reassure the clinician and parent that there is not old and intra-abdominal ultrasounds every 3 months a systemically active process at the time of evaluation. until 8 years old.64 Children with hemihypertrophy The exact etiology of “growing pains” remains due to vascular malformations, such as with Klippelunknown, although numerous theories seem plausiTrenaunay, are not at risk of intra-abdominal tumors ble.71 Families should be encouraged to continue to 65 and screening is not necessary. monitor for concerning signs or symptoms, such as limp, fever, joint swelling, and progressively worsening pain that may suggest a pathologic entity and Leg Pains warrant reevaluation even if all is Excellent articles exist on the normal initially. Many children’s evaluation and differential diagnosymptoms improve with physical sis of leg pains and limping in therapy for stretching and/or strengthchildren.66 Disorders of the hip Developmental delay ening (including core strength) and/or may present with hip pain, but use of orthotics.72,73 occurs secondary to buttock, groin, anterior thigh or neuromuscular, not knee pain commonly represents orthopedic, disease. pain referred from the hip. ThereSpine Deformity fore, assessment of pain in these Abnormal appearance of the areas should include evaluation of back may present in the sagittal the hip for potential infection, arplane, due to excessive thoracic kyphosis (⬎50 dethritis, slipped capital femoral epiphysis, or Legggrees on the lateral view) or lumbar lordosis, or may Calve-Perthes disease, especially if there is limitation occur as the result of scoliosis, which is defined as a in hip range of motion. Also, slipped capital femoral curve of greater than 10 degrees on a an AP or PA epiphysis may present outside the classic age range scoliosis x-ray (Fig 32). Examination of the spine and in nonobese children. This “atypical SCFE” usushould be performed at all well-child checks from ally occurs in the setting of an endocrinologic disorkindergarten through skeletal maturity using the der. It may occur either as part of primary hypothyAdam’s forward-bending test (Fig 33). Prior to this roidism or during initiation of thyroid replacement. time, recognition of possible deformity often comes Children who receive growth hormone, for either from the parents, who notice that the child sits tilted to growth hormone deficiency, short stature, or other 1 side, or 1 shoulder is higher than the other. Thorough disorders, such as Turner syndrome or Prader–Willi 67 neurologic evaluation, including strength testing, syndrome, are at risk. should be performed for any concerns regarding the The term “growing pains” is frequently misused to back or spine. Cutaneous abnormalities over the spine describe any recurrent pain children experience in may suggest occult spinal dysraphism and may require their legs. Classic growing pains are bilateral, located further evaluation with ultrasound, MRI, or neurosurbehind the knees, in the calves, or over the anterior 68 gical consultation. thigh and occur in the evening or at night. Pain When identified in the infant, congenital spine deshould not interfere with daytime activity and the child should be without systemic symptoms or focal findformity should be suspected as the cause of both

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Curr Probl Pediatr Adolesc Health Care, January 2011

Fig 29. (A) A patient with a 6.5-cm leg length discrepancy as measured on a scanogram, a special radiograph whereby a metal ruler is placed between the patient’s legs and separate images of the hips, knees, and ankles are performed on the same cassette. A scanogram allows for accurate measurement of leg length discrepancy and the contributions from the femur and the tibia. (B) Lengthening using an external fixator corrects the entire discrepancy in this patient before skeletal maturity.

Fig 30. A 4-year old referred for a leg length discrepancy. The proximal position of the dislocated femoral head (arrow), relative to location of the anatomic acetabulum (arrowheads), creates the appearance of a foreshortened femur and limb.

kyphosis and scoliosis, and radiographs may reveal underlying abnormalities in vertebral body development. Additional evaluation of the cardiac, renal, and central nervous system should occur because concom-

Curr Probl Pediatr Adolesc Health Care, January 2011

itant abnormalities are common. If scoliosis without vertebral malformations is found, MRI evaluation should still occur due to the association with underlying Chiari malformation and spinal dysraphism.74 Regardless of radiographic findings, infants with spinal deformity should be evaluated by an orthopedist, and potentially a neurologist, for underlying causes and appropriate treatment. Juvenile scoliosis presents after the first year of life, but before the prepubertal growth spurt. Children with neuromuscular disorders often develop scoliosis during this time span, but in the absence of known disease, other primary etiologies should be sought, such as Marfan syndrome, Ehler-Danlos syndrome, neurofibromatosis, or central nervous system anomalies, such as Chiari malformation, spinal cord syrinx, or tethered cord. Approximately 20% of children with juvenile scoliosis will have 1 of these abnormalities.75 Clinical evaluation by genetics or neurology should be considered, and an MRI of the head and total spine is recommended. Management of the curve focuses in slowing progression using bracing or growing rods. Ultimately, spinal fusion is necessary in most children, occurring around adolescence, when there is little remaining spine growth.

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Fig 31. Imaging of a patient who presented with leg pain and a final diagnosis of an osteoid osteoma. (A) Plain radiographs demonstrate the thickened bone (arrow). (B) CT scan reveals thickening of the cortical bone around the osteoid osteoma lesion (arrowhead). (C) Axial CT image showing the central nidus (arrowhead).

Fig 32. (A) A normal, straight spine on the PA view of the entire spine. Standing scoliosis films are taken from posterior-anterior to minimize radiation exposure to the thyroid and breast tissue. As a result, the heart appears on the left side, as if the patient was being viewed from behind, and does not represent situs inversus. (B) The lateral view of the spine demonstrates kyphosis of the thoracic spine (between arrows) and lordosis of the lumbar spine (between arrowheads).

Typically, spine deformity is noted during puberty or afterward. Scoliosis worsens with spinal growth, so progression usually occurs most rapidly during the time before peak height velocity. Scoliosis evaluations

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should occur at each well-child check, but if a screening approach is used, females should be screened twice, at 10 and 12 years old, while males should be screened once at age 13 or 14.76 Not every abnormal

Curr Probl Pediatr Adolesc Health Care, January 2011

Fig 33. Adam’s forward bending test revealing rotation of the trunk with elevation of the right rib cage relative to the left in a patient with mild scoliosis. (Color version of figure is available online.)

examination requires radiographs, but more pronounced abnormalities on clinical examination should be evaluated with standing PA and lateral scoliosis x-rays to visualize the entire spine and all potential components of the deformity. Up to half of all curves with an apex (convex) to the left in the thoracic spine have associated central nervous system abnormalities

(Fig 34), so MRI of the head and spine should be performed77; syndromic associations should also be considered. Curves of 10-25 degrees, as measured by the Cobb angle, should be followed clinically and radiographically every 4-6 months until skeletal maturity.78 Curves of greater than 25 degrees should be referred to an orthopedist for further management. Typically, bracing is used for curves of 25-40 degrees when there is considered a risk for further progression, ie, sufficient remaining growth. Curves greater than 50 degrees have a risk of further progression even after skeletal maturity, so spinal fusion is recommended to prevent progression. Kyphosis is usually due to postural deformity or underlying Scheuermann kyphosis. Scheuerman kyphosis is an abnormality in vertebral body development identified on the lateral scoliosis x-ray and is defined as anterior wedging of ⬎5 degrees in 3 consecutive vertebrae (Fig 35). The benefits of physical therapy or reminders to “standup straight” on postural kyphosis are unknown, but neither will change the course of Scheuermann kyphosis. In fact, children with Scheuermann kyphosis cannot stand up straight due to the underlying bony deformity, so parents should be encouraged to abandon these re-

Fig 34. (A) Plain radiographs in a patient with an apex left thoracic curve. (B) A cervical syrinx (arrowheads) was found on MRI [same patient as (A)].

Curr Probl Pediatr Adolesc Health Care, January 2011

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lack of training and experience. Development of an appropriate differential diagnosis helps guide evaluation. Once a diagnosis is made, use of other available resources helps guide treatment and decisions regarding the need for further consultation.

References

Fig 35. Lateral radiograph displays wedging of thoracic vertebral bodies (arrows) and endplate irregularities (arrowheads) of Scheuermann disease in a patient presenting with excessive kyphosis.

minders. As long as the overall kyphotic deformity is ⬍50 degrees, no intervention is necessary. For Scheuermann kyphosis ⬎50 degrees, bracing may be considered, or surgical correction may be offered for kyphosis ⬎75 degrees, progressive deformity, and/or relief of pain. Atypical Scheuermann disease is an abnormality of the lumbar spine. There is a decrease in lumbar lordosis, and endplate abnormalities (Schmorl’s nodes) are seen (Fig 35). Spontaneous recovery usually occurs, although patients may present with low back pain. Physical therapy may relieve symptoms. A rare cause of uneven shoulders is Sprengel deformity, an abnormality in scapular development and descent. Sprengel deformity is seen in 30% of patients with Klippel–Feil syndrome, so cervical x-rays should be performed to identify associated Klippel–Feil syndrome, which is also associated with spinal stenosis and precludes participation in contact sports due to the risk of catastrophic spinal cord injury.

Conclusions The diagnosis and management of orthopedic disorders may feel daunting to child health clinicians due to

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